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SECTION 1 Headache and Facial Pain Syndromes

Chapter 1
ICE PICK HEADACHE
ICD-9 CODE 784.0
ICD-10 CODE

R51

The Clinical Syndrome


Ice pick headache is a constellation of symptoms consisting of
paroxysms of stabbing jabs and jolts that occur primarily in
the first division of the trigeminal nerve. These paroxysms of
pain may occur as a single jab or a series of jabs that last for a
fraction of a second followed by relatively pain-free episodes.
The pain of ice pick headache occurs in irregular intervals of
hours to days. Similar to cluster headache, ice pick headache is
an episodic disorder that is characterized by clusters of painful attacks followed by pain-free periods. Episodes of ice pick
headache usually occur on the same side, but in rare patients,
the pain may move to the same anatomical region on the contralateral side. Ice pick headache occurs more commonly in
women and is generally not seen before the fourth decade of
life, but rare reports of children suffering from ice pick headache sporadically appear in the literature. Synonyms for ice
pick headache include jabs and jolts headache and idiopathic
stabbing headache.

Testing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put the
patient at risk for neurological disasters secondary to intracranial
and brainstem pathological conditions, including tumors and
demyelinating disease (Figure 1-2). Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms,
which may be responsible for the patients neurological findings.
In patients who cannot undergo MRI, such as a patient with a
pacemaker, computed tomography (CT) is a reasonable second
choice. Radionuclide bone scanning and plain radiography are
indicated if fracture or bony abnormality, such as metastatic disease, is considered in the differential diagnosis.

Signs and Symptoms


A patient suffering from ice pick headache complains of jolts or
jabs of pain in the orbit, temple, or parietal region (Figure 1-1).
Some patients describe the pain of ice pick headache as a sudden
smack or slap on the side of the head. Similar to patients suffering from trigeminal neuralgia, a patient suffering from ice pick
headache may exhibit involuntary muscle spasms of the affected
area in response to the paroxysms of pain. In contrast to trigeminal neuralgia, involving the first division of the trigeminal
nerve, there are no trigger areas that induce the pain of ice pick
headache. The neurological examination of a patient suffering
from ice pick headache is normal. Some patients exhibit anxiety
and depression because the intensity of pain associated with ice
pick headache leads many patients to believe they have a brain
tumor.

Figure 1-1 Ice pick headache is characterized by jabs or jolts in the


orbit, temple, or parietal region.

2 SECTION 1 Headache and Facial Pain Syndromes

chronic paroxysmal hemicrania lasts much longer than the pain of


ice pick headache and is associated with redness and watering of
the ipsilateral eye.

Treatment
Ice pick headache uniformly responds to treatment with indomethacin. Failure to respond to indomethacin puts the diagnosis
of ice pick headache in question. A starting dosage of 25 mg daily
for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased
to 150 mg per day. Indomethacin must be used carefully, if at
all, in patients with peptic ulcer disease or impaired renal function. Anecdotal reports of a positive response to cyclooxygenase-2
(COX-2) inhibitors in the treatment of ice pick headache have
been noted in the headache literature. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a
single bedtime dose of 25 mg.

Complications and Pitfalls

Figure 1-2 Diffuse pachymeningeal and calvarial metastasis from carcinoma of the breast. Axial T1-weighted postgadolinium MRI shows
diffuse nodular and bandlike contrast-enhanced thickening of the dura
over the high right frontoparietal convexity. (From Haaga JR, Lanzieri
CF, Gilkeson RC, editors: CT and MR imaging of the whole body, 4th ed,
Philadelphia, 2003, Mosby, p 198.)

Screening laboratory tests consisting of complete blood cell


count, erythrocyte sedimentation rate, and automated blood
chemistry should be performed if the diagnosis of ice pick headache is in question. Intraocular pressure should be measured if
glaucoma is suspected.

Failure to correctly diagnose ice pick headache may put the patient
at risk if intracranial pathological conditions or demyelinating disease, which may mimic the clinical presentation of chronic paroxysmal hemicrania, is overlooked. MRI is indicated in all patients
thought to be suffering from ice pick headache. Failure to diagnose glaucoma, which also may cause intermittent ocular pain,
may result in permanent loss of sight.

Clinical Pearls
The diagnosis of ice pick headache is made by obtaining
a thorough, targeted headache history. Patients suffering
from ice pick headache should have a normal neurological
examination. If the results of the neurological examination
are abnormal, the diagnosis of ice pick headache should be
discarded and a careful search for the cause of the neurological findings should be undertaken.

Differential Diagnosis
Ice pick headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic ice pick headache
include trigeminal neuralgia involving the first division of the
trigeminal nerve, demyelinating disease, and chronic paroxysmal
hemicrania. Trigeminal neuralgia involving the first division of
the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic
neuritis and other motor and sensory abnormalities. The pain of

SUGGESTED READINGS
Cutrer FM, Boes CJ: Cough, exertional, and sex headaches, Neurol Clin 22:
133149, 2004.
Dafer RM: Neurostimulation in headache disorders, Neurol Clin 28:835841,
2010.
Mathew NT: Indomethacin responsive headache syndromes: headache, J Head
Face Pain 21:147150, 1981.
Pascual J: Other primary headaches, Neurol Clin 27:557571, 2009.
Tuba T, Serap , Esra O, etal: Features of stabbing, cough, exertional and sexual headaches in a Turkish population of headache patients, J Clin Neurosci
15:774777, 2008.

Chapter 2
SUPRAORBITAL NEURALGIA

ICD-9 CODE 350.1


ICD-10 CODE G50.0
The Clinical Syndrome
The pain of supraorbital neuralgia is characterized as persistent
pain in the supraorbital region and forehead with occasional sudden, shocklike paresthesias in the distribution of the supraorbital
nerves. Sinus headache involving the frontal sinuses, which is
much more common than supraorbital neuralgia, can mimic the
pain of supraorbital neuralgia. Supraorbital neuralgia is the result
of compression or trauma of the supraorbital nerves as the nerves
exit the supraorbital foramen. Such trauma can be in the form of
blunt trauma directly to the nerve, such as when the forehead hits
the steering wheel during a motor vehicle accident, or repetitive
microtrauma resulting from wearing welding or swim goggles that
are too tight. This clinical syndrome also is known as swimmers
headache.

and brainstem pathological conditions, including tumors and


demyelinating disease (Figure 2-3). Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms,
which may be responsible for the patients neurological findings.
In patients who cannot undergo MRI, such as a patient with a
pacemaker, computed tomography (CT) is a reasonable second
choice. Radionuclide bone scan, CT, and plain radiography are
indicated if sinus disease, fracture, or bony abnormality such as
metastatic disease is considered in the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of supraorbital neuralgia is in question. Intraocular pressure should be measured if glaucoma is suspected (Figure 2-4).

Differential Diagnosis
Supraorbital neuralgia is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, CT, and MRI. Pain syndromes that may mimic supraorbital

Signs and Symptoms


The supraorbital nerve arises from fibers of the frontal nerve,
which is the largest branch of the ophthalmic nerve. The frontal
nerve enters the orbit via the superior orbital fissure and passes
anteriorly beneath the periosteum of the roof of the orbit. The
frontal nerve gives off a larger lateral branch, the supraorbital
nerve, and a smaller medial branch, the supratrochlear nerve. Both
exit the orbit anteriorly. The supraorbital nerve sends fibers all
the way to the vertex of the scalp and provides sensory innervation to the forehead, upper eyelid, and anterior scalp (Figure 2-1).
The pain of supraorbital neuralgia is characterized as persistent
pain in the supraorbital region and forehead with occasional sudden, shocklike paresthesias in the distribution of the supraorbital
nerves. Occasionally, a patient suffering from supraorbital neuralgia complains that the hair on the front of the head hurts
(Figure 2-2). Supraorbital nerve block is useful in the diagnosis
and treatment of supraorbital neuralgia.

Inflamed
supraorbital n.

Testing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put the
patient at risk for neurological disasters secondary to intracranial

Figure 2-1 The supraorbital nerve sends fibers all the way to the vertex
of the scalp and provides sensory innervation to the forehead, upper
eyelid, and anterior scalp. n, Nerve.

4 SECTION 1 Headache and Facial Pain Syndromes

Figure 2-2 Occasionally, a patient with supraorbital neuralgia complains that the hair on the front of the head hurts. The supraorbital
nerve sends fibers all the way to the vertex of the scalp and provides
sensory innervation to the forehead, upper eyelid, and anterior scalp.

neuralgia include ice pick headache, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, and chronic paroxysmal hemicrania. Trigeminal neuralgia
involving the first division of the trigeminal nerve is uncommon
and is characterized by trigger areas and tic-like movements.
Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory
abnormalities. The pain of chronic paroxysmal hemicrania lasts
much longer than the paroxysmal pain of supraorbital neuralgia
and is associated with redness and watering of the ipsilateral eye.

Treatment
The primary treatment intervention for supraorbital neuralgia is
the identification and removal of anything causing compression
of the supraorbital nerves (e.g., tight welding or swim goggles).
A brief trial of simple analgesics alone or in combination with
gabapentin also should be considered. For patients who do not
respond to these treatments, supraorbital nerve block with local
anesthetic and a steroid is a reasonable next step.
To perform supraorbital nerve block, the patient is placed in
the supine position. Using a 10-mL sterile syringe, 3 mL of local
anesthetic is drawn up. When treating supraorbital neuralgia with

supraorbital nerve block, 80 mg of depot steroid is added to the


local anesthetic with the first block, and 40 mg of depot steroid is
added with subsequent blocks.
The supraorbital notch on the affected side is identified by
palpation. The skin overlying the notch is prepared with antiseptic solution, with care taken to avoid spillage into the eye. A
25-gauge, 1-inch needle is inserted at the level of the supraorbital notch and is advanced medially approximately 15 degrees
off the perpendicular to avoid entering the foramen. The needle
is advanced until it approaches the periosteum of the underlying
bone (Figure 2-5). A paresthesia may be elicited, and the patient
should be warned of such. The needle should not enter the supraorbital foramen; if this occurs, the needle should be withdrawn
and redirected slightly more medially.
Because of the loose alveolar tissue of the eyelid, a gauze sponge
should be used to apply gentle pressure on the upper eyelid and
supraorbital tissues before injection of solution to prevent the
injectate from dissecting inferiorly into these tissues. This pressure should be maintained after the procedure to avoid periorbital
hematoma and ecchymosis.
After gentle aspiration, 3 mL of solution is injected in a fanlike
distribution. If blockade of the supratrochlear nerve also is desired,
the needle is redirected medially and, after careful aspiration, an
additional 3 mL of solution is injected in a fanlike manner.
Underlying sleep disturbance and depression associated with
the pain of supraorbital neuralgia are best treated with a tricyclic antidepressant compound, such as nortriptyline. The tricyclic
antidepressant can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls


Failure to diagnose supraorbital neuralgia correctly may put the
patient at risk if an intracranial pathological condition or demyelinating disease, which may mimic the clinical presentation of
supraorbital neuralgia, is overlooked. MRI is indicated in all
patients thought to have supraorbital neuralgia. Failure to diagnose glaucoma, which also may cause intermittent ocular pain,
may result in permanent loss of sight.
The forehead and scalp are highly vascular, and when performing supraorbital nerve block the clinician should carefully
calculate the total milligram dosage of local anesthetic that may
be given safely, especially if bilateral nerve blocks are being performed. This vascularity gives rise to an increased incidence of
postblock ecchymosis and hematoma formation. Despite the
vascularity of this anatomical region, this technique can be performed safely in the presence of anticoagulation by using a 25or 27-gauge needle, albeit at increased risk for hematoma, if the
clinical situation dictates a favorable risk-to-benefit ratio. These
complications can be decreased if manual pressure is applied to
the area of the block immediately after injection. Application of
cold packs for 20-minute periods after the block also decreases the
amount of postprocedure pain and bleeding.

2 Supraorbital Neuralgia 5

C
Figure 2-3 Subdural empyema in a patient with sinusitis. A, T2-weighted MRI shows high-signal-intensity extraaxial fluid collection in the right frontal convexity and along the falx on the right side. B and C, Gadolinium-enhanced MRI shows extraaxial fluid collections in the right frontal convexity
and along the falx with intense peripheral enhancement. The signal intensity of the fluid collection is slightly higher than that of cerebrospinal fluid.
(From Haaga JR, Lanzieri CF, Gilkeson RC, editors: CT and MR imaging of the whole body, 4th ed, Philadelphia, 2003, Mosby, p 209.)

6 SECTION 1 Headache and Facial Pain Syndromes

Hazy corneal reflex


signifying edema

Fixed
semidilated
pupil
Cataractous
lens

Opaque thickened
edematous cornea

Cataractous lens

Shallow anterior
chamber

Figure 2-4 Acute angle closure resulting from an intumescent cataractous lens. The eye is red with a hazy view of the anterior segment from corneal
edema, with a fixed, irregular, semidilated pupil from iris infarction. The slit image shows the corneal edema and a very shallow anterior chamber.
Some uveitis may be present because of ischemia, and this must be differentiated from the larger accumulations of lens material and macrophages
seen with phacolytic glaucoma. (From Spalton DJ, Hitchings RA, Hunter P: Atlas of clinical ophthalmology, 3rd ed, London, 2005, Mosby, p 225.)

Clinical Pearls

Supraorbital n.
Supraorbital notch

Supraorbital nerve block is especially useful in the diagnosis


and palliation of pain secondary to supraorbital neuralgia.
The first step in the management of this unusual cause of
headache is the correct fitting of swimming goggles that
do not compress the supraorbital nerves. Coexistent frontal sinusitis should be ruled out in patients who do not
respond rapidly to a change in swim goggles and a series of
the previously mentioned nerve blocks. Any patient with
headaches severe enough to require neural blockade as part
of the treatment plan should undergo MRI of the head to
rule out unsuspected intracranial pathological conditions.
SUGGESTED READINGS
Levin M: Nerve blocks and nerve stimulation in headache disorders, Tech Reg
Anesth Pain Manage 13:4249, 2009.
Levin M: Nerve blocks in the treatment of headache, Neurotherapeutics 7:197
203, 2010.
Waldman SD: The trigeminal nerve. In Waldman SD, editor: Pain review, Philadelphia, 2009, Saunders, pp 1517.
Waldman SD: Swimmers headache. In Waldman SD, editor: Atlas of pain management injection techniques, Philadelphia, 2007, Saunders, pp 710.

Figure 2-5 Injection technique for relieving the pain of supraorbital


neuralgia. (From Waldman SD: Atlas of pain management injection techniques, 2nd ed, Philadelphia, 2007, Saunders.)

Chapter 3
CHRONIC PAROXYSMAL HEMICRANIA

As in cluster headache, the patient may become agitated during


attacks, rather than seeking dark and quiet as does the patient with
migraine. In contrast to cluster headache, alcohol consumption
does not seem to trigger attacks of chronic paroxysmal hemicrania. Between attacks, the neurological examination of a patient
with chronic paroxysmal hemicrania should be normal.

ICD-9 CODE 784.0


ICD-10 CODE

R51

The Clinical Syndrome


Chronic paroxysmal hemicrania shares many characteristics of its
more common analogue, cluster headache, but has several important differences (Table 3-1). Similar to cluster headache, chronic
paroxysmal hemicrania is a severe, episodic, unilateral headache
that affects the periorbital and retroorbital regions. In contrast
to cluster headache, which occurs 10 times more commonly in
men, chronic paroxysmal hemicrania occurs primarily in women
(Figure 3-1). The duration of pain associated with chronic paroxysmal hemicrania is shorter than that of cluster headache, lasting 5
to 45 minutes. This pain does not follow the chronobiological pattern seen in patients with cluster headache. Patients with chronic
paroxysmal hemicrania usually experience more than five attacks
per day. Chronic paroxysmal hemicrania uniformly responds to
indomethacin, whereas cluster headache does not.

Signs and Symptoms


During attacks of chronic paroxysmal hemicrania, patients exhibit
the following physical findings suggestive of Horners syndrome
on the ipsilateral side of the pain:
Conjunctival and scleral injection
Lacrimation
Nasal congestion
Rhinorrhea
Ptosis of the eyelid

Testing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put the
patient at risk for neurological disasters secondary to intracranial
and brainstem pathological conditions, including tumors and
demyelinating disease (Figure 3-2). Magnetic resonance angiography (MRA) also may be useful in identifying aneurysms, which
may be responsible for the patients neurological findings. In
patients who cannot undergo MRI, such as a patient with a pacemaker, computed tomography (CT) is a reasonable second choice.
Radionuclide bone scanning and plain radiography are indicated
if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of chronic

TABLE 3-1

Comparison of Cluster Headache and Chronic


Paroxysmal Hemicrania
Comparison Factors

Cluster
Headache

Chronic
Paroxysmal
Hemicrania

Gender predominance

Male

Female

Response to indomethacin

Negative

Positive

Chronobiological pattern

Positive

Negative

Alcohol trigger

Positive

Negative

Length of attacks

Longer

Shorter

Horners syndrome

Present

Present

Figure 3-1 In contrast to cluster headache, which occurs primarily in


men, chronic paroxysmal hemicrania occurs primarily in women.

8 SECTION 1 Headache and Facial Pain Syndromes

uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other
neurological findings, including optic neuritis and other motor
and sensory abnormalities. The pain of cluster headache lasts much
longer than the pain of chronic paroxysmal hemicrania, and cluster
headache has a male predominance, a chronobiological pattern of
attacks, and a lack of response to treatment with indomethacin.

Treatment

Chronic paroxysmal hemicrania uniformly responds to treatment


with indomethacin. Failure to respond to indomethacin puts the
diagnosis of chronic paroxysmal hemicrania in question. A starting dose of 25 mg daily for 2 days and titrating to 25 mg three
times per day is a reasonable treatment approach. This dose may
be carefully increased up to 150 mg per day. Indomethacin must
be used carefully, if at all, in patients with peptic ulcer disease or
impaired renal function. Anecdotal reports of a positive response
to cyclooxygenase-2 (COX-2) inhibitors in the treatment of
chronic paroxysmal hemicrania have been noted in the headache
literature. Underlying sleep disturbance and depression are best
treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls


Failure to diagnose chronic paroxysmal hemicrania correctly may
put the patient at risk if intracranial pathological conditions or
demyelinating disease that may mimic the clinical presentation of
chronic paroxysmal hemicrania is overlooked. MRI is indicated in
all patients thought to have chronic paroxysmal hemicrania. Failure to diagnose glaucoma, which may cause intermittent ocular
pain, may result in permanent loss of sight.

Clinical Pearls

B
Figure 3-2 Sagittal (A) and semiaxial (B) T2-weighted images of a massive prolactinoma in a 41-year-old man with chronic daily headache.
(From Benitez-Rosario MA, McDarby G, Doyle R, Fabby G. Chronic clusterlike headache secondary to prolactinoma: uncommon cephalalgia in association with brain tumors, J Pain Symptom Manage 37:271276, 2009.

paroxysmal hemicrania is in question. Intraocular pressure should


be measured if glaucoma is suspected.

Differential Diagnosis
Chronic paroxysmal hemicrania is a clinical diagnosis supported by
a combination of clinical history, abnormal physical examination
during attacks, radiography, and MRI. Pain syndromes that may
mimic chronic paroxysmal hemicrania include cluster headache,
trigeminal neuralgia involving the first division of the trigeminal
nerve, demyelinating disease, and ice pick headache. Trigeminal
neuralgia involving the first division of the trigeminal nerve is

The diagnosis of chronic paroxysmal hemicrania is made


by obtaining a thorough, targeted headache history.
Between attacks, patients with chronic paroxysmal hemicrania should have a normal neurological examination. If
the neurological examination is abnormal between attacks,
the diagnosis of chronic paroxysmal hemicrania should be
discarded and a careful search for the cause of the patients
neurological findings should be undertaken.
SUGGESTED READINGS
Benitez-Rosario MA, McDarby G, Doyle R, Fabby C: Chronic cluster-like headache secondary to prolactinoma: uncommon cephalalgia in association with
brain tumors, J Pain Symptom Manage 37:271276, 2009.
Benoliel R, Sharav Y: Paroxysmal hemicrania: case studies and review of the literature, Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol 85:285292,
1998.
Camarda C, Camarda R, Monastero R: Chronic paroxysmal hemicrania and
hemicrania continua responding to topiramate: two case reports, Clin Neurol
Neurosurg 110:8891, 2008.
Klasser GD, Balasubramaniam R: Trigeminal autonomic cephalalgias. II. Paroxysmal hemicrania, Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol
104:640646, 2007.
Sjaastad O: Chronic paroxysmal hemicrania: clinical aspects and controversies. In
Blau JN, editor: Migraine: clinical, therapeutic, conceptual and research aspects,
London, 1987, Chapman & Hall, pp 135152.
Talvik I, Koch K, Kolk A, Talvik T: Chronic paroxysmal hemicrania in a 3-year,
10-month-old female, Pediatr Neurol 34:225227, 2006.

Chapter 4
CHARLINS SYNDROME

ICD-9 CODE 350.1


ICD-10 CODE G50.0
The Clinical Syndrome
Charlins syndrome, also known as nasociliary neuralgia, is an
uncommon cause of head and face pain. As with most headache
syndromes, the exact cause of the pain of Charlins syndrome is
unknown. However, the pathogenesis of this uncommon cause
of head and face pain is thought to be dysfunction of the nasociliary ganglion in a manner analogous to the dysfunction of the
sphenopalatine ganglion thought to be the source of cluster headache. The pain of Charlins syndrome has a rapid onset to peak,
with attacks lasting 45 to 60 minutes. In some patients, these
attacks can be triggered by sensory stimulation of the affected
areas. Although in many ways similar to cluster headache (e.g.,
retroorbital location of pain, profuse unilateral rhinorrhea, rapid
onset to peak, and short duration of attacks), many dissimilarities
also exist. In contrast to cluster headache, alcohol consumption
does not appear to trigger attacks of Charlins syndrome and the
seasonal and chronobiological patterns so characteristic of cluster
headache do not seem to be a factor (Table 4-1). Blockade of the
sphenopalatine ganglion, which is so effective in the treatment of
cluster headache, is of little value in the treatment of Charlins
syndrome. Patients suffering from Charlins syndrome uniformly
respond to daily nasociliary nerve blocks with local anesthetic, as
described subsequently.

Signs and Symptoms


Patients suffering from Charlins syndrome present with the complaint of severe paroxysms of ocular or retroorbital pain that radiates into the ipsilateral forehead, nose, and maxillary region. This
pain is associated with voluminous ipsilateral rhinorrhea and congestion of the nasal mucosa and significant inflammation of the
affected eye (Figure 4-1).

Testing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put the
patient at risk for neurological disasters secondary to intracranial
and brainstem pathological conditions, including tumors and

demyelinating disease (Figure 4-2). Magnetic resonance angiography (MRA) also may be useful in helping identify aneurysms,
which may be responsible for the patients neurological findings.
In patients who cannot undergo MRI, such as a patient with a
pacemaker, computed tomography (CT) is a reasonable second
choice. Radionuclide bone scanning and plain radiography are
indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of Charlins syndrome is in question. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis
Charlins syndrome is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic Charlins
syndrome include cluster headache, temporal arteritis, trigeminal neuralgia involving the first division of the trigeminal nerve,
demyelinating disease, and chronic paroxysmal hemicrania (see
Table 4-1). Trigeminal neuralgia involving the first division of
the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic
neuritis and other motor and sensory abnormalities. The pain of
chronic paroxysmal hemicrania lasts much longer than the pain of
Charlins syndrome.

Treatment
The treatment of Charlins syndrome is analogous to the treatment of trigeminal neuralgia. The use of anticonvulsants such as
carbamazepine and gabapentin represents a reasonable starting
point. High-dose steroids tapered over 10 days also have been
anecdotally reported to provide relief. For patients who do not
respond to the previously mentioned treatments, daily nasociliary
ganglion block with local anesthetic and steroid is a reasonable
next step. Underlying sleep disturbance and depression associated
with the pain of supraorbital neuralgia are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can
be started at a single bedtime dose of 25 mg.

Complications and Pitfalls


Failure to diagnose Charlins syndrome correctly may put
the patient at risk if an intracranial pathological condition or
9

10 SECTION 1 Headache and Facial Pain Syndromes


TABLE 4-1

Comparison of Cluster Headache


and Charlins Syndrome
Comparison Factors

Cluster
Headache

Charlins
Syndrome

Ocular and retroorbital location

Yes

Yes

Unilateral

Yes

Yes

Rapid onset to peak

Yes

Yes

Severe intensity

Yes

Yes

Attacks occur in paroxysms

Yes

Yes

Duration of attacks short

Yes

Yes

Pain free between attacks

Yes

Yes

Significant rhinorrhea during attacks

Yes

Yes

Alcohol triggers attacks

Yes

No

Tactile trigger areas

No

Yes

Seasonal pattern of attacks

Yes

No

Chronobiological pattern of attacks

Yes

No

Significant eye inflammation

No

Yes

Responds to sphenopalatine
ganglion block

Yes

No

Responds to nasociliary block

No

Yes

Figure 4-2 Multiple sclerosis. Fluid-attenuated inversion recovery (FLAIR)


parasagittal MR image depicts the extensive demyelinated plaques of
progressive multiple sclerosis. (From Haaga JR, Lanzieri CF, Gilkeson RC,
editors: CT and MR imaging of the whole body, 4th ed, Philadelphia,
2003, Mosby, p 466.)

demyelinating disease, which may mimic the clinical presentation of Charlins syndrome, is overlooked. MRI is indicated in all
patients thought to have Charlins syndrome. Failure to diagnose
glaucoma or temporal arteritis, which also may cause intermittent
ocular pain, may result in permanent loss of sight.

Clinical Pearls
Nasociliary nerve block via the medial orbital approach
is especially useful in the diagnosis and palliation of pain
secondary to Charlins syndrome. Given the uncommon
nature of this headache syndrome and its overlap with the
symptoms of cluster headache and other neurological problems, including cavernous sinus thrombosis and intracranial
and retroorbital tumors, Charlins syndrome must remain a
diagnosis of exclusion. All patients suspected to have Charlins syndrome require MRI of the brain with and without
gadolinium contrast material and thorough ophthalmological and neurological evaluation. Nasociliary nerve block via
the medial orbital approach should be performed only by
clinicians familiar with the regional anatomy.

SUGGESTED READINGS

Figure 4-1 Patients suffering from Charlins syndrome present with the
complaint of severe paroxysms of ocular or retroorbital pain that radiates into the ipsilateral forehead, nose, and maxillary region. The pain is
associated with voluminous ipsilateral rhinorrhea and congestion of the
nasal mucosa and significant inflammation of the affected eye.

Becker M, Kohler R, Vargas MI, Viallon M, Delavelle J: Pathology of the trigeminal


nerve, Neuroimaging Clin N Am 18:283307, 2008.
Craven J: Anatomy of the cranial nerves, Anaesth Intensive Care Med 11:529534,
2010.
Lewis DW, Gozzo YF, Avner MT: The other primary headaches in children and
adolescents [review], Pediatr Neurol 33:303313, 2005.
Waldman SD: The trigeminal nerve. In Waldman SD, editor: Pain review, Philadelphia, 2009, Saunders, pp 1517.
Waldman SD: Charlins syndrome. In Waldman SD, editor: Atlas of pain management injection techniques, Philadelphia, 2007, Saunders, pp 2024.

Chapter 5
SEXUAL HEADACHE

ICD-9 CODE 784.0


ICD-10 CODE

R51

The Clinical Syndrome


Sexual headache is a term used to describe a group of headaches
associated with sexual activity. Clinicians have identified the following three general types of headache associated with sexual
activity:
Explosive type
Dull type
Postural type
Each of these sexual headache types was previously called benign
coital headache, but this term has been replaced by sexual headache because each may occur with sexual activity other than coitus
(Figure 5-1). In general, sexual headache includes a benign group
of disorders, but a rare patient may have acute subarachnoid hemorrhage during sexual activity, which may be erroneously diagnosed as the benign explosive type of sexual headache. There is
no gender predilection for sexual headache, and the occurrence of
all types of sexual headache may be episodic rather than chronic.
Rarely, more than one type of sexual headache occurs in the same
patient.

reported. The pain usually remains intense for 10 to 15 minutes


and then gradually abates. Some patients note some residual headache pain for 2 days.
Dull Type of Sexual Headache
The dull type of sexual headache begins during the early portion of sexual activity. This headache type has an aching character and begins in the occipital region. The headache becomes
holocranial as sexual activity progresses toward orgasm. It may
peak at orgasm, but in contrast to the explosive type of sexual
headache, the dull type disappears rapidly after orgasm. Ceasing
sexual activity usually aborts the dull type of sexual headache.
Some headache specialists think the dull type of sexual headache is simply a milder version of the explosive type of sexual
headache.
Postural Type of Sexual Headache
The postural type of sexual headache is similar to the explosive
type of sexual headache in that it occurs just before or during
orgasm. Its rapid onset to peak and severe intensity also are similar
to that of the explosive type. It differs from the explosive type of
headache in that the headache symptoms recur when the patient
stands up, in a manner analogous to postdural puncture headache. The postural component of this type of sexual headache is
thought to be due to minute tears in the dura that may occur during intense sexual activity.

Signs and Symptoms


Patients with sexual headache present differently depending on
the type of sexual headache experienced. Each clinical presentation is discussed subsequently.
Explosive Type of Sexual Headache
The explosive type of sexual headache is the most common type
of sexual headache encountered in clinical practice. The patient
usually fears he or she has had a stroke. The patient may be less
forthcoming about the circumstances surrounding the onset of
headache, and tactful questioning may be required to ascertain
the actual clinical history. The explosive type of sexual headache
occurs suddenly, with an almost instantaneous onset to peak just
before or during orgasm. The intensity of the explosive type of
sexual headache is severe and has been likened to the pain of acute
subarachnoid hemorrhage. The location of pain is usually occipital, but some patients volunteer that the pain felt like the top of
my head was going to blow off. The pain is usually bilateral, but
isolated cases of unilateral explosive sexual headache have been

Figure 5-1 Sexual headaches show no gender predilection and are generally benign.

11

12 SECTION 1 Headache and Facial Pain Syndromes

Testing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put the
patient at risk for neurological disasters secondary to intracranial
and brainstem pathological conditions, including tumors, demyelinating disease, and hemorrhage. More important, MRI helps
identify bleeding associated with leaking intracranial aneurysms.
Magnetic resonance angiography (MRA) may be useful in helping identify aneurysms responsible for the patients neurological
symptoms. In patients who cannot undergo MRI, such as patients
with pacemakers, computed tomography (CT) is a reasonable
second choice. Even if blood is not present on MRI or CT, if
intracranial hemorrhage is suspected, lumbar puncture should be
performed.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of sexual
headache is in question. Intraocular pressure should be measured
if glaucoma is suspected.

Differential Diagnosis
Sexual headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, MRI, and MRA. Pain syndromes that may mimic sexual
headache include trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, cluster headache, migraine, and chronic paroxysmal hemicrania. Trigeminal
neuralgia involving the first division of the trigeminal nerve is
uncommon and is characterized by trigger areas and tic-like
movements. Demyelinating disease is generally associated with
other neurological findings, including optic neuritis and other
motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania and cluster headache is associated with redness
and watering of the ipsilateral eye, nasal congestion, and rhinorrhea during the headache. These findings are absent in all types
of sexual headache. Migraine headache may or may not be associated with nonpainful neurological findings known as aura, but
the patient almost always reports some systemic symptoms, such
as nausea or photophobia, not typically associated with sexual
headache.

Treatment
It is generally thought that avoiding the inciting activity for a few
weeks decreases the propensity to trigger sexual headaches. If this
avoidance technique fails or is impractical because of patient preference, a trial of propranolol is a reasonable next step. A low dose
of 20 to 40 mg as a daily dose and titrating in 20-mg increments
to 200 mg as a divided daily dose until prophylaxis occurs treats

most patients suffering from sexual headache. Propranolol should


be used with caution in patients with asthma or cardiac failure and
patients with brittle diabetes.
If propranolol is ineffective, indomethacin may be tried. A
starting dose of 25 mg daily for 2 days and titrating to 25 mg
three times per day is a reasonable treatment approach. This dose
may be carefully increased to 150 mg per day. Indomethacin must
be used carefully, if at all, in patients with peptic ulcer disease or
impaired renal function. Anecdotal reports of a positive response
to cyclooxygenase-2 (COX-2) inhibitors in the treatment of sexual
headache have been noted in the headache literature. Underlying
sleep disturbance and depression are best treated with a tricyclic
antidepressant compound, such as nortriptyline, which can be
started at a single bedtime dose of 25 mg.

Complications and Pitfalls


Failure to diagnose sexual headache correctly may put the patient
at risk if intracranial pathology or demyelinating disease (which
may mimic the clinical presentation of sexual headache) is overlooked. MRI and MRA are indicated in all patients thought to
have sexual headache. Failure to diagnose glaucoma, which also
may cause intermittent ocular pain, may result in permanent loss
of sight.

Clinical Pearls
The diagnosis of sexual headache is made by obtaining a
thorough, targeted headache history. As mentioned earlier,
patients may not be forthcoming about the events surrounding the onset of their headache, and the clinician should be
sensitive to this fact. Patients suffering from sexual headache should have a normal neurological examination. If
the neurological examination is abnormal, the diagnosis of
sexual headache should be discarded and a careful search
for the cause of the patients neurological findings should
be undertaken.

SUGGESTED READINGS
Evans RW: Diagnostic testing for migraine and other primary headaches, Neurol
Clin 27:393415, 2009.
Hu CM, Lin YJ, Fan YK, etal: Isolated thunderclap headache during sex: orgasmic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci
17:13491351, 2010.
Jolobe OMP: The differential diagnosis includes reversible cerebral vasoconstrictor
syndrome, Am J Emerg Med 28:637, 2010.
Kim HJ, Seo SY: Recurrent emotion-triggered headache following primary headache associated with sexual activity, J Neurol Sci 273:142143, 2008.
Tuba T, Serap , Esra O, etal: Features of stabbing, cough, exertional and sexual headaches in a Turkish population of headache patients, J Clin Neurosci
15:774777, 2008.

Chapter 6
COUGH HEADACHE

ICD-9 CODE 784.0


ICD-10 CODE

R51

The Clinical Syndrome


Cough headache is a term used to describe headaches triggered by
coughing and other activities associated with a Valsalva maneuver,
such as laughing, straining at stool, lifting, and bending the head
toward the ground (Figure 6-1). Clinicians have identified the following two types of cough headache:
Benign
Symptomatic
Initially, both types of cough headache were thought to be
related to sexual and exertional headaches, but they are now considered distinct clinical entities. A strong male predilection is seen
for benign cough headache and no gender predilection for symptomatic cough headache.

Signs and Symptoms


Patients suffering from cough headache present differently
depending on the type of cough headache experienced. Each clinical presentation is discussed.
Benign Cough Headache
Benign cough headache is not associated with obvious neurological or musculoskeletal disease. More than 80% of patients with
benign cough headache are males, in contradistinction to symptomatic cough headache, in which no gender predilection is seen.
The onset of benign cough headache is abrupt, occurring immediately after coughing or other activities that cause a Valsalva maneuver. Although the intensity of pain is severe and peaks rapidly, it
lasts only seconds to minutes. The character of the pain associated
with benign cough headache is splitting or sharp, and the pain is
located in the occipital region bilaterally and occasionally the vertex of the skull. No accompanying neurological or systemic symptoms are seen, as with cluster and migraine headaches. The age of
onset of benign cough headache is generally in the late fifth or sixth
decade of life. If such headaches occur before age 50, there should
be strong clinical suspicion that the patient either has symptomatic cough headache or a pathological condition in the posterior
fossa, such as Arnold-Chiari malformation or tumor. Tumors of
the foramen magnum also may mimic the presentation of benign
cough headache even if no neurological symptoms are present.

Symptomatic Cough Headache


Symptomatic cough headache is almost always associated with
structural abnormalities of the cranium, such as Arnold-Chiari
malformation I and II or intracranial tumors (Figure 6-2). The
symptoms associated with symptomatic cough headache are
thought to be due to herniation of the cerebellar tonsil through
the foramen magnum into the space normally occupied by the
upper portion of the cervical spinal cord. Similar to benign cough
headache, the onset of pain associated with symptomatic cough
headache is abrupt, occurring immediately after coughing or other
activities that cause a Valsalva maneuver. Although the intensity
of pain is severe and peaks rapidly, it lasts only seconds to minutes. In contrast to benign cough headache, associated neurological symptoms may be present, including difficulty swallowing,
faintness, and numbness in the face and upper extremities. These
associated symptoms should be taken very seriously because they
are indicative of increased intracranial pressure and herniation of
the intracranial contents.
The character of the pain associated with symptomatic cough
headache is splitting or sharp, and pain is located in the occipital
region bilaterally and occasionally the vertex of the skull. The
age of onset of symptomatic cough headache is generally in the
third decade of life, although, depending on the amount of neurological compromise, it may occur at any age. In contrast to
benign cough headache, which occurs predominantly in men,
symptomatic cough headache occurs with equal prevalence in
both genders.

Testing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put
the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors
and demyelinating disease. Special attention to the foramen magnum may help identify more subtle abnormalities responsible
for posterior fossa neurological signs and symptoms. MRI helps
identify bleeding associated with leaking intracranial aneurysms,
which may mimic the symptoms of both types of cough headache. Magnetic resonance angiography (MRA) may be useful in
helping identify aneurysms responsible for the patients neurological symptoms. In patients who cannot undergo MRI, such as
patients with pacemakers, computed tomography (CT) is a reasonable second choice. Lumbar puncture should be performed if
intracranial hemorrhage is suspected, even if blood is not present
on MRI or CT. Plain radiographs of the cervical spine also may
be useful in the evaluation of Arnold-Chiari malformations and
13

14 SECTION 1 Headache and Facial Pain Syndromes

Herniation of
cerebellar tonsil
Figure 6-1 Symptomatic cough headache is often
associated with structural abnormalities, such as
Arnold-Chiari malformation, and usually occurs in the
third decade of life.

Spinal cord

headache is in question. Intraocular pressure should be measured


if glaucoma is suspected.

Differential Diagnosis
Cough headache is a clinical diagnosis supported by a combination
of clinical history, physical examination, radiography, MRI, and
MRA. Pain syndromes that may mimic cough headache include
benign exertional headache, ice pick headache, sexual headache,
trigeminal neuralgia involving the first division of the trigeminal
nerve, demyelinating disease, cluster headache, and chronic paroxysmal hemicrania. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized
by trigger areas and tic-like movements. Demyelinating disease
is generally associated with other neurological findings, including
optic neuritis and other motor and sensory abnormalities. The
pain of chronic paroxysmal hemicrania and cluster headache is
associated with redness and watering of the ipsilateral eye, nasal
congestion, and rhinorrhea during the headache. These findings
are absent in all types of cough headache. Migraine headache
may or may not be associated with painless neurological findings known as aura, but the patient almost always reports some
systemic symptoms, such as nausea or photophobia, not typically
associated with cough headache.

Treatment
Figure 6-2 Low-lying cerebellar tonsils (straight arrows) of a Chiari malformation are shown deforming the medulla (curved arrow) in a sagittal
T1-weighted spin echo image. 4, Fourth ventricle. (From Stark DD, Bradley WG Jr, editors: Magnetic resonance imaging, 3rd ed, St Louis, 1999,
Mosby, p 1841.)

should be included in the evaluation of all patients with cough


headache.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of cough

Indomethacin is the treatment of choice for benign cough headache. A starting dose of 25 mg daily for 2 days and titrating to
25 mg three times per day is a reasonable treatment approach.
This dose may be carefully increased up to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic
ulcer disease or impaired renal function. Headache specialists have
noted anecdotal reports of a positive response to cyclooxygenase-2
(COX-2) inhibitors in the treatment of benign cough headache.
Underlying sleep disturbance and depression are best treated with
a tricyclic antidepressant compound, such as nortriptyline, which
can be started at a single bedtime dose of 25 mg.
The only uniformly effective treatment for symptomatic cough
headache is surgical decompression of the foramen magnum.

6 Cough Headache 15

This surgery is usually done via suboccipital craniectomy. Surgical decompression prevents the low-lying cerebellar tonsils from
obstructing the flow of spinal fluid from the cranium to the spinal
subarachnoid space during a Valsalva maneuver.

Complications and Pitfalls


Failure to diagnose cough headache correctly may put the patient
at risk if intracranial pathology or demyelinating disease (which
may mimic the clinical presentation of cough headache) is overlooked. MRI and MRA are indicated in all patients thought to
have cough headache. Failure to diagnose glaucoma, which also
may cause intermittent ocular pain, may result in permanent loss
of sight.

Clinical Pearls
Any patient presenting with headaches associated with
exertion or Valsalva maneuver should be taken very seriously. Although statistically most of these headaches ultimately are proved to be of benign cause, a few patients have
potentially life-threatening disease. The diagnosis of cough
headache is made by obtaining a thorough, targeted headache history and performing a careful physical examination.
The clinician must separate patients suffering from benign
cough headache from patients suffering from symptomatic cough headache. Patients with benign cough headache
should have a normal neurological examination. If the neurological examination is abnormal, the diagnosis of benign
cough headache should be discarded and a careful search
for the cause of the patients neurological findings should
be undertaken.

SUGGESTED READINGS
Berciano J, Poca M-A, Garca A, Sahuquillo J: Paroxysmal cervicobrachial coughinduced pain in a patient with syringomyelia extending into spinal cord posterior gray horns, J Neurol 54:678681, 2007.
Chen YY, Lirng JF, Fuh JL, etal: Primary cough headache is associated with posterior fossa crowdedness: a morphometric MRI study, Cephalalgia 24:694699,
2004.
Pascual J: Primary cough headache, Curr Pain Headache Rep 9:272276, 2005.
Pascual J, Rubn Martn A, Oterino A: Headaches precipitated by cough, prolonged exercise or sexual activity: a prospective etiological and clinical study,
JHeadache Pain 9:259266, 2008.
Waldman SD: Arnold Chiari malformation type I. In Waldman SD, Campbell
RS, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 2728.
Waldman SD: Arnold Chiari malformation type II. In Waldman SD, Campbell
RS, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 2930.

Chapter 7
SUDDEN UNILATERAL
NEURALGIFORM CONJUNCTIVAL
INJECTION TEARING HEADACHE
ICD-9 CODE 350.1
ICD-10 CODE G50.0
The Clinical Syndrome
Sudden unilateral neuralgiform conjunctival injection tearing
(SUNCT) headache is an uncommon primary headache disorder
that is one of a group of three headache syndromes known as the
trigeminal autonomic cephalgias (Table 7-1). Whether SUNCT
headache is in fact a distinct headache entity or simply a constellation of symptoms that occurs on a continuum along with
the other trigeminal autonomic cephalgias is a point of ongoing
debate among headache and pain management specialists (Figure
7-1). As with most headache syndromes, the exact cause of the
pain of SUNCT headache is unknown; however, the pathogenesis
of this uncommon cause of head and face pain is thought to be
dysfunction of the trigeminal autonomic reflex.
The pain of SUNCT headache has a rapid onset to peak, with
attacks lasting 5 seconds to 4 minutes and the frequency of attacks
ranging from 20 to 200 attacks per day. In some patients, these
attacks can be triggered by sensory stimulation of the affected
areas, such as when washing the face, brushing the teeth, and so
forth. Although in many ways similar to cluster headache (e.g.,
unilateral, periorbital and frontal location of pain, sclera injection, rapid onset to peak, short duration of attacks, and pain-free
periods between attacks), SUNCT exhibits many dissimilarities as
well. In contrast to cluster headache, alcohol consumption does
not seem to trigger attacks of SUNCT headache, and there do not
seem to be the seasonal and chronobiological patterns so characteristic of cluster headache, although SUNCT headache occurs
most frequently in the morning and afternoon (Table 7-2).
Blockade of the sphenopalatine ganglion, which is so effective in the treatment of cluster headache, is of little value in the
treatment of SUNCT headache. Patients suffering from SUNCT
headache may respond to daily trigeminal nerve blocks with local
anesthetic, as described subsequently.

Signs and Symptoms


Patients with SUNCT headache present with the complaint of
severe paroxysms of ocular or periorbital pain that radiate into
the ipsilateral temple, forehead, nose, cheek, throat, and maxillary
region. This pain is associated with significant inflammation of the
affected eye (Figure 7-2). The pain is neuralgiform and severe to
16

excruciating in intensity (Table 7-3). SUNCT occurs on the right


side 70% of the time in a manner analogous to trigeminal neuralgia. Like trigeminal neuralgia, rare cases of bilateral SUNCT
headache have been reported. Also like trigeminal neuralgia, the
pain of SUNCT headache rarely switches sides. SUNCT headache occurs slightly more frequently in males. It can occur at any
age, with a peak incidence in the fifth decade.

Testing
Magnetic resonance imaging (MRI) of the brain provides the clinician with the best information regarding the cranial vault and
its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters
secondary to intracranial and brainstem pathological conditions,
including tumors and demyelinating disease. Magnetic resonance
angiography (MRA) also may be useful in helping identify aneurysms, which may be responsible for the patients neurological
findings. In patients who cannot undergo MRI, such as patients
with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography
are indicated if fracture or bony abnormality such as metastatic
disease is considered in the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of SUNCT
TABLE 7-1

Trigeminal Autonomic Cephalgias


Cluster headache
Chronic paroxysm hemicrania
SUNCT headache
SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing.

SUNCT

Paroxysmal
hemicrania

Cluster
headache

5 s4 min

230 min

15180 min

Overlap
between duration

Time

Overlap
between duration

Figure 7-1 Overlap between attack duration in trigeminal autonomic


cephalalgias. SUNCT, Sudden unilateral neuralgiform conjunctival
injection tearing. (From Leone M, Bussone G: Pathophysiology of trigeminal autonomic cephalalgias, Lancet Neurol 8:755774, 2009.)

7 Sudden Unilateral Neuralgiform Conjunctival Injection Tearing Headache 17

headache is in question. Intraocular pressure should be measured


if glaucoma is suspected.

Differential Diagnosis
SUNCT headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography,
and MRI. Pain syndromes that may mimic SUNCT headache
include cluster headache, temporal arteritis, trigeminal neuralgia
TABLE 7-2

Comparison of Cluster Headache and Sudden Unilateral


Neuralgiform Conjunctival Injection Tearing Headache
Comparison Factors

Cluster
Headache

SUNCT
Headache

Ocular and retroorbital location

Yes

Yes

Unilateral

Yes

Yes

Rapid onset to peak

Yes

Yes

Severe intensity

Yes

Yes

Attacks occur in paroxysms

Yes

Yes

Duration of attacks short

Yes

Yes

Pain free between attacks

Yes

Yes

involving the first division of the trigeminal nerve, demyelinating


disease, primary stabbing headache, hypnic headache, and chronic
paroxysmal hemicranias, although because of the overlapping features of all headache and facial pain syndromes, SUNCT headache can be easily mistaken for another type of headache or facial
pain (Figure 7-3; Table 7-4). Trigeminal neuralgia involving the
first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating
disease is generally associated with other neurological findings,
including optic neuritis and other motor and sensory abnormalities. The pain of chronic paroxysmal hemicrania lasts much longer
than the pain of SUNCT headache.

Treatment
The treatment of SUNCT headache is analogous to the treatment
of trigeminal neuralgia, although the pharmacological management of this uncommon headache disorder is disappointing. The
use of anticonvulsants such as lamotrigine and gabapentin represents a reasonable starting point. High-dose steroids tapered over
10 days also have been anecdotally reported to provide relief. For
patients who do not respond to the previously mentioned treatments, daily trigeminal nerve block with a local anesthetic and
steroid is a reasonable next step.
Occasionally, retrogasserian injection of glycerol, balloon compression of the Gasserian ganglion, and microvascular decompression of the trigeminal nerve root are required to provide palliation
of pain. Underlying sleep disturbance and depression associated
with the pain of SUNCT headache are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be
started at a single bedtime dose of 25 mg.

Significant rhinorrhea during attacks

Yes

No

Alcohol triggers attacks

Yes

No

Tactile trigger areas

No

Yes

Seasonal pattern of attacks

Yes

No

Chronobiological pattern of attacks

Yes

No

Significant eye inflammation

No

Yes

Responds to sphenopalatine ganglion


block

Yes

No

Complications and Pitfalls

Responds to trigeminal nerve block

No

Yes

Failure to diagnose SUNCT headache correctly may put the


patient at risk if an intracranial pathological condition or demyelinating disease, which may mimic the clinical presentation of
SUNCT headache, is overlooked. MRI is indicated in all patients
thought to have SUNCT headache. Failure to diagnose glaucoma
or temporal arteritis, which also may cause intermittent ocular
pain, may result in permanent loss of sight.

SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing.

TABLE 7-3

Descriptors of Pain Associated with Sudden Unilateral


Neuralgiform Conjunctival Injection Tearing Headache
Stabbing
Shooting
Lancinating
Shocklike
Sharp
Piercing
Pricking
Figure 7-2 Patients with SUNCT headache present with severe paroxysms of ocular or periorbital pain that radiates into the ipsilateral temple,
forehead, nose, cheek, throat, and maxillary region that is associated
with significant inflammation of the affected eye.

Staccato-like

18 SECTION 1 Headache and Facial Pain Syndromes


Cluster headache

Neck
Paroxysmal hemicrania

Figure 7-3 Pain location in the trigeminal autonomic cephalgias (TACs)


and neurovascular orofacial pain. The TACs are characterized by orbital
and periorbital pain. In paroxysmal hemicrania and hemicrania continua, large adjacent areas are affected. Migraine is largely unilateral but
may be bilateral in up to 30% of cases (this has been marked by a lighter
shaded area contralaterally). Neurovascular orofacial pain is characterized by its location in the lower two thirds of the face with intraoral and
perioral areas frequently involved as primary sites. Two-headed arrow
above diagram indicates the side shift that occurs in specific headache.
NVOP, Neurovascular orofacial pain. (Modified from Benoliel R, Sharav Y:
The trigeminal autonomic cephalgias (TACs). In: Sharav Y, editor: Orofacial
pain and headache, Edinburgh, 2008, Elsevier, pp 225254.)

TABLE 7-4

Differential Diagnosis: Sudden Unilateral Neuralgiform


Conjunctival Injection Tearing Headache
Shoulder,
neck, arm
SUNCT

Cluster headache
Temporal arteritis
Trigeminal neuralgia
Demyelinating disease
Primary stabbing headache
Hypnic headache
Chronic paroxysmal hemicrania

Clinical Pearls
Hemicrania continua

Migraine

Trigeminal nerve block with local anesthetic is especially


useful in the diagnosis and palliation of pain secondary to
SUNCT headache. Given the uncommon nature of this
headache syndrome and its overlap with the symptoms of
cluster headache and other neurological problems, including cavernous sinus thrombosis and intracranial and retroorbital tumors, SUNCT headache must remain a diagnosis of
exclusion. All patients suspected to have SUNCT headache
require MRI of the brain with and without gadolinium contrast material and thorough ophthalmological and neurological evaluation. Trigeminal nerve block should be performed
only by clinicians familiar with the regional anatomy.
SUGGESTED READINGS

NVOP

Leone M, Bussone G: Pathophysiology of trigeminal autonomic cephalalgias, Lancet Neurol 8:755774, 2009.
Levin M: Nerve blocks and nerve stimulation in headache disorders, Tech Reg
Anesth Pain Manage 13:4249, 2009.
Levin M: Nerve blocks in the treatment of headache, Neurotherapeutics 7:197
203, 2010.
Klasser GD, Balasubramaniam R: Trigeminal autonomic cephalalgias. III. Short-
lasting unilateral neuralgiform headache attacks with conjunctival injection and
tearing, Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol 104: 773771.
2007.
Rozen TD: Trigeminal autonomic cephalalgias, Neurol Clin 27:537557, 2009.
Waldman SD: The trigeminal nerve. In Waldman SD, editor: Pain Review, Philadelphia, 2009, Saunders, pp 1517.
Waldman SD: Gasserian ganglion block. In Waldman SD, editor: Atlas of interventional pain management, ed 3, Philadelphia, 2009, Saunders, pp 3238.
Waldman SD: Gasserian ganglion block: balloon compression technique. In
Waldman SD, editor: Atlas of interventional pain management, ed 3, Philadelphia, 2009, Saunders, pp 4347.
Waldman SD: Trigeminal nerve block: coronoid approach. In Waldman SD, editor: Atlas of interventional pain management, ed 3, Philadelphia, 2009, Saunders,
pp 4750.
Williams MH, Broadley SA: SUNCT and SUNA: clinical features and medical
treatment, J Clin Neurosci 15:527534, 2008.

Chapter 8
PRIMARY THUNDERCLAP
HEADACHE

ICD-9 CODE 339.43


ICD-10 CODE G 44.53

focal neurological signs often associated with acute subarachnoid


hemorrhage and other neurologically devastating syndromes in
which severe headache of acute onset are a prominent feature are
uniformly absent.

Testing
The Clinical Syndrome
Thunderclap headache is an uncommon type of headache that
may be the result of an underlying vascular or nonvascular intracranial abnormality or may represent a primary headache syndrome of unknown cause. Common and uncommon causes of
thunderclap headache are listed in Table 8-1. The more benign,
though no less painful, primary thunderclap headache occurs over
three times more frequently than the serious secondary thunderclap headache. Because of the often-threatening causes of the less
common secondary thunderclap headache (e.g., subarachnoid
hemorrhage, cerebral venous thrombosis), urgent evaluation
including computed tomography (CT) and/or magnetic resonance
imaging of the brain and cerebrospinal fluid analysis are indicated
in all patients suspected of having thunderclap headache.
One of the most severe headaches encountered in clinical
practice, thunderclap headache is characterized by a very rapid
onset to peak of less than 1 minute. The headache may last
from 1 to 10 days and, because of its intensity, almost always
provokes an urgent trip to the emergency department, where
the headache is invariably initially misdiagnosed as the sentinel
headache of acute subarachnoid hemorrhage or other potentially
catastrophic headache syndromes (Tables 8-2 and 8-3). This is
not surprising in that primary thunderclap headache is virtually
indistinguishable clinically from subarachnoid hemorrhage, one
of the most neurologically devastating forms of cerebrovascular
accident. Thus, because of the serious consequences of misdiagnosis, by necessity primary thunderclap headache is a diagnosis
of exclusion.

Signs and Symptoms


As mentioned earlier, primary thunderclap headache is characterized by a very rapid onset to peak of less than 1 minute
without obvious inciting factors (e.g., sexual activity, coughing, straining at stool). The patient with primary thunderclap
headache is almost always convinced that he or she is having
a stroke and often appears frightened and anxious. The headache of primary thunderclap headache can be located anywhere
in the head or neck. Nausea and vomiting is present approximately 75% of the time. However, the nuchal rigidity and other

Testing in patients suspected of having primary thunderclap


headache has two immediate goals: (1) to identify occult intracranial pathological conditions or other diseases that may mimic
primary thunderclap headache and may require specific urgent
treatment and (2) to identify the presence of subarachnoid
hemorrhage. All patients with a recent onset of severe headache
thought to be secondary to primary thunderclap headache should
undergo emergent CT of the brain to rule out any pathological
condition that could be responsible for the patients symptoms
TABLE 8-1

Main and Rare Causes of Thunderclap Headache


Main Causes

Rare Causes

Vascular disorders
Subarachnoid hemorrhage

Pituitary apoplexy, arteritis,


angiitis

Intracerebral hemorrhage

Unruptured vascular
malformation, aneurysm

Cerebral venous thrombosis

Arterial hypertension

Spontaneous intracranial
hypotension

Cerebral segmental
vasoconstriction

Cervical artery dissection


Nonvascular disorders
Greater occipital neuralgia
Intermittent hydrocephalus by
colloid cyst
Infections
Meningitis, encephalitis

Erve virus
Sinusitis

Primary headache disorders


Migraine

Cluster headache

Primary thunderclap headache

Tension headache, new daily


persistent headache

Primary exertional headache


Primary cough headache
Linn FHH: Primary thunderclap headache. In: Aminoff MJ, editor: Handbook of
clinical neurology, vol 97, New York, 2010, Elsevier, pp 473481.

19

20 SECTION 1 Headache and Facial Pain Syndromes


TABLE 8-2

Comparison of Primary Thunderclap Headache and


Subarachnoid Hemorrhage
Primary Thunderclap
Headache

Subarachnoid
Hemorrhage

Severe headache

Yes

Yes

Nausea and vomiting

Yes

Yes

Focal neurological signs

No

Yes

Nuchal rigidity

No

Yes

Photophobia

No

Yes

Vertigo

No

Yes

Neck and back pain

No

Yes

Comparison Factors

TABLE 8-3

Diseases That May Mimic Primary Thunderclap


Headache
Hemorrhagic
Ischemic
Neoplasm
Infection

Meningitis
Encephalitis
Abscess
Parasitic
Hypertensive crisis
Loss of spinal fluid
Postdural puncture headache
Spontaneous spinal fluid leak
Collagen-vascular disease
Lupus cerebritis
Vasculitis
Polymyositis
Headache
Cluster headache
Primary exertional headache
Primary cough headache
Migraine
Ice pick headache
Primary sexual headache

(Figure 8-1). Modern multidetector CT scanners have a diagnostic accuracy approaching 100% for subarachnoid hemorrhage if
CT angiography of the cerebral vessels is part of the scanning
protocol. Cerebral angiography may also be required if surgical
intervention is being considered and the site of bleeding cannot
be accurately identified.
Magnetic resonance imaging (MRI) of the brain and magnetic
resonance angiography may be useful if an aneurysm is not identified on CT studies and may be more accurate in the diagnosis of
arteriovenous malformations (Figure 8-2). Screening laboratory
tests, including an erythrocyte sedimentation rate, complete blood
count, coagulation studies, and automated blood chemistry,

C
Figure 8-1 Computed tomography scan showing subarachnoid hemorrhage (SAH). Right middle cerebral artery aneurysm in a 58-year-old
man with SAH and intracranial hematoma (IH). A, Volume rendering
image from computed tomography angiography (CTA) clearly displays
the relationship of the aneurysm to bone structures, adjacent branch
vessels, and aneurysmal neck (arrow). B, Maximum intensity projection (MIP) image from CTA clearly demonstrates the relationship of the
aneurysm (arrow). C, Thin-MIP image from CTA shows the relationship
of the aneurysm to IH (arrowhead), and the ruptured aneurysm has a
small nipple (arrow). (From Chen W, Yang Y, Xing W, etal: Applications
of multislice CT angiography in the surgical clipping and endovascular coiling of intracranial aneurysms, J Biomed Res 24:467473, 2010.)

should be performed in patients with subarachnoid hemorrhage.


Blood typing and crossmatching should be considered in any
patient in whom surgery is being contemplated or who has preexisting anemia. Careful serial ophthalmological examination
should be performed on all patients with subarachnoid hemorrhage to chart the course of papilledema.
Lumbar puncture is useful in revealing the presence or absence
of blood in the spinal fluid, but its utility may be limited by the
presence of increased intracranial pressure, making lumbar puncture too dangerous. Electrocardiographic abnormalities are common in patients with subarachnoid hemorrhage and are thought
to be due to abnormally high levels of circulating catecholamines

8 Primary Thunderclap Headache 21

Figure 8-2 Magnetic resonance imaging showing arteriovenous malformation. Patient with aneurysm-related false aneurysm (FA) in right parietal
region. Preangiographic T1-weighted magnetic resonance axial image (A) and T2-weighted magnetic resonance coronal image (B) show round lesion
(arrow) with flow void and mixed signal in the center and mixed signal on the periphery. Fluid attenuated inversion recovery image (C) reveals small
area of surrounding edema (arrow). D, Flow in the center of FA (arrow) on two-dimensional time-of-flight magnetic resonance angiography. E, Preembolization digital subtraction angiography image. F, Residual inflow to FA (arrow) on postembolization DSA image. (From Brzozowski K, Frankowska E,
Piasecki P, etal. The use of routine imaging data in diagnosis of cerebral pseudoaneurysm prior to angiography, Eur J Radiol. 80:e401e409, 2011.)

and hypothalamic dysfunction; however, they are rarely present in


patients with primary thunderclap headache.

Differential Diagnosis
The differential diagnosis of primary thunderclap headache generally can be thought of as the diagnosis of the lesser of two evils
because most of the diseases that mimic primary thunderclap
headache are also associated with significant mortality and morbidity. Table 8-3 lists diseases that may be mistaken for primary
thunderclap headache. Prominent among them is subarachnoid
hemorrhage, stroke, collagen-vascular disease, infection, neoplasm, hypertensive crisis, spinal fluid leaks, and a variety of more
benign causes of headache.

Treatment
Although no generally accepted treatment for primary thunderclap headache has been defined, the following guidelines may be
useful for the clinician when faced with a patient thought to have
this uncommon headache syndrome. First and foremost, if test
results reveal no evidence of intracranial pathology or other serious, life-threatening diseases, constant reassurance that the patient

does not have a stroke or brain tumor is indicated. In general,


drugs used to treat headaches whose primary mechanism of action
is vasoconstriction (e.g., ergots, triptans) should be avoided. Anecdotal reports indicate that intravenous nimodipine may help abort
acute attacks and prevent recurrent headache episodes. Gabapentin also has been advocated as a reasonable treatment for primary
thunderclap headache and given its favorable risk-to-benefit ratio
may be a reasonable therapeutic option.

Complications and Pitfalls


Complications and pitfalls in the diagnosis and treatment of primary thunderclap headache generally fall into three categories.
The first category involves the failure to recognize a sentinel bleed
associated with subarachnoid hemorrhage and evaluate and treat
the patient before significant morbidity or mortality occurs. The
second category involves misdiagnosis that results in unnecessary
testing, in particular, cerebral angiography, which itself is associated with significant morbidity and rarely death. The third category involves iatrogenic morbidity and rarely mortality from the
use of medications to treat primary thunderclap headache (e.g.,
triptans, ergots) that not only do not treat this primary headache
syndrome but also have significant side effects.

22 SECTION 1 Headache and Facial Pain Syndromes

Clinical Pearls
Primary thunderclap headache is a diagnosis of exclusion.
It is frequently misdiagnosed as the sentinel headache of
subarachnoid hemorrhage, causing the treating physician
to order urgent diagnostic testing, which is associated with
its own significant mortality and morbidity. The lack of
focal neurological findings in a patient with acute headache should point the clinician toward the diagnosis of
benign primary headaches including primary thunderclap
headache, cough headache, exertional headache atypical
migraine, and headache associated with sexual activity. This
does not mean that urgent computerized scanning of the
brain and analysis of the patients cerebrospinal fluid are
not indicated.

SUGGESTED READINGS
Anderson T: Current and evolving management of subarachnoid hemorrhage,
Crit Care Nurs Clin North Am 21:529539, 2009.
Chih-Ming H, Ya-Ju L, Yang-Kai F, Shih-Pin C, Tzu-Hsien L: Isolated thunderclap headache during sex: orgasmic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci 17:13491351, 2010.
de Bruijn SFTM, Stam J, Kappelle LJ: CVST Study Group: Thunderclap headache as
first symptom of cerebral venous sinus thrombosis, Lancet 348:16231625, 1996.
Janardhan V, Biondi A, Riina HA, etal: Vasospasm in aneurysmal subarachnoid
hemorrhage: diagnosis, prevention, and management, Neuroimaging Clin N Am
6:483496, 2006.
Linn FHH: Primary thunderclap headache. In: Aminoff MJ, editor: Handbook of
clinical neurology, vol 97, New York, 2010, Elsevier, pp 473481.
Manno EM: Subarachnoid hemorrhage, Neurol Clin 22:347366, 2004.
Newfield P: Intracranial aneurysms: vasospasm and other issues. In Atlee JL, editor:
Complications in anesthesia, ed 2, Philadelphia, 2007, Saunders, pp 719723.
Palestrant D, Connolly ES: Subarachnoid hemorrhage, Neurobiol Dis 265270,
2007.
Pouration N, Dumont AS, Kassell NF: Subarachnoid hemorrhage. In Alves W
and Skolnick B, editors: Handbook of neuroemergency clinical trials, New York,
2006, Elsevier, pp 1744.

Chapter 9
HYPNIC HEADACHE

ICD-9 CODE 339.81


ICD-10 CODE G44.81
The Clinical Syndrome
Also known as alarm clock headache, hypnic headache is a term
used to describe an uncommon headache syndrome characterized by its propensity to wake the person at the same time each
night. Always occurring during sleep, hypnic headache is of
short duration and rarely lasts more than 15 minutes after the
patient is awakened by the pain (Figure 9-1). Research suggests
that hypnic headache occurs most commonly during rapid eye
movement (REM) sleep. Hypnic headache occurs frequently,
with a mean incidence of at least 15 attacks per month. The
location of the headache pain is variable and the intensity of
pain described as moderate with an aching character. Unlike
as in cluster headache, which also occurs after sleep, patients
with hypnic headache exhibit no autonomic signs or symptoms.
Hypnic headache is a disease of the late fifth and sixth decades,
with a mean age of onset of 63 years. It occurs more commonly
in females.

Testing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put
the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors
and demyelinating disease. MRI helps identify bleeding associated with leaking intracranial aneurysms, which may mimic the
symptoms of both types of hypnic headache. Magnetic resonance
angiography (MRA) may be useful in identifying aneurysms
responsible for the patients neurological symptoms. In patients
who cannot undergo MRI, such as patients with pacemakers,
computed tomography (CT) is a reasonable second choice. Lumbar puncture should be performed if intracranial hemorrhage is

First night: 2:34 AM

Signs and Symptoms


Hypnic headache is associated with no obvious neurological or
musculoskeletal disease. Specifically, there are no autonomic
signs or symptoms as are often seen with cluster headache.
Furthermore, no accompanying focal neurological signs or systemic symptoms occur as with cluster headache and migraine
headache, although rarely nausea can occur. The age of onset
of benign hypnic headache is generally in the late fifth or sixth
decade of life. Although no specific location is seen in hypnic
headache, they are bilateral in 66% of patients. When the headaches are unilateral, they tend to occur on the same side night
after night. What is fascinating to the treating physician and
frustrating to the patient is the fact that hypnic headache wakes
the patient from a sound sleep at almost the same time each
night. Because the onset of hypnic headache occurs later in life,
it must be considered a diagnosis of exclusion as with the other
uncommon primary headache syndromes, for example, cough
headache and thunderclap headache. The clinician must assiduously search for other explanations for the patients headache
symptomatology, including intracranial pathological conditions
and systemic disease.

Second night: 2:34 AM

Third night: 2:34 AM


Figure 9-1 Hypnic headache is also known as alarm clock headache due
to its propensity to wake the person up at the same time each night.

23

24 SECTION 1 Headache and Facial Pain Syndromes

suspected even if blood is not present on MRI or CT. Plain radiographs of the cervical spine also may be useful in the evaluation
of Arnold-Chiari malformations and should be included in the
evaluation of all patients with hypnic headache.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of hypnic
headache is in question. Intraocular pressure should be measured
if glaucoma is suspected.

Differential Diagnosis
Hypnic headache is a clinical diagnosis supported by a combination of clinical history, physical examination, radiography, MRI,
and MRA. Pain syndromes that may mimic hypnic headache
include the uncommon primary headaches benign exertional
headache, ice pick headache, and sexual headache, although the
unique same-time nocturnal occurrence should help the clinician
easily identify the patients symptoms as hypnic headache. The
clinician must consider other types of headache that occur more
frequently at night, including cluster headache and headaches
associated with sleep apnea, nocturnal arterial hypertension, analgesic rebound, and increased intracranial pressure (Table 9-1).
Less commonly, hypnic headache may be confused with trigeminal neuralgia involving the first division of the trigeminal nerve or
demyelinating disease. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by
trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic
neuritis and other motor and sensory abnormalities. The pain of
chronic paroxysmal hemicrania and cluster headache is associated
with redness and watering of the ipsilateral eye, nasal congestion,
and rhinorrhea during the headache. These findings are absent in
hypnic headache. Migraine headache may or may not be associated with painless neurological findings known as aura, but patients
almost always report some systemic symptoms, such as nausea or
photophobia, not typically associated with hypnic headache.

Treatment
Indomethacin and lithium carbonate are the treatments of choice
for hypnic headache, with indomethacin being slightly more
effective for the unilateral form of the syndrome. Indomethacin
at a starting dose of 25 mg daily for 2 days and titrating to 25 mg
three times per day is a reasonable treatment approach. This dose
may be carefully increased up to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer
disease or impaired renal function. Headache specialists have
noted anecdotal reports of a positive response to cyclooxygenase-2
(COX-2) inhibitors in the treatment of benign hypnic headache.
TABLE 9-1

Nocturnal Headaches That May Be Confused with


Hypnic Headache
Cluster headache
Headache associated with sleep apnea
Headache associated with nocturnal arterial hypertension
Headache associated with increased intracranial pressure
Analgesic rebound headache

Lithium carbonate is used in the same manner as in the treatment


of cluster headache and has its basis in use in its proven efficacy in
the treatment of other diseases thought to have a chronobiological
basis, such as cluster headache and bipolar disorders. However,
the therapeutic window of lithium carbonate is small and this
drug should be used with caution. A starting dose of 300 mg at
bedtime may be increased after 48 hours to 300 mg twice per
day. If no side effects are noted after 48 hours, the dose may be
increased again to 300 mg three times per day. Anecdotal reports
exist that gabapentin and pregabalin also may be useful in decreasing the frequency and intensity of attacks of hypnic headache.
Unlike with cluster headache, oxygen inhalation has been ineffective in aborting attacks of hypnic headache once the patient has
been awakened by the pain.

Complications and Pitfalls


Failure to diagnose hypnic headache correctly may put the
patient at risk if an intracranial pathological condition or demyelinating disease (which may rarely mimic the clinical presentation
of hypnic headache) is overlooked. MRI and MRA are indicated
in all patients thought to have hypnic headache. Failure to diagnose glaucoma, which also may cause intermittent ocular pain,
may result in permanent loss of sight.

Clinical Pearls
Any patient presenting with nocturnal headaches should
be taken very seriously. Although statistically most of these
headaches ultimately are proved to be of benign cause, a
few patients have potentially life-threatening disease. The
diagnosis of hypnic headache is made by obtaining a thorough, targeted headache history and performing a careful
physical examination. The clinician must separate patients
with hypnic headache from patients with headaches caused
by an intracranial pathological condition such as tumors
or systemic disease such as nocturnal arterial hypertension. Patients with hypnic headache should have a normal
neurological examination. If the neurological examination is abnormal, the diagnosis of benign hypnic headache
should be discarded and a careful search for the cause of the
patients neurological findings should be undertaken.

SUGGESTED READINGS
Alberti A: Headache and sleep, Sleep Med Rev 10:431437, 2006.
Berciano J, Poca M-A, Garca A, Sahuquillo J: Paroxysmal cervicobrachial hypnicinduced pain in a patient with syringomyelia extending into spinal cord posterior gray horns, J Neurol 254:678681, 2007.
Chen Y-Y, Lirng J-F, Fuh J-L, etal: Primary hypnic headache is associated with
posterior fossa crowdedness: a morphometric MRI study, Cephalalgia 24:
694699, 2004.
Fowler MV, Capobianco DJ, Dodick DW: Headache in the elderly, Semin Pain
Med 2:123128, 2004.
Manni R, Ghiotto N: In Aminoff M, editor: Handbook of clinical neurology, New
York, 2010, Elsevier, pp 469472.
Pascual J: Primary hypnic headache, Curr Pain Headache Rep 9:272276, 2005.
Pascual J, Gonzlez-Mandly A, Martn R, Oterino A: Headaches precipitated by
cough, prolonged exercise or sexual activity: a prospective etiological and clinical study, J Headache Pain 9:259266, 2008.
Waldman SD: Arnold Chiari malformation type I. In Waldman SD, Campbell
RS, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 2728.
Waldman SD: Arnold Chiari malformation type II. In Waldman SD, Campbell
RS, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 2930.

Chapter 10
NUMMULAR HEADACHE

ICD-9 CODE 784.0


ICD-10 CODE

R51

The Clinical Syndrome


Nummular headache is an uncommon chronic headache syndrome characterized by constant localized pain with superimposed paroxysms of stabbing jabs and jolts of mild to moderate
intensity that occur in a coin-shaped localized area of the scalp.
Most commonly located in the parietal region, the pain of nummular headache is unilateral and localized to a single area. It rarely
if ever switches sides. The scalp overlying the area may be tender
to touch and stimulation of the area; for example, the brushing of
hair may exacerbate the pain. Nummular headache occurs slightly
more commonly in women and is generally not seen before the
fourth decade of life, but rare reports of children suffering from
nummular headache sporadically appear in the literature. Nummular headache is also known as coin-shaped headache.

tomography (CT) is a reasonable second choice. Radionuclide


bone scanning and plain radiography are indicated if fracture or
bony abnormality, such as metastatic disease, is considered in the
differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry should be performed if the diagnosis of nummular
headache is in question.

Differential Diagnosis
Nummular headache is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic nummular
headache include chronic paroxysmal hemicranias and jolts and
jabs headache. Trigeminal neuralgia involving the first division
of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic
neuritis and other motor and sensory abnormalities. The pain of
chronic paroxysmal hemicrania lasts much longer than the pain of
nummular headache and is associated with redness and watering
of the ipsilateral eye.

Signs and Symptoms


A patient with nummular headache complains of a unifocal region
of pain and sensitivity most commonly occurring in the vertex
of the parietal region (Figure 10-1). The pain is almost always
unilateral and does not switch sides, although rare reports exist of
bilateral nummular headache. Some patients describe the pain of
nummular headache as a constant dull ache or sensitivity in the
affected area with superimposed paroxysms of lancinating pain.
The pain is chronic, although spontaneous remissions have been
rarely reported. Some patients with nummular headache exhibit
anxiety and depression because the intensity of the associated pain
leads many patients to believe they have a brain tumor.

Testing
Magnetic resonance imaging (MRI) of the brain provides the best
information regarding the cranial vault and its contents. MRI is
highly accurate and helps identify abnormalities that may put the
patient at risk for neurological disasters secondary to intracranial
and brainstem pathological conditions, including tumors and
calvarial lesions (Figure 10-2). Magnetic resonance angiography
(MRA) also may be useful in helping identify aneurysms, which
may be responsible for the patients pain. In patients who cannot undergo MRI, such as patients with pacemakers, computed

Figure 10-1 Patients suffering from nummular headache complain of


unifocal area of pain and scalp sensitivity.

25

26 SECTION 1 Headache and Facial Pain Syndromes

Figure 10-2 Calvarial metastases. A, Abnormal enhancement (arrows) is present within the diplo on this gadolinium-enhanced T1-weighted image.
Expansion of the left parietal bone occurs, affecting the inner table more than the outer table. B, Heterogeneous hyperintensity (arrows) persists within
the calvaria on this T2-weighted image. The right parietal lesion is no longer imaged on this more superior section. (From Edelman RR, Hesselink JR,
Zlatkin MB, Crues JV III, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2005, Saunders.)

Treatment
Nummular headache uniformly responds to treatment with indomethacin. Failure to respond to indomethacin puts the diagnosis
of nummular headache in question. A starting dosage of 25 mg
daily for 2 days and titrating to 25 mg three times a day is a reasonable treatment approach. This dose may be carefully increased
to 150 mg/day. Indomethacin must be used carefully, if at all,
in patients with peptic ulcer disease or impaired renal function.
Anecdotal reports of a positive response to cyclooxygenase-2
(COX-2) inhibitors in the treatment of nummular headache have
been noted in the headache literature, as well as a successful treatment with gabapentin. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound,
such as nortriptyline, which can be started at a single bedtime
dose of 25 mg.

Complications and Pitfalls


Failure to diagnose nummular headache correctly may put the
patient at risk if an intracranial pathological condition or calvarial
disease, which may mimic the clinical presentation of nummular
headache, is overlooked. MRI is indicated in all patients thought
to have nummular headache.

Clinical Pearls
The diagnosis of nummular headache is made by taking a
careful, targeted headache history. Patients with nummular
headache should have a normal neurological examination.
If the results of the neurological examination are abnormal,
the diagnosis of nummular headache should be discarded
and a careful search for the cause of the patients neurological findings should be undertaken.

SUGGESTED READINGS
Cohen GL: Nummular headache: what denomination? Headache 10:14171418,
2005.
Evens RW, Pareja JA: Nummular headache, Headache 45:164165, 2005.
Mathew NT: Indomethacin responsive headache syndromes, Headache J Head
Face Pain 21:147150, 1981.
Pareja JA, Caminero AB, Serra J, etal: Nummular headache: a coin-shaped cephalgia, Neurology 58:16781679, 2002.
Pareja JA, Pareja J, Barriga FJ, etal: Nummular headache: a prospective series of
14 new cases, Headache 44:611614, 2004.
Pareja JA, Pareja J, Yangela J: Nummular headache, trochleitis, supraorbital neuralgia, and other epicranial headaches and neuralgias: the epicranias, J Headache
Pain 4:125131, 2003.

Chapter 11
HEADACHE ASSOCIATED
WITHTEMPORAL ARTERITIS
Signs and Symptoms

ICD-9 CODE 446.5


ICD-10 CODE M31.6
The Clinical Syndrome
As the name suggests, headache associated with temporal arteritis is located primarily in the temples, with secondary pain often
located in the frontal and occipital regions. A disease of the sixth
decade and beyond, temporal arteritis affects whites almost exclusively, and there is a 3:1 female gender predominance. Temporal
arteritis is also known as giant cell arteritis because of the finding of giant multinucleated cells that infiltrate arteries containing
elastin, including the temporal, ophthalmic, and external carotid
arteries (Figure 11-1, A). Approximately half of patients with temporal arteritis also have polymyalgia rheumatica.

Headache is seen in most patients with temporal arteritis. The


headache is located in the temples and is usually continuous. The
character of the headache pain associated with temporal arteritis
is aching and has a mild to moderate level of intensity. A patient
with temporal arteritis also may complain of soreness of the scalp,
making the combing of hair or resting the head on a firm pillow
extremely uncomfortable.
Although temporal headache is present in almost all patients
with temporal arteritis, the finding of intermittent jaw claudication is pathognomonic for the disease (see Figure 11-1, B). In an
elderly patient, jaw pain while chewing should be considered to
be secondary to temporal arteritis until proved otherwise. In the
presence of strong clinical suspicion that the patient has temporal arteritis, immediate treatment with corticosteroids is indicated
(see discussion of treatment). The reason immediate treatment is
needed is the potential for sudden painless deterioration of vision
in one eye secondary to ischemia of the optic nerve.

Temporal artery

External
carotid artery
Ophthalmic
artery

Figure 11-1 A, Temporal arteritis is a disease of the sixth decade that occurs almost exclusively in whites, with a predilection of 3:1 for women.
B, The sine qua non of temporal arteritis is jaw claudication.

27

28 SECTION 1 Headache and Facial Pain Syndromes

In addition to the signs and symptoms mentioned previously,


patients with temporal arteritis experience myalgia and morning
stiffness. Muscle weakness associated with inflammatory muscle
disease and many other collagen-vascular diseases is absent in temporal arteritis, unless the patient has been treated with prolonged
doses of corticosteroids for other systemic disease, such as polymyalgia rheumatica. The patient also may experience nonspecific
systemic symptoms, including malaise, weight loss, night sweats,
and depression.
On physical examination, a swollen, indurated, nodular temporal artery is present. Diminished pulses are often noted, as is
tenderness to palpation. Scalp tenderness to palpation is often
seen. Funduscopic examination may reveal a pale, edematous
optic disc. The patient with temporal arteritis often appears
chronically ill, depressed, or both.

Testing
Erythrocyte sedimentation rate should be obtained in all patients
suspected to have temporal arteritis. In temporal arteritis, the
erythrocyte sedimentation rate is greater than 50 mm/hr in more
than 90% of patients. Less than 2% of patients with biopsy-proved
temporal arteritis have normal erythrocyte sedimentation rates.
Ideally, the blood for the erythrocyte sedimentation rate should
be obtained before beginning corticosteroid therapy because the
initial level of elevation of this test is useful not only to help diagnose the disease but also as a mechanism to establish the efficacy of
therapy. The erythrocyte sedimentation rate is a nonspecific test,
and other diseases that may manifest clinically in a manner similar
to that of temporal arteritis, such as malignancy or infection, also
may markedly elevate the erythrocyte sedimentation rate. Confirmation of the clinical diagnosis of temporal arteritis requires a
temporal artery biopsy.
Given the simplicity and safety of temporal artery biopsy, it
probably should be performed on all patients suspected of having temporal arteritis. The presence of an inflammatory infiltrate
with giant cells in the biopsied artery is characteristic of the disease. Edema of the intima and disruption of the internal elastic
lamina strengthen the diagnosis. A small percentage of patients
with clinical signs and symptoms strongly suggestive of temporal arteritis who also exhibit a significantly elevated erythrocyte
sedimentation rate have a negative temporal artery biopsy result.
As mentioned, in the presence of a strong clinical impression that
the patient has temporal arteritis, an immediate blood sample for
erythrocyte sedimentation rate testing should be obtained and the
patient started on corticosteroids. Complete blood cell count and
automated chemistries, including thyroid testing, are indicated in
all patients with suspected temporal arteritis to help rule out other
systemic disease that may mimic the clinical presentation of temporal arteritis.
If the diagnosis of temporal arteritis is in doubt, magnetic resonance imaging (MRI) of the brain provides the best information
regarding the cranial vault and its contents. MRI is highly accurate
and helps identify abnormalities that may put the patient at risk
for neurological disasters secondary to intracranial and brainstem
pathological conditions, including tumors and demyelinating disease. More important, MRI helps identify bleeding associated with
leaking intracranial aneurysms. Magnetic resonance angiography
(MRA) may be useful to help identify aneurysms responsible for

neurological symptoms. In patients who cannot undergo MRI,


such as patients with pacemakers, computed tomography (CT)
is a reasonable second choice. If intracranial hemorrhage is suspected, lumbar puncture should be performed, even if blood is
not present on MRI or CT. Intraocular pressure should be measured if glaucoma is suspected.

Differential Diagnosis
Headache associated with temporal arteritis is a clinical diagnosis
supported by a combination of clinical history, abnormal findings on physical examination of the temporal artery, normal radiography, MRI findings, an elevated erythrocyte sedimentation
rate, and a positive temporal artery biopsy result. Pain syndromes
that may mimic temporal arteritis include tension type of headache, brain tumor, other forms of arteritis, trigeminal neuralgia
involving the first division of the trigeminal nerve, demyelinating
disease, migraine headache, cluster headache, and chronic paroxysmal hemicrania. Trigeminal neuralgia involving the first division
of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic
neuritis and other motor and sensory abnormalities. The pain of
chronic paroxysmal hemicrania and cluster headache is associated
with redness and watering of the ipsilateral eye, nasal congestion,
and rhinorrhea during the headache. These findings are absent in
all types of sexual headache. Migraine headache may or may not
be associated with painless neurological findings known as aura,
but the patient almost always reports some systemic symptoms,
such as nausea or photophobia, not typically associated with the
headache of temporal arteritis.

Treatment
The mainstay of treatment for temporal arteritis and its associated headaches and other systemic symptoms is the immediate use
of corticosteroids. If visual symptoms are present, an initial dose
of 80 mg of prednisone is indicated. This dose should be continued until the symptoms of temporal arteritis have completely
abated. At this point, the dose may be decreased by 5 mg/wk as
long as the symptoms remain quiescent and the erythrocyte sedimentation rate does not increase. Cytoprotection of the stomach
mucosa should be considered because ulceration and gastrointestinal bleeding are possible. If the patient cannot tolerate corticosteroids, or the maintenance dose of steroids remains so high as to
produce adverse effects, azathioprine is a reasonable next choice.

Complications and Pitfalls


Failure to recognize, diagnose, and treat temporal arteritis
promptly may result in the permanent loss of vision. Failure to
diagnose the headache associated with temporal arteritis correctly
may put the patient at risk if an intracranial pathological condition or demyelinating disease (which may mimic the clinical
presentation of temporal arteritis) is overlooked. MRI of the brain
is indicated in all patients thought to have headaches associated
with temporal arteritis. Failure to diagnose glaucoma, which also
may cause intermittent ocular pain, may result in permanent loss
of sight.

11 Headache Associated with Temporal Arteritis 29

Clinical Pearls
The diagnosis of headache associated with temporal arteritis
is made by obtaining a thorough, targeted headache history. As mentioned, jaw claudication is pathognomonic for
temporal arteritis, and its presence should be sought in all
elderly patients presenting with headache. Failure to recognize, diagnose, and treat temporal arteritis promptly may
result in the permanent loss of vision.

SUGGESTED READINGS
Hazelman BL: Polymyalgia rheumatica. In Waldman SD, editor: Pain management, Philadelphia, 2009, Saunders, pp 449454.
Paget SA, Spiera RF: Polymyalgia rheumatica and temporal arteritis. In Goldman L,
Ausiello D, editors: Cecil medicine, 23rd ed, Philadelphia, 2007, Saunders, pp
11231127.
Waldman SD: Connective tissue diseases. In Waldman SD, editor: Pain review,
Philadelphia, 2009, Saunders, pp 431448.
Waldman SD: Temporal arteritis. In Waldman SD, editor: Pain review, Philadelphia, 2009, Saunders, pp 222223.

Chapter 12
POSTDURAL PUNCTURE
HEADACHE

ICD-9 CODE 349.0


ICD-10 CODE

G97.1

The Clinical Syndrome


When the dura is intentionally or accidentally punctured, the
potential for headache exists. The clinical presentation of post
dural puncture headache is classic and makes the diagnosis
straightforward if considering this diagnostic category of headache. The diagnosis may be obscured if the clinician is unaware
that dural puncture may have occurred or in the rare instance
when this type of headache occurs spontaneously after a bout of
sneezing or coughing. The symptoms and rare physical findings
associated with postdural puncture headache are due to low cerebrospinal fluid pressure resulting from continued leakage of spinal
fluid out of the subarachnoid space.
The symptoms of postdural puncture headache begin almost
immediately after the patient moves from a horizontal to an
upright position. The intensity peaks within 1 or 2 minutes and
abates within several minutes of the patient again assuming the
horizontal position. The headache is pounding in character, and
its intensity is severe, with the intensity increasing the longer the
patient remains upright. The headache is almost always bilateral
and located in the frontal, temporal, and occipital regions. Nausea
and vomiting and dizziness frequently accompany the headache
pain, especially if the patient remains upright for long periods. If
cranial nerve palsy occurs, visual disturbance may occur.

Signs and Symptoms


The diagnosis of postdural puncture headache is most often
made on the basis of clinical history rather than physical findings
on examination. The neurological examination in most patients
suffering from postdural puncture headache is normal. If the
spinal fluid leak is allowed to persist, or if the patient remains in
the upright position for long periods despite the headache, cranial
nerve palsies may occur, with the sixth cranial nerve affected most
commonly. This complication may be transient, but may become
permanent, especially in patients with vulnerable nerves, such as
those with diabetes. If the neurological examination is abnormal,
other causes of headache should be considered, including subarachnoid hemorrhage.
The onset of headache pain and other associated symptoms
such as nausea and vomiting that occurs when the patient moves
30

from the horizontal to the upright position and then abates when
the patient resumes a horizontal position is the sine qua non of
postdural puncture headache (Figure 12-1). A history of intentional dural puncture, such as lumbar puncture, spinal anesthesia, or myelography, or accidental dural puncture, such as failed
epidural block or dural injury during spinal surgery, strongly
points to the diagnosis of postdural puncture headache. As mentioned, a spontaneous postural headache that manifests identically
to headache after dural puncture can occur after bouts of heavy
sneezing or coughing and is thought to be due to traumatic rents
in the dura. In this setting, a diagnosis of postdural puncture
headache is one of exclusion.

Testing
Magnetic resonance imaging (MRI) with and without gadolinium
is highly accurate in helping confirm the diagnosis of postdural
puncture headache. Enhancement of the dura with low-lying
cerebellar tonsils invariably is present. Poor visualization of the
cisterns and subdural and epidural fluid collections also may be
identified.
No additional testing is indicated for a patient who has undergone dural puncture and then develops a classic postural headache, unless infection or subarachnoid hemorrhage is suspected.
In this setting, lumbar puncture, complete blood cell count, and
erythrocyte sedimentation rate are indicated on an emergent basis.

Differential Diagnosis
If the clinician is aware that the patient has undergone dural
puncture, the diagnosis of postdural puncture headache is usually made. Delayed diagnosis most often occurs in settings in
which dural puncture is not suspected. Occasionally, postdural
puncture headache is misdiagnosed as migraine headache because
of the associated nausea and vomiting coupled with visual disturbance. In any patient with dural puncture, infection remains
an ever-present possibility. If fever is present, immediate lumbar
puncture and blood cultures should be obtained and the patient
started on antibiotics that cover resistant strains of Staphylococcus. MRI to rule out epidural abscess also should be considered
if fever is present. Subarachnoid hemorrhage may mimic post
dural puncture headache, but should be identified on MRI of
the brain.

Treatment
The mainstay of treatment of postdural puncture headache is
the administration of autologous blood into the epidural space.

12 PostDural Puncture Headache 31

Cauda equina
Dura mater

Figure 12-1 The onset of headache that occurs when the patient moves from the horizontal to the upright position is the sine qua non of postdural
puncture headache.

This technique is known as epidural blood patch and is highly


successful in the treatment of postdural puncture headache. A
volume of 12 to 18 mL of autologous blood is injected slowly
into the epidural space at the level of dural puncture under
strict aseptic precautions. The patient should remain in the
horizontal position for the next 12 to 24 hours. Relief occurs
within 2 to 3 hours in more than 90% of patients. Approximately 10% of patients experience temporary relief and then a
recurrence of symptoms when assuming the upright position.
These patients should undergo a second epidural blood patch
within 24 hours.
If the patient has experienced significant nausea and vomiting,
antiemetics combined with intravenous fluids help speed recovery. Some clinicians have advocated the use of alcoholic beverages
to suppress the secretion of antidiuretic hormone and increase
cerebrospinal fluid production. Caffeine also has been reported to
be helpful in treating the headache pain.

Complications and Pitfalls


Failure to recognize, diagnose, and treat postdural puncture
headache promptly may result in considerable pain and suffering
for the patient. If the low cerebrospinal fluid pressure is allowed
to persist, cranial nerve deficits may occur. In most instances, the
cranial nerve deficits are temporary, but in rare instances, these
deficits may become permanent, especially in patients with vulnerable nerves, such as those with diabetes. MRI of the brain is
indicated in all patients thought to be suffering from headaches

associated with dural puncture. Failure to diagnose central nervous system infection correctly can result in significant mortality
and morbidity.

Clinical Pearls
The diagnosis of postdural puncture headache is made by
obtaining a thorough, targeted headache history and performing a careful physical examination. The postural nature
is pathognomonic for postdural puncture headache, and
its presence should lead the clinician to strongly consider
the diagnosis of postdural puncture headache. The incidence of postdural puncture headache after lumbar puncture, myelography, or spinal anesthesia can be decreased
by using needles with a smaller diameter and placing the
needle bevel parallel to the dural fibers. Special noncutting
needles may decrease further the incidence of postdural
puncture headache.

SUGGESTED READINGS
Ghaleb A, Pablo C, Mandoff VL, Albataniah J, Candido K: Postdural puncture
cephalgia, Semin Pain Med 2:215219, 2004.
Harrington BE: Postdural puncture headache, Adv Anesth 28:121146, 2010.
Neal JM: Update on postdural puncture headache, Tech Reg Anesth Pain Manage
2:202210, 1998.
Waldman SD, Feldstein GS, Allen ML: Cervical epidural blood patch for treatment
of cervical dural puncture headache, Anesth Rev 14:2325, 1987.

Chapter 13
RAMSAY HUNT SYNDROME

ICD-9 CODE 053.11


ICD-10 CODE B02.21
The Clinical Syndrome
Ramsay Hunt syndrome is the eponym given to acute herpes zoster involvement of the geniculate ganglion. The syndrome results
from reactivation of the varicella-zoster virus (VZV) within the
geniculate ganglion. VZV is also the causative agent of chickenpox (varicella). Primary infection in the nonimmune host manifests itself clinically as the childhood disease chickenpox. It is
postulated that during the course of primary infection with VZV,
the virus invades the geniculate ganglia. The virus remains dormant in the ganglia, producing no clinically evident disease. In
some individuals, the dormant virus reactivates and travels along
the pathways of the geniculate ganglion, producing the pain and
skin lesions characteristic of shingles. The reason that reactivation
occurs in only some individuals is not fully understood, but it is
theorized that a decrease in cell-mediated immunity may play an
important role in the evolution of this disease by allowing the
virus to multiply in the ganglia and spread to the corresponding
sensory nerves, producing clinical disease. Patients with malignancies (particularly lymphoma), patients who are receiving immunosuppressive therapy (chemotherapy, steroids, radiation), and
patients with chronic diseases are generally debilitated and much
more likely than healthy individuals to develop acute herpes zoster. These patients all have in common a decreased cell-mediated
immune response, which may be the reason for their propensity
to develop shingles. This decreased immune response may also
explain why the incidence of shingles increases dramatically in
individuals older than 60 years and is uncommon in individuals
younger than age 20.
The first division of the trigeminal nerve is the second most
common site for the development of acute herpes zoster after the
thoracic dermatomes. Rarely, the virus may attack the geniculate
ganglion, resulting in facial pain, hearing loss, vertigo, vesicles in
the ear, and pain. This constellation of symptoms is called Ramsay Hunt syndrome and must be distinguished from acute herpes
zoster involving the first division of the trigeminal nerve.

of the geniculate ganglion. This pain may be accompanied by flulike symptoms and generally progresses from a dull, aching sensation to dysesthetic neuritic pain in the distribution of the geniculate
ganglion. In most patients, the pain of acute herpes zoster precedes
the eruption of rash by 3 to 7 days, often leading to erroneous diagnosis (see discussion of differential diagnosis). The clinical diagnosis of shingles is readily made, however, in most patients when
the characteristic rash appears. Similar to chickenpox, the rash of
herpes zoster appears in crops of macular lesions, which rapidly
progress to papules and then to vesicles (Figure 13-1).
As the disease progresses, the vesicles coalesce, and crusting
occurs. The area affected by the disease can be extremely painful,
and the pain tends to be exacerbated by any movement or contact
(e.g., with clothing or sheets). As healing occurs, the crusts fall
away, leaving pink scars in the distribution of the rash that gradually become hypopigmented and atrophic.

Vesicles in ear

Signs and Symptoms


As viral reactivation occurs, ganglionitis and peripheral neuritis
cause pain, which is generally localized to the segmental distribution
32

Figure 13-1 Ramsay Hunt syndrome results from infection of the geniculate ganglion by the varicella-zoster virus.

13 Ramsay Hunt Syndrome 33

In most patients, the hyperesthesia and pain generally resolve as


the skin lesions heal. In some patients, pain and neurological findings may persist beyond lesion healing (Figure 13-2). This most
common and feared complication of acute herpes zoster is postherpetic neuralgia. Elderly patients are affected at a higher rate than
the general population suffering from acute herpes zoster. The
symptoms of postherpetic neuralgia can vary from a mild, selflimited problem to a debilitating, constantly burning pain exacerbated by light touch, movement, anxiety, or temperature change.
This unremitting pain may be so severe that it completely devastates the patients life, even leading ultimately to suicide. To avoid
these disastrous sequelae to a usually benign self-limited disease,
the clinician must use all possible therapeutic efforts for the patient
suffering from acute herpes zoster in the geniculate ganglion.

Testing
Although in most instances the diagnosis of acute herpes zoster involving the geniculate ganglion is easily made on clinical grounds, confirmatory testing is occasionally required. Such
testing may be desirable in patients with other skin lesions that
confuse the clinical picture, such as patients with acquired immunodeficiency syndrome who have Kaposis sarcoma. In such
patients, the diagnosis of acute herpes zoster may be confirmed by
obtaining a Tzanck smear from the base of a fresh vesicle, which
reveals multinucleated giant cells and eosinophilic inclusions. To
differentiate acute herpes zoster from localized herpes simplex
infection, the clinician can obtain fluid from a fresh vesicle and
submit it for immunofluorescent testing.

Figure 13-2 Neurological examination revealed a flattened right nasolabial fold (black arrows) and ptosis of the right angle of the mouth (white
arrow). (From Taguchi T, Ueda S, Kudo T, etal: Ramsay-Hunt syndrome,
J Infect 62:180181, 2011.)

Differential Diagnosis
Careful initial evaluation, including a thorough history and physical examination, is indicated in all patients suffering from acute
herpes zoster involving the geniculate ganglion to rule out occult
malignancy or systemic disease that may be responsible for the
patients immunocompromised state and allow early recognition
of changes in clinical status that may presage the development of
complications, including myelitis or dissemination of the disease.
Other causes of pain in the distribution of the geniculate ganglion
include trigeminal neuralgia, sinus disease, glaucoma, retroorbital
tumors, inflammatory diseases such as Tolosa-Hunt syndrome,
and intracranial pathology, including tumors.

Treatment
The therapeutic challenge of a patient with acute herpes zoster
involving the geniculate ganglion is twofold: (1) to provide immediate relief of acute pain and symptoms and (2) to prevent complications, including postherpetic neuralgia. It is the consensus
of most pain specialists that the earlier in the natural course of
the disease that treatment is initiated, the less likely it is that the
patient will develop postherpetic neuralgia. Because older patients
are at highest risk for developing postherpetic neuralgia, early
aggressive treatment of these patients is mandatory.
Nerve Blocks
Sympathetic neural blockade with local anesthetics and steroids
via stellate ganglion block seems to be the treatment of choice to
relieve the symptoms of acute herpes zoster involving the geniculate ganglion and to prevent the occurrence of postherpetic neuralgia. Sympathetic nerve block is thought to achieve these goals
by blocking the profound sympathetic stimulation that results
from the viral inflammation of the nerve and geniculate ganglion.
If untreated, this sympathetic hyperactivity can cause ischemia
secondary to decreased blood flow of the intraneural capillary bed.
If this ischemia is allowed to persist, endoneural edema forms,
increasing endoneural pressure and causing a further reduction of
endoneural blood flow with irreversible nerve damage.
As vesicular crusting occurs, the addition of steroids to the
local anesthetic may decrease neural scarring and decrease further
the incidence of postherpetic neuralgia. These sympathetic blocks
should be continued aggressively until the patient is pain free and
should be reimplemented at the return of pain. Failure to use sympathetic neural blockade immediately and aggressively, especially
in elderly patients, may sentence the patient to a lifetime of suffering from postherpetic neuralgia. Occasionally, some patients suffering from acute herpes zoster involving the geniculate ganglion
may not experience pain relief from stellate ganglion block, but
they do respond to blockade of the trigeminal nerve.
Opioid Analgesics
Opioid analgesics may be useful in relieving the aching pain
that is often present during the acute stages of herpes zoster as
sympathetic nerve blocks are being implemented. They are less
effective in the relief of the neuritic pain that is often present.
Careful administration of potent, long-acting opioid analgesics
(e.g., oral morphine elixir or methadone) on a time-contingent
rather than as-needed basis may represent a beneficial adjunct to
the pain relief provided by sympathetic neural blockade. Because
many patients with acute herpes zoster are elderly or may have
severe multisystem disease, close monitoring for the potential

34 SECTION 1 Headache and Facial Pain Syndromes

side effects of potent opioid analgesics (e.g., confusion or dizziness, which may cause a patient to fall) is warranted. Daily
dietary fiber supplementation and Milk of Magnesia should be
started, along with opioid analgesics to prevent the side effect of
constipation.
Adjuvant Analgesics
The anticonvulsant gabapentin represents a first-line treatment in
the palliation of neuritic pain of acute herpes zoster involving the
geniculate ganglion. Studies suggest that gabapentin also may help
prevent the development of postherpetic neuralgia. Treatment with
gabapentin should begin early in the course of the disease, and this
drug may be used concurrently with neural blockade, opioid analgesics, and other adjuvant analgesics, including the antidepressant
compounds if care is taken to avoid central nervous system side
effects. Gabapentin is started at a bedtime dose of 300 mg and is
titrated in 300-mg increments to a maximum dose of 3600 mg
given in divided doses as side effects allow. Carbamazepine should
be considered in patients with severe neuritic pain who have failed
to respond to nerve blocks and gabapentin. If this drug is used, rigid
monitoring for hematological parameters is indicated, especially in
patients receiving chemotherapy or radiation therapy. Phenytoin
also may be beneficial to treat neuritic pain, but it should not be
used in patients with lymphoma because the drug may induce a
pseudolymphoma state that is difficult to distinguish from the
actual lymphoma itself.
Antidepressants also may be useful adjuncts in the initial treatment of patients with acute herpes zoster. On an acute basis, these
drugs help alleviate the significant sleep disturbance that is commonly seen with acute herpes zoster. In addition, antidepressants
may be valuable in helping ameliorate the neuritic component of
the pain, which is treated less effectively with opioid analgesics.
After several weeks of treatment, the antidepressants may exert
a mood-elevating effect that may be desirable in some patients.
Patients must be observed closely for central nervous system side
effects. These drugs may cause urinary retention and constipation
that may be mistakenly attributed to herpes zoster myelitis.

cannot or will not undergo sympathetic neural blockade and do


not tolerate pharmacological interventions.
Topical application of aluminium sulfate as a tepid soak provides excellent drying of the crusting and weeping lesions of acute
herpes zoster, and most patients find these soaks soothing. Zinc
oxide ointment also may be used as a protective agent, especially
during the healing phase when temperature sensitivity is a problem. Disposable diapers can be used as an absorbent padding to
protect healing lesions from contact with clothing and sheets.

Complications and Pitfalls


In most patients, acute herpes zoster involving the geniculate ganglion is a self-limited disease. In elderly and immunosuppressed
patients, complications may occur, however. Cutaneous and visceral dissemination may range from a mild rash resembling chickenpox to an overwhelming, life-threatening infection in patients
already suffering from severe multisystem disease. Myelitis may
cause bowel, bladder, and lower extremity paresis. Ocular complications from trigeminal nerve involvement may range from severe
photophobia to keratitis with loss of sight.

Clinical Pearls
Because the pain of herpes zoster usually precedes the eruption of skin lesions by 5 to 7 days, erroneous diagnosis of
other painful conditions (e.g., trigeminal neuralgia, glaucoma) may be made. In this setting, the astute clinician
advises the patient to call immediately if a rash appears
because the diagnosis of acute herpes zoster is a possibility.
Some pain specialists believe that in a few immunocompetent patients, when reactivation of virus occurs, a rapid
immune response may attenuate the natural course of the
disease and the characteristic rash of acute herpes zoster may
not appear. This pain in the distribution of the geniculate
ganglion without associated rash is termed zoster sine herpete
and is, by necessity, a diagnosis of exclusion. Other causes
of head pain must be ruled out first before invoking this
diagnosis. Because of the potential for hearing loss in Ramsay Hunt syndrome, patients should be warned of this possibility to avoid erroneously blaming this complication on
a therapeutic intervention, such as stellate ganglion block.

Antiviral Agents
A few antiviral agents, including famciclovir and acyclovir, have
been shown to shorten the course of, and may help prevent the
development of, acute herpes zoster. They are probably useful
in attenuating the disease in patients with immunosuppression.
These antiviral agents can be used in conjunction with the treatment modalities mentioned earlier. Careful monitoring for side
effects is mandatory with these drugs.

SUGGESTED READINGS

Adjunctive Treatments
The application of ice packs to the lesions of acute herpes zoster
may provide relief in some patients. Application of heat increases
pain in most patients, presumably because of increased conduction of small fibers, but it is beneficial occasionally and may be
worth trying if application of cold is ineffective. Transcutaneous
electrical nerve stimulation and vibration also may be effective in
a few patients. The favorable risk-to-benefit ratio of all of these
modalities makes them reasonable alternatives for patients who

Bhagra A, Stead LG: Ramsay Hunt syndrome: a rare entity, Ann Emerg Med
47:579, 2006.
Gantz BJ, Redleaf MI, Perry BP, Gubbels SP: Management of Bells palsy and
Ramsay Hunt syndrome. In Brackmann DE, etal: Otologic surgery, ed 3, Philadelphia, 2010, Saunders, pp 335346.
Persson A, Bergstrm T, Lindh M, Namvar L, Studahl M: Varicella-zoster virus
CNS disease: viral load, clinical manifestations and sequels, J Clin Virol 46:249
253, 2009.
Taguchi T, Ueda S, Kudo T, etal: Ramsay-Hunt syndrome, J Infect 62:180181,
2011.
Ulusoy , zkan G, Bekta D, etal: Ramsay Hunt syndrome in renal transplantation recipient: a case report, Transplant Proc 42:19861988, 2010.

Chapter 14
EAGLE SYNDROME

ICD-9 CODE 756.71


ICD-10 CODE M62.89

The Clinical Syndrome


An uncommon cause of facial pain, Eagle syndrome (also known
as stylohyoid syndrome) is caused by pressure on the internal
carotid artery and surrounding structures, including branches of
the glossopharyngeal nerve, by an abnormally elongated styloid
process, a calcified stylohyoid ligament, or both. The pain of Eagle
syndrome is sharp and stabbing and occurs with movement of the
mandible or turning of the neck. The pain starts below the angle
of the mandible and radiates into the tonsillar fossa, temporomandibular joint, and base of the tongue. A trigger point may be
present in the tonsillar fossa. Injection of the attachment of the
stylohyoid ligament to the styloid process with local anesthetic
and steroid serves as a diagnostic maneuver and a therapeutic
maneuver.

Signs and Symptoms


Eagle syndrome is most often a diagnosis of exclusion. Patients
suffering from Eagle syndrome present with a history of sudden, sharp neuritic pain that begins below the angle of the
mandible and radiates into the tonsillar fossa, temporomandibular joint, and base of the tongue. The pain is triggered by
swallowing, movement of the mandible, or turning of the neck
(Figure 14-1). The intensity of pain is moderate to severe and
unpleasant. The neurological examination is normal. The pain
of Eagle syndrome may be triggered by palpation of the tonsillar fossa.

Testing
In patients with Eagle syndrome, radiographs and computed
tomography (CT) scans of the region of the styloid process show
an elongated styloid process that is often associated with a calcified stylohyoid ligament. The diagnosis of Eagle syndrome may be
strengthened by a diagnostic injection of the attachment of the stylohyoid ligament to the styloid process with local anesthetic. Pain
relief after this injection suggests a local cause for the pain rather
than a more distant cause, such as glossopharyngeal neuralgia or
retropharyngeal tumor (Figure 14-2).

Differential Diagnosis
Eagle syndrome can be distinguished from glossopharyngeal neuralgia because the pain of glossopharyngeal neuralgia is characterized by paroxysms of shocklike pain in a manner analogous
to trigeminal neuralgia, rather than the sharp, shooting pain on
movement that is associated with Eagle syndrome. Because glossopharyngeal neuralgia may be associated with serious cardiac
bradyarrhythmias and syncope, the clinician must distinguish the
two syndromes.
The clinician should always evaluate a patient with pain in this
anatomical region for occult malignancy. Tumors of the larynx,
hypopharynx, and anterior triangle of the neck may manifest with
clinical symptoms identical to those of Eagle syndrome. Because
of the low incidence of Eagle syndrome relative to pain secondary
to malignancy in this anatomical region, Eagle syndrome must be
considered a diagnosis of exclusion.

Treatment
Many patients with Eagle syndrome respond to a series of therapeutic injections of the attachment of the stylohyoid ligament to the
styloid process with local anesthetic and steroid. To perform this
procedure, an imaginary line is visualized running from the mastoid process to the angle of the mandible (Figure 14-3). The styloid
process should lie just below the midpoint of this line. The skin
is prepared with antiseptic solution. A 22-gauge, 1-inch needle

Temporomandibular joint
Styloid process
Glossopharyngeal nerve

Tongue

Styloid ligament
Internal carotid

Mandible

Figure 14-1 The pain of Eagle syndrome is triggered by swallowing,


movement of the mandible, or turning of the neck.

35

36 SECTION 1 Headache and Facial Pain Syndromes

Figure 14-2 Tumor (T) of the piriform sinus. The lesion protrudes
through the thyroarytenoid gap between thyroid cartilage and arytenoid (arrow). The tumor invades the paraglottic space (arrowhead) of
the supraglottic larynx. Compare with the fat in the paraglottic space on
the normal side. C, Carotid artery. (From Haaga JR, Lanzieri CF, Gilkeson
RC, editors: CT and MR imaging of the whole body, 4th ed, Philadelphia,
2003, Mosby, p 611.)

attached to a 14-mL syringe is advanced at this midpoint location


in a plane perpendicular to the skin. The styloid process should be
encountered within 3 cm. After contact is made, the needle is withdrawn slightly out of the periosteum or substance of the calcified ligament. After careful aspiration reveals no blood or cerebrospinal fluid,
5 mL of 0.5% preservative-free lidocaine combined with 80 mg
of methylprednisolone is injected in incremental doses. Subsequent
daily nerve blocks are performed in a similar manner, substituting
40 mg of methylprednisolone for the initial 80-mg dose.
The sharp, shooting pain associated with Eagle syndrome also
may be treated with gabapentin. Gabapentin is started at a single
nighttime dose of 300 mg and titrated by 300-mg increments
every 2 days in divided doses until pain relief is achieved or a total
daily dose of 3600 mg is reached. Alternatively, carbamazepine or
phenytoin may be tried.

Figure 14-3 An imaginary line from the mastoid process to the angle
of the mandible is an aid in needle placement for injection in a patient
with Eagle syndrome.

blocked, dysphonia secondary to paralysis of the ipsilateral vocal


cord may occur. A reflex tachycardia secondary to vagal nerve
block also is observed in some patients. Inadvertent block of the
hypoglossal and spinal accessory nerves during glossopharyngeal
nerve block results in weakness of the tongue and trapezius muscle.

Clinical Pearls
Eagle syndrome is an uncommon cause of facial pain.
Because of the low incidence of Eagle syndrome relative
to pain secondary to malignancy in this anatomical region,
Eagle syndrome must be considered a diagnosis of exclusion. The clinician should always evaluate a patient with
pain in this anatomical region for occult malignancy.
Tumors of the larynx, hypopharynx, and anterior triangle
of the neck may manifest with clinical symptoms identical
to those of Eagle syndrome.

Complications and Pitfalls

SUGGESTED READINGS

The major complications associated with this injection technique


are related to trauma to the internal jugular and carotid artery.
Hematoma formation and intravascular injection of local anesthetic with subsequent toxicity are common complications of this
technique. Inadvertent blockade of the motor portion of the glossopharyngeal nerve can result in dysphagia secondary to weakness
of the stylopharyngeus muscle. If the vagus nerve is inadvertently

Blythe JN, Matthews NS, Connor S: Eagles syndrome after fracture of the elongated styloid process, Br J Oral Maxillofac Surg 47:233235, 2009.
Callahan B, Kang J, Dudekula A, Eusterman V, Rabb CH: New Eagles syndrome
variant complicating management of intracranial pressure after traumatic brain
injury, Injury Extra 41:4144, 2010.
Johnson GM, Rosdy NM, Horton SJ: Manual therapy assessment findings in patients
diagnosed with Eagles syndrome: a case series, Man Ther 16:199202, 2011.
Klcha A, Hafian H, Devauchelle B, Lefvre B: A report of post-traumatic Eagles
syndrome, Int J Oral Maxillofac Surg 37:970972, 2008.

Chapter 15
ATYPICAL ODONTALGIA

ICD-9 CODE 525.9


ICD-10 CODE K08.9
The Clinical Syndrome
Atypical odontalgia (also known as persistent orodental pain syndrome) describes a heterogeneous group of pain syndromes that
share in common the fact that the odontalgia cannot be classified
as classic trigeminal neuralgia. The pain is continuous but may
vary in intensity. It is almost always unilateral and may be characterized as aching or cramping rather than the shocklike neuritic
pain typical of trigeminal neuralgia. The vast majority of patients
suffering from atypical odontalgia are female. Atypical odontalgia
can occur at any age, but has a peak incidence in the fifth decade
of life. The pain is felt in a single tooth or its surrounding area
and occurs most commonly in the maxillary region (Figure 15-1).
Headache may occur with atypical odontalgia and is clinically
indistinguishable from the tension type of headache. Stress is often
the precipitating, or an exacerbating, factor in the development
of atypical odontalgia. Depression and sleep disturbance are also
present in a significant number of patients. A history of dental or
facial trauma, including dental extractions, root canal treatment,
infection, or tumor of the head and neck may be elicited in some
patients with atypical odontalgia, but in many cases no precipitating event can be identified.

to identify a tumor or bony abnormality. Magnetic resonance


imaging (MRI) of the brain and sinuses can help the clinician
identify intracranial pathology such as tumor, sinus disease, and
infection (Figure 15-2). A complete blood count, erythrocyte sedimentation rate, and antinuclear antibody testing are indicated if
inflammatory arthritis or temporal arteritis is suspected. Injection
of the painful tooth with small amounts of local anesthetic can
serve as a diagnostic maneuver to determine whether the tooth or
adjacent structures are the source of the patients pain. Differential neural blockade can help distinguish primary tooth pathology
from atypical odontalgia and reflex sympathetic dystrophy of the
face (Table 15-2). Complete relief of pain after injection of the
painful tooth with local anesthetic suggests a local pathological
process, whereas incomplete pain relief suggests the pathological
process is more central. Thus the diagnosis of atypical odontalgia
is a strong possibility of underlying pathological condition of the
trigeminal nerve, adjacent bone, brain, or brainstem. Complete
relief of pain after ipsilateral stellate ganglion block is highly suggestive of reflex sympathetic dystrophy of the face. Psychological

Signs and Symptoms


Table 15-1 compares atypical odontalgia with trigeminal neuralgia.
Unlike trigeminal neuralgia, which is characterized by sudden paroxysms of neuritic shocklike pain, atypical odontalgia is constant
and has a dull, aching quality but may vary in intensity. The pain of
trigeminal neuralgia is almost always within the distribution of one
division of the trigeminal nerve, whereas atypical odontalgia invariably involves just a single tooth, its surrounding gingival tissue, or
underlying bone. The trigger areas characteristic of trigeminal neuralgia are absent in patients with atypical odontalgia. Most important, no findings of pathological condition of the painful tooth or
adjacent gingival tissues are seen on physical examination.

Testing
Radiographs of the head are usually within normal limits in
patients suffering from atypical odontalgia, but they may be useful

Figure 15-1 Patients with atypical odontalgia often rub the affected
area; those with trigeminal neuralgia do not.

37

38 SECTION 1 Headache and Facial Pain Syndromes


TABLE 15-1

Comparison of Trigeminal Neuralgia and Atypical


Odontalgia
Atypical
Odontalgia

Pain Factor

Trigeminal Neuralgia

Temporal pattern
of pain

Sudden and
intermittent

Constant

Character of pain

Shocklike and neuritic

Dull, aching,
cramping

Pain-free interval

Usual

Rare

Distribution
of pain

One division of the


trigeminal nerve

One tooth and


surrounding area

Trigger areas

Present

Uncommon

Underlying
psychopathology

Rare

Common

evaluation should be considered if significant coexistent depression or sleep disturbance is present.

Differential Diagnosis
The clinical symptoms of atypical odontalgia may be confused
with pain of dental or sinus origin or may be erroneously characterized as trigeminal neuralgia. Careful questioning and physical examination usually allow the clinician to distinguish these
overlapping pain syndromes. Tumors of the zygoma, maxilla, and
mandible, as well as posterior fossa and retropharyngeal tumors,
may produce ill-defined pain that is attributed to atypical odontalgia. These potentially life-threatening diseases must be excluded in
any patient with odontalgia (see Figure 15-2). Reflex sympathetic
dystrophy of the face should also be considered in any patient with
ill-defined odontalgia after trauma, infection, or central nervous

Figure 15-2 Computed tomography (CT) scan and magnetic resonance imaging (MRI) of the lesion. CT shows a well-defined expansile lesion with
thin cortical margin and high-density area (A). MRI of the lesion revealed the well-circumscribed lesion (B) to be homogeneously and relatively
hypointense on T2-weighted imaging (C). The lesion was weakly enhanced by gadolinium (D). (From Nozaki S, Yamazaki M, Koyama T, etal: Primary
extracranial meningioma of the maxillary sinus presenting as buccal swelling, Asian J Oral Maxillofac Surg 23:134137, 2011.)

15 Atypical Odontalgia 39
TABLE 15-2

Differential Nerve Block in the Diagnosis of Atypical


Odontalgia
1. Record the patients pain level on a visual analogue scale of 0 to 10.
2. Isolate the painful area with cotton rolls and cheek retractor.
3. Dry the painful area with gauze.
4. Apply 20% topical benzocaine gel to the painful area.
5. Record the patients pain level on a visual analogue scale of
0 to 10 every 3 minutes for 15 minutes.
6. If the patient experiences incomplete pain relief, perform localized block of the painful tooth with 1% lidocaine 1.5 mL.
7. Record the patients pain level on a visual analogue scale of
0 to 10 every 3 minutes for 15 minutes.
8. If the patient experiences incomplete relief, perform ipsilateral
stellate ganglion block with 0.5% preservative-free lidocaine
7 to 10 mL.
9. Record the patients pain level on a visual analogue scale of
0 to 10 every 3 minutes for 15 minutes.
10. Repeat this sequence on a separate visit to confirm the results.

system injury. As noted, atypical odontalgia is dull and aching,


whereas reflex sympathetic dystrophy of the face causes burning
pain and significant allodynia is often present. Stellate ganglion
block may help distinguish these two pain syndromes; the pain of
reflex sympathetic dystrophy of the face readily responds to this
sympathetic nerve block, whereas atypical odontalgia does not.
Atypical odontalgia must also be distinguished from the pain of
jaw claudication associated with temporal arteritis.

Treatment
The mainstay of therapy is a combination of drug treatment with
tricyclic antidepressants and physical modalities such as oral orthotic
devices and physical therapy. Trigeminal nerve block and intraarticular injection of the temporomandibular joint with small amounts
of local anesthetic and steroid also may be of value. Antidepressants
such as nortriptyline at a single bedtime dose of 25 mg can help
alleviate sleep disturbance and treat any underlying myofascial pain
syndrome. Orthotic devices help the patient avoid jaw clenching
and bruxism, which may exacerbate the clinical syndrome. Management of underlying depression and anxiety is also mandatory.

Complications and Pitfalls


The major pitfall in caring for patients thought to have atypical odontalgia is failure to diagnose underlying pathology that
may be responsible for the patients pain. Atypical odontalgia is
essentially a diagnosis of exclusion. If trigeminal nerve block or
intraarticular injection of the temporomandibular joint is being
considered as part of the treatment plan, it must be remembered
that the regions vascularity and proximity to major blood vessels
can lead to an increased incidence of postblock ecchymosis and
hematoma formation, and the patient should be warned of this
potential complication.

Clinical Pearls
Atypical odontalgia requires careful evaluation to design
an appropriate treatment plan. Infection and inflammatory
causes, including collagen-vascular diseases, must be ruled
out. Stress and anxiety often accompany atypical odontalgia, and these factors must be addressed and treated. The
myofascial pain component of atypical odontalgia is best
treated with tricyclic antidepressants such as amitriptyline.
Dental malocclusion and nighttime bruxism should be
treated with an acrylic bite appliance. Opioid analgesics and
benzodiazepines should be avoided in patients with atypical
odontalgia.

SUGGESTED READINGS
Clark GT: Persistent orodental pain, atypical odontalgia, and phantom tooth pain:
when are they neuropathic disorders? J Calif Dent Assoc 34:599609, 2006.
Marbach JJ: Is phantom tooth pain a deafferentation (neuropathic) pain syndrome? Oral Surg Oral Med Oral Pahtol 75:95-105, 1993.
Marbach JJ: Orofacial phantom pain: theory and phenomenology, JADA
127:221229, 1996.
Marbach JJ, Raphael KG: Phantom tooth pain: a new look at an old dilemma,
Pain Med 1:6877, 2000.
Matwychuk MJ: Diagnostic challenges of neuropathic tooth pain, J Can Dent
Assoc 70:542546, 2004.
McQuay HJ, Tramr M, Nye BA, etal: A systematic review of antidepressants in
neuropathic pain, Pain 68:217227, 1996.
Melis M, Lobo SL, Ceneviz C, etal: Atypical odontalgia: a review of the literature, Headache 43:10601074, 2003.
Pertes RA, Bailey DR, Milone AS: Atypical odontalgia: a nondental toothache,
J N J Dent Assoc 66:2931, 33, 1995.

Chapter 16
BURNING MOUTH SYNDROME

ICD-9 CODE 529.6


ICD-10 CODE K14.6
The Clinical Syndrome
Burning mouth syndrome is an infrequent but serious cause
of oral pain. Although mouth pain has many causes with readily demonstrable pathological conditions, such as herpes simplex
infections and aphthous ulcers, burning mouth syndrome is the
diagnosis given to patients who complain of mouth and tongue
pain in the presence of a completely normal physical examination.
Therefore burning mouth syndrome is by definition a diagnosis of
exclusion. Included in the diagnosis of burning mouth syndrome
are the clinical syndromes of burning tongue syndrome, glossalgia, glossodynia, stomatodynia, and oral dysesthesia syndrome.
Affecting females 7 to 8 times more frequently than men, burning
mouth syndrome is a disease of the fifth decade and beyond. The
pain of burning mouth syndrome is characterized as a burning,
hot, or scalded sensation of the mouth and tongue that may be
accompanied by tingling. Most commonly the anterior two thirds
of the tongue, palate, gingiva of the upper and lower alveolar
region, and lips are involved, with the sublingual region less commonly affected. The exact pathophysiology responsible for burning
mouth syndrome remains elusive, and the putative causes in most
cases are multifactorial. Underlying nutritional disorders, psychiatric illness, allergic stomatitis, xerostomia, diabetes mellitus, menopause, and other endocrinopathies are often identified in patients
with burning mouth syndrome, even though the oral examination
is completely negative.

Testing
No specific test exists for burning mouth syndrome, and a presumptive diagnosis can be made only if (1) the clinical examination is normal and (2) a workup for all underlying pathological findings fails
to identify a specific cause for the patients pain symptomatology. A
suggested workup based on the experience at the Mayo Clinic is outlined in Table 16-1 and should always include laboratory testing for
vitamin deficiencies and diabetes and a culture for candida infection.

Differential Diagnosis
Myriad causes of burning mouth and tongue pain have been
identified, many of which are readily treatable (Table 16-2). It is
therefore imperative that the clinician faced with a patient with
burning mouth and tongue pain obtain an extremely thorough
history and perform an oral examination with these diseases in
mind. It should be kept in mind that more often than not the
patient with burning mouth syndrome has more than one pathological condition contributing to the pain, and the possibility of
multiple diagnosis should always be considered.

Treatment
The successful treatment of burning mouth syndrome requires
the clinician to endeavor to identify the underlying pathology

Signs and Symptoms


The hallmark of burning mouth syndrome is mouth and tongue
burning pain in the absence of clinically demonstrable oral pathology. Depressive affect or a phobic preoccupation with occult cancer
is often present, as is xerostomia. The classic oral findings of nutritional deficiencies such as iron and zinc deficiency, pernicious anemia, and vitamin B complex deficiency may be absent in patients
with burning mouth syndrome and must be confirmed with appropriate laboratory testing. The clinician should observe the patient
closely for abnormal tongue and mouth movements, such as bruxism, tongue thrusting, repetitive running of the tongue against the
teeth, because these are suggestive of behavioral abnormalities that
may contribute to the patients pain symptomatology (Figure 16-1).
40

Figure 16-1 The hallmark of burning mouth syndrome is mouth and


tongue burning pain in the absence of clinically demonstrable oral
pathology.

16 Burning Mouth Syndrome 41

responsible for the patients pain. All underlying medical conditions (e.g., diabetes, deficiency syndromes) must be treated, along
with the removal of any local irritants such as mouth washes,
spicy foods, and cinnamon and mint products. Providing the
patient with a supportive and positive emotional environment
and reassurance that cancer is not the cause of the pain is paramount if symptom relief is to be achieved. Coexistent behavioral
and psychiatric abnormalities also must be addressed in a positive
therapeutic milieu. Empirical treatments, including anticandiadal
agents, vitamin B complex supplementation, and low-dose antidepressants, are also worthy of consideration.
Treatment often involves some combination of elimination of
any local irritants, treatment of underlying medical conditions,
pharmacological therapy, and behavioral therapy.
First, any nidus of tissue trauma that is contributing to the
ongoing sympathetic dysfunction responsible for the symptoms
must be identified and removed. Second, interruption of the sympathetic innervation of the face by stellate ganglion block with
local anesthetic must be implemented. This may require daily stellate ganglion block for a considerable period. Occupational therapy consisting of tactile desensitization of the affected mucosa also
may be of value. Underlying depression and sleep disturbance are
best treated with a tricyclic antidepressant such as nortriptyline,
given as a single 25-mg dose at bedtime. Gabapentin may help palliate any neuritic pain component and is best started slowly with
TABLE 161

Workup of Burning Mouth Syndrome


Thorough history and review of symptoms
Medications causing xerostomia
Dental or denture work

a single bedtime dose of 300 mg, titrating the dosage upward in


divided doses to a maximum dose of 3600 mg per day. Pregabalin
is a reasonable alternative to gabapentin and is better tolerated in
some patients. Pregabalin is started at 50 mg three times per day
and may be titrated upward to 100 mg three times per day as disease effects allow. Pregabalin is excreted primarily by the kidneys,
and the dosage should be decreased in patients with compromised
renal function.
Opioid analgesics and benzodiazepines should be avoided to
prevent iatrogenic chemical dependence.
TABLE 16-2

Causes of Burning Mouth and Tongue Pain


Systemic

Local

Psychogenic,
Psychiatric,
and Idiopathic

Deficiencies

Denture factors

Psychiatric

Iron

Dental work

Depression

Vitamin B12

Mechanical

Anxiety

Folate

Oral habit or parafunctional behavior

Obsessivecompulsive
disorder

Zinc

Clenching

Somatoform
disorder

B complex vitamins

Bruxism

Cancerphobia

Endocrine

Tongue thrusting

Psychosocial
stressors

Diabetes mellitus

Myofasical pain

Hypothyroidism

Allergic contact stomatitis

Menopause or
hormonal

Dental restoration or
denture materials

Oral care, oral products


Oral habits or parafunctional behavior

Foods
Xerostomia

Preservative, additives,
flavorings

Family history of oral cancer, psychiatric diagnoses, and connective


tissue disease

Connective tissue
disease

Neurologic

Oral examination

Sjgrens syndrome

Referred from tonsils or


teeth

Sicca syndrome

Lingual nerve neuropathy

Drug related

Glossopharyngeal
neuropathy

Anxiety or stress

Acoustic neuroma

Medication

Infection
Candidiasis

Vitamin B12, folate, zinc

Angiotensinconverting enzyme
inhibitor

Glucose, glycosylated hemoglobin

Esophageal reflux

Antibiotic related

Anemia

Denture related

History of depression, anxiety, cancerphobia

Erythema, candidiasis, xerostomia, or other mucosal abnormalities


Tongue disorders, such as a geographic, fissured, or atrophic
tongue
Dental work or dentures
Laboratory tests
Complete blood count
Iron, total iron binding capacity, iron saturation, ferreting

Culture for Candida


Patch testing
Include standard series, metal series, oral flavors, and preservatives
Further consultation if indicated by history and review of systems

Local trauma
Corticosteroid
Diabetes mellitus

Psychometric testing and psychiatric consultation

Fusospirochetal

Dentistry

Xerostomia

Neurology

Irradiation

Otorhinolaryngology

Local disease

From Drage LA, Rogers RSR III: Burning mouth syndrome, Dermatol Clin
21:135145, 2003.

From Drage LA, Rogers RSR III: Burning mouth syndrome, Dermatol Clin
21:135145, 2003.

42 SECTION 1 Headache and Facial Pain Syndromes

Complications and Pitfalls


The main complications surrounding the treatment of burning mouth syndrome are those associated with its misdiagnosis.
Chemical dependence, depression, and multiple failed therapeutic
procedures are the rule rather than the exception. A diagnosis of a
psychiatric basis for the patients pain should be made only after
all somatic causes of burning mouth syndrome have carefully been
ruled out.

Clinical Pearls
The key to diagnosing burning mouth syndrome is a high
index of clinical suspicion. Once causes of burning mouth
and tongue that have clinically identifiable pathological
processes have been ruled out, a rational treatment plan
addressing the often multifactorial nature of the patients
pain can be initiated. A supportive therapeutic environment
is crucial if symptom reduction is to be achieved.

SUGGESTED READINGS
Drage LA, Rogers RSR III: Burning mouth syndrome, Dermatol Clin 21:135145,
2003.
Miziara ID, Arajo Filho BC, Oliveira R, Rodrigues dos Santos RM: Group psychotherapy: an additional approach to burning mouth syndrome, Psychosom Res
67:443448, 2009.
Mock D: Burning tongue/mouth syndrome, J Oral Maxillofac Surg 67(Suppl 1):5,
2009.
Moore PA, Guggenheimer J, Orchard T: Burning mouth syndrome and peripheral
neuropathy in patients with type 1 diabetes mellitus, J Diabetes Complications
21:397402, 2007.

Chapter 17
NERVUS INTERMEDIUS NEURALGIA

ICD-9 CODE 053.11


ICD-10 CODE B02.21
The Clinical Syndrome
Nervus intermedius neuralgia is an uncommon cause of primary
otalgia. Also known as geniculate neuralgia, nervus intermedius
neuralgia is believed to be caused by compression of the nervus
intermedius portion of the cranial nerve VII (facial) by aberrant
blood vessels or tumor in a manner analogous to trigeminal and
glossopharyngeal neuralgia. Although cranial nerve VII is primarily a motor nerve comprising special visceral efferent fibers that
innervate the facial muscles, a small number of sensory and parasympathetic fibers are also present. These sensory fibers provide
sensory innervations to the skin of the external auditory meatus,
portions of the nasal and nasopharyngeal muscosa, and the anterior two thirds of the tongue. When the nervus intermedius and
its associated geniculate ganglion are infected with herpes zoster
virus, a clinical syndrome known as Ramsey Hunt syndrome
occurs (see Chapter 13).
The pain of nervus intermedius neuralgia is severe and is
rivaled only by that of trigeminal and glossopharyngeal neuralgia and cluster headache. The pain has been described as like
having an ice pick repeatedly jabbed into the ear. Uncontrolled
pain of this severity has been associated with suicide and should
therefore be treated as an emergency. Attacks can be triggered
by daily activities involving contact with the external acoustic
meatus or auricle. Patients have also noted that attacks of nervus
intermedius neuralgia can be triggered by lying on the affected
side (Figure 17-1). Pain can be controlled with medication in
some patients, but surgical resection of the nervus intermedius is
required in approximately 50% of cases. The association between
multiple sclerosis and trigeminal neuralgia does not to appear as
strong in patients with nervus intermedius neuralgia, but a single
case has been reported.

Patients with nervus intermedius neuralgia go to great lengths


to avoid any contact with trigger areas. In contrast, persons with
other types of facial pain, such as temporomandibular joint dysfunction, tend to constantly rub the affected area or apply heat
or cold to it. Patients with uncontrolled nervus intermedius
neuralgia frequently require hospitalization for rapid control of
pain. Between attacks, patients are relatively pain free. A dull
ache remaining after the intense pain subsides may indicate
persistent compression of the nerve by a structural lesion. This
disease is almost never seen in persons younger than 30 years.
Patients with nervus intermedius neuralgia often have severe
depression (sometimes to the point of being suicidal), with high
levels of superimposed anxiety during acute attacks. Both of these
problems may be exacerbated by the sleep deprivation that often
accompanies painful episodes.

Testing
All patients with a new diagnosis of nervus intermedius neuralgia
should undergo magnetic resonance imaging (MRI) of the brain
and brainstem, with and without gadolinium contrast medium,
to rule out posterior fossa or brainstem lesions and demyelinating disease (Figure 17-2). Magnetic resonance angiography
is also useful to confirm vascular compression of the nervus

Signs and Symptoms


Nervus intermedius neuralgia causes severe, episodic pain afflicting the area of the acoustic auditory meatus supplied by the nervus
intermedius. The pain is unilateral and characterized by paroxysms of electric shocklike pain lasting from several seconds to less
than 2 minutes. The progression from onset to peak is essentially
instantaneous.

Figure 17-1 Nervus intermedius neuralgia is characterized by


severe episodic neuritic pain affecting the area of the acoustic
auditory meatus.

43

44 SECTION 1 Headache and Facial Pain Syndromes

Treatment

Axial

Coronal
Figure 17-2 Gadolinium-enhanced magnetic resonance imaging (MRI).
Images show a centrally enhancing lesion in the geniculate ganglion
(arrow), measuring 5 mm 10 mm in diameter. (From Miyashita T,
Hoshikawa H, Kagawa M, Mori N: A case report of facial nerve hemangioma, Auris Nasus Larynx 34:519522, 2007.)

intermedius or geniculate ganglion by aberrant blood vessels.


Additional imaging of the sinuses should be considered in the
case of any question of occult or coexisting sinus disease. If the
first division of the trigeminal nerve is affected, ophthalmological evaluation to measure intraocular pressure and rule out intraocular pathological conditions is indicated. Screening laboratory
tests consisting of a complete blood count, erythrocyte sedimentation rate, and automated blood chemistry should be performed
if the diagnosis of trigeminal neuralgia is in question. A complete
blood count is required for baseline comparisons before starting
treatment with carbamazepine (see discussion of treatment).

Differential Diagnosis
Nervus intermedius neuralgia is generally a diagnosis of exclusion, although the clinical presentation makes it a straightforward
clinical diagnosis that can be made on the basis of a targeted history and physical examination. Diseases of the eyes, ears, nose,
throat, and teeth may mimic nervus intermedius neuralgia or
may coexist and confuse the diagnosis. Atypical facial pain or
temporomandibular joint dysfunction is sometimes confused
with nervus intermedius neuralgia, but it can be distinguished by
the character of the painatypical facial pain is dull and aching,
whereas the pain of nervus intermedius neuralgia is sharp and
neuritic. Additionally, the pain of nervus intermedius neuralgia
occurs in the distribution of the nervus intermedius, whereas the
pain of atypical facial pain does not follow a specific nerve distribution. Multiple sclerosis should be considered in all patients
who present with nervus intermedius neuralgia before the fifth
decade of life.

Drug Therapy
Carbamazepine
Carbamazepine is considered first-line treatment for nervus intermedius neuralgia. In fact, a rapid response to this drug helps confirms
the clinical diagnosis. Despite the safety and efficacy of carbamazepine, some confusion and anxiety exist surrounding its use. This
medication, which may be the patients best chance for pain control, is sometimes discontinued because of laboratory abnormalities erroneously attributed to it. Therefore baseline measurements
consisting of a complete blood count, urinalysis, and automated
blood chemistry profile should be obtained before starting the drug.
Carbamazepine should be initiated slowly if the pain is not
out of control, with a starting dose of 100 to 200 mg at bedtime
for 2 nights. The patient should be cautioned about side effects,
including dizziness, sedation, confusion, and rash. The drug is
increased in 100- to 200-mg increments given in equally divided
doses over 2 days, as side effects allow, until pain relief is obtained
or a total dose of 1200 mg per day is reached. Careful monitoring
of laboratory parameters is mandatory to avoid the rare possibility of a life-threatening blood dyscrasia. At the first sign of blood
count abnormality or rash, this drug should be discontinued. Failure
to monitor patients on carbamazepine can be disastrous, because
aplastic anemia can occur. When pain relief is obtained, the
patient should be kept at that dosage of carbamazepine for at least
6 months before tapering of the medication is considered. The
patient should be informed that under no circumstances should
the drug dosage be changed or the drug refilled or discontinued
without the physicians knowledge.
Gabapentin
In the uncommon event that carbamazepine does not adequately
control a patients pain, gabapentin may be considered. As with
carbamazepine, baseline blood tests should be obtained before
starting therapy and the patient should be cautioned about potential side effects, including dizziness, sedation, confusion, and rash.
The initial dose is 300 mg at bedtime for 2 nights. The drug is
then increased in 300-mg increments given in equally divided
doses over 2 days, as side effects allow, until pain relief is obtained
or a total dose of 2400 mg per day is reached. At this point, if the
patient has experienced only partial pain relief, blood values are
measured and the drug is carefully titrated upward using 100-mg
tablets. Rarely is a dosage greater than 3600 mg per day required.
Pregabalin
Pregablin represents a reasonable alternative to gabapentin and
is better tolerated in some patients. Pregablin is started at 50 mg
three times per day and may be titrated upward to 100 mg three
times per day as side effects allow. Pregablin is excreted primarily
by the kidneys, and thus the dosage should be decreased in patients
with compromised renal function.
Baclofen
Baclofen may be of value in some patients who fail to obtain relief
from carbamazepine, gabapentin, or pregabalin. As with those
drugs, baseline laboratory tests should be obtained before beginning baclofen therapy and the patient should be warned about the
same potential adverse effects. Start with a 10-mg dose at bedtime
for 2 nights, then increase the drug in 10-mg increments given in
equally divided doses over 7 days, as side effects allow, until pain
relief is obtained or a total dose of 100 mg per day is reached. This

17 Nervus Intermedius Neuralgia 45

drug has significant hepatic and central nervous system side effects,
including weakness and sedation. As with carbamazepine, careful
monitoring of laboratory values is indicated when using baclofen.
When treating individuals with any of these drugs, the physician should make sure the patient knows that premature tapering
or discontinuation of the medication may lead to the recurrence
of pain, which will be more difficult to control.
Invasive Therapy
Section of the Nervus Intermedius
This neurosurgical technique is the invasive treatment of choice
for those patients with nervus intermedius neuralgia who have
failed to respond to conservative pharmacological management.
To perform this procedure, the nervus intermedius and geniculate
ganglion are identified and isolated and the nervus intermedius
is sectioned in two places. Some surgeons also advocate extirpation of the geniculate ganglion. Section of the nervus intermedius
alone provides excellent palliation of pain in 75% to 90% of cases.

Complications and Pitfalls


The pain of nervus intermedius neuralgia is severe and can lead
to suicide; therefore it must be considered a medical emergency,
and strong consideration should be given to hospitalizing such
patients. If a dull ache remains between the intense paroxysms of
pain, the clinician should have a high index of suspicion that the
nidus of the patients pain is persistent compression of the nerve
by a structural lesion such as a brainstem tumor or schwannoma.

Clinical Pearls
Nervus intermedius neuralgia is an uncommon cause of otalgia. Because of the potential for disastrous clinical outcome
should a more common cause of otic pain be overlooked
(e.g., tumor or the temporal bone, brainstem, or nasopharynx), the diagnosis of nervus intermedius neuralgia must by
necessity be one of exclusion. Because of the severity of the
pain associated with this syndrome, aggressive pharmacological management in an inpatient setting may be required.
Surgical treatment consisting of the sectioning of the nervus
intermedius is often the patients best option for complete
and long-lasting pain relief.

SUGGESTED READINGS
Alcaraz N, King WA, Wackym PA: Endoscopy during neurotomy of the nervus
intermedius for geniculate neuralgia, Otolaryngol Head Neck Surg 121:334336,
1999.
Bhagra A, Stead LG: Nervus intermedius neuralgia: a rare entity, Ann Emerg Med
47:579, 584, 2006.
Gantz BJ, Redleaf MI, Perry BP, Gubbels SP: Management of Bells palsy and
nervus intermedius neuralgia. In Brackmann DE, Shelton C, Arriaga MA,
editors:Otologic surgery, ed 3, Philadelphia, 2010, Elsevier, pp 335346.
Persson A, Bergstrm T, Lindh M, Namvar L, Studahl M: Varicella-zoster
virus CNS disease: viral load, clinical manifestations and sequels, J Clin Virol
46:249253, 2009.
Taguchi T, Ueda S, Kudo T, etal: Ramsay-Hunt syndrome, J Infect 62:180181,
2011.
Ulusoy , zkan G, Bekta D, etal: Nervus intermedius neuralgia in renal transplantation recipient: a case report, Transplant Proc 42:19861988, 2010.

Chapter 18
RED EAR SYNDROME

ICD-9 CODE 350.1


ICD-10 CODE G50.0
The Clinical Syndrome
Red ear syndrome is an uncommon primary pain disorder thought
to be a variant of one of a group of three headache syndromes known
as the trigeminal autonomic cephalgias (Table 18-1). Whether red
ear syndrome is in fact a distinct pain syndrome resulting from
auriculo-autonomic dysfunction or simply a constellation of symptoms that occurs on a continuum along with the other trigeminal
autonomic cephalgias is a point of ongoing debate among headache
and pain management specialists. As with most headache and facial
pain syndromes, the exact cause of the pain of red ear syndrome is
unknown; however, the pathogenesis of this uncommon cause of
head and face pain is thought to be dysfunction of the trigeminal
autonomic reflex. The rapid onset of ear redness and associated pain
may be caused by an antidromic release of vasoactive peptides from
the terminal afferent fibers of the third cervical nerve root, which
provides sensory innervations to the pinna of the ear.
As its name implies, the pathognomonic finding of red ear syndrome is in fact a unilateral red ear (Figure 18-1). This redness
involves the entire ear, including the pinna, and is associated with
neuralgia-like pain reminiscent of sudden unilateral neuralgiform
conjunctival injection tearing (SUNCT) headache (see Chapter
7). The pain and erythema associated with red ear syndrome have
a rapid onset to peak, with attacks lasting 15 seconds to 5 minutes
and the frequency of attacks ranging from 20 to 200 attacks per
day. In some patients, these attacks can be triggered by sensory
stimulation of the affected area, such as when brushing the hair.
Although in many ways similar to SUNCT headache (i.e., unilateral, rapid onset to peak, short duration of attacks, pain-free
periods between attacks), many dissimilarities also exist, including
the location and pronounced autonomic phenomenon manifested
by the red ear.

Signs and Symptoms


Patients with red ear syndrome present with the complaint of
severe paroxysms of sudden onset of unilateral ear redness associated with pain involving the ipsilateral ear. The pain is neuralgiform in quality and severe to excruciating in intensity. Like
trigeminal neuralgia, the pain of red ear syndrome rarely switches
sides. Red ear syndrome occurs slightly more frequently in males.
It can occur at any age, with a peak incidence in the fifth decade.

Testing
Magnetic resonance imaging (MRI) of the brain provides the clinician with the best information regarding the cranial vault and its
contents. MRI is highly accurate and helps identify abnormalities
that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathology, including tumors and
demyelinating disease. Magnetic resonance angiography (MRA)
also may be useful in helping identify aneurysms that may be
responsible for the patients neurological findings. In patients who
cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scan and plain radiography are indicated if a fracture or

TABLE 18-1

The Trigeminal Autonomic Cephalgias


Cluster headache
Paroxysmal hemicranias
Short-lasting unilateral neuralgiform headache with conjunctival
injection tearing (SUNCT)

46

Figure 18-1 Red ear syndrome is characterized by the complaint of


severe paroxysms of sudden onset of unilateral ear redness associated
with ipsilateral ear pain.

18 Red Ear Syndrome 47

bony abnormality such as metastatic disease is considered in the


differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood chemistry should be performed if the diagnosis of red ear syndrome is in
question. Additional testing to rule out collagen-vascular disease is
indicated if polychondritis is suspected.

Differential Diagnosis
Red ear syndrome is a clinical diagnosis supported by a combination of clinical history, normal physical examination, radiography, and MRI. Pain syndromes that may mimic red ear syndrome
include erythromelalgia of the ear, polychondritis, cluster headache, temporal arteritis, trigeminal neuralgia, demyelinating
disease, primary stabbing headache, SUNCT, and chronic paroxysmal hemicranias. However, because of the overlapping features
of all headache and facial pain syndromes, red ear syndrome easily
can be mistaken for another type of headache or facial pain. Trigeminal neuralgia is more common and is characterized by trigger
areas and tic-like movements. Demyelinating disease is generally associated with other neurological findings, including optic
neuritis and other motor and sensory abnormalities. The pain of
chronic paroxysmal hemicrania lasts much longer than the pain of
red ear syndrome.

Treatment
The treatment of red ear syndrome is analogous to the treatment of
trigeminal neuralgia, although the pharmacological management
of this uncommon headache disorder is disappointing. The use of
anticonvulsants such as lamotrigine and gabapentin represents a
reasonable starting point. High-dose steroids tapered over 10 days
also have been anecdotally reported to provide relief. For patients
who do not respond to these treatments, a few case reports suggest
that daily ipsilateral C2-C3 facet joint blocks with local anesthetic
and steroid may provide relief of both the pain and the autonomic
dysfunction. Underlying sleep disturbance and depression associated with the pain of red ear syndrome are best treated with a
tricyclic antidepressant compound, such as nortriptyline, which
can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls


Failure to diagnose red ear syndrome correctly may put the
patient at risk if intracranial pathology or demyelinating disease,
which may mimic the clinical presentation of red ear syndrome,
is overlooked. MRI is indicated in all patients thought to have red
ear syndrome. A careful evaluation of the ear to rule out localized
pathological conditions is also indicated, as is laboratory testing
for collagen-vascular disease if polychondritis is suspected.

Clinical Pearls
Given the poor response to treatment with drugs traditionally used to treat trigeminal neuralgia, facet block of
the ipsilateral C2-C3 facet joints with local anesthetic and
steroids should be considered in patients thought to have
red ear syndrome. Given the uncommon nature of this
headache syndrome and its overlap with the other trigeminal autonomic cephalgias and other more serious forms of
intracranial pathological conditions such as tumors and
vascular abnormalities, red ear syndrome must remain
a diagnosis of exclusion. All patients thought to have red
ear syndrome require MRI of the brain with and without
gadolinium contrast material and thorough otic and neurological evaluation. Cervical facet block should be performed
only by clinicians familiar with the regional anatomy.

SUGGESTED READINGS
Kumar N, Swanson JW: The red ear syndrome revisited: two cases and a review
of literature, Cephalalgia 24:305308, 2004.
Lance JW: The red ear syndrome, Neurology 47:617620, 1996.
Leone M, Bussone G: Pathophysiology of trigeminal autonomic cephalalgias, Lancet
Neurol 8:18551884, 2009.
Purdy RA, Dodick DW: Red ear syndrome, Curr Pain Headache Rep 11:313316,
2007.
Waldman SD: Cervical facet block. In Waldman SD, editor: Atlas of interventional
pain management, ed 3, Philadelphia, 2009, Saunders, pp 165168.

Chapter 19
GLOSSOPHARYNGEAL NEURALGIA

ICD-9 CODE 352.1


ICD-10 CODE G52.10
The Clinical Syndrome
Glossopharyngeal neuralgia is a rare condition characterized by
paroxysms of pain in the sensory division of the cranial nerve IX.
Although the pain of glossopharyngeal neuralgia is similar to that
of trigeminal neuralgia, it occurs 100 times less frequently. Glossopharyngeal neuralgia occurs more commonly in patients older
than 50 years. The pain is located in the tonsil, laryngeal region,
and posterior tongue. The pain is unilateral in most patients, but
can occur bilaterally 2% of the time. Rarely, the pain of glossopharyngeal neuralgia is associated with bradyarrhythmias; in some
patients, it is associated with syncope. These cardiac symptoms are
thought to be due to overflow of neural impulses from the glossopharyngeal nerve to the vagus nerve. Although rare, this unusual
combination of pain and cardiac arrhythmia can be lethal.

at risk for neurological disasters secondary to intracranial and


brainstem pathology, including tumors and demyelinating disease
(see Figure 19-2). Magnetic resonance angiography (MRA) may
be helpful in identifying aneurysms responsible for neurological
symptoms. In patients who cannot undergo MRI, such as patients
with pacemakers, computed tomography (CT) is a reasonable
second choice.
Clinical laboratory tests consisting of complete blood cell count,
automated chemistry profile, and erythrocyte sedimentation rate
are indicated to rule out infection, temporal arteritis, and malignancy that may mimic glossopharyngeal neuralgia. Endoscopy of
the hypopharynx with special attention to the piriform sinuses
also is indicated to rule out occult malignancy. Differential neural
blockade of the glossopharyngeal nerve may help strengthen the
diagnosis of glossopharyngeal neuralgia.

Differential Diagnosis
Glossopharyngeal neuralgia is generally a straightforward clinical
diagnosis that can be made on the basis of a targeted history and
physical examination. Diseases of the eye, ears, nose, throat, and
teeth may mimic trigeminal neuralgia or may coexist and confuse
the diagnosis. Tumors of the hypopharynx, including the tonsillar

Signs and Symptoms


The pain of glossopharyngeal neuralgia is in the distribution of cranial nerve IX (Figure 19-1). In some patients, overflow pain may
occur in areas innervated by the trigeminal nerve, upper cervical
segments, or both. The pain is neuritic and is unilateral in 98% of
patients. It is often described as shooting or stabbing, with a severe
intensity level. The pain of glossopharyngeal neuralgia is often
triggered by swallowing, chewing, coughing, or talking. With the
exception of trigger areas in the distribution of cranial nerve IX,
the patients neurological examination should be normal. Because
tumors at the cerebellopontine angle may produce symptoms identical to those of glossopharyngeal neuralgia, an abnormal neurological examination is cause for serious concern (Figure 19-2). Dull,
aching pain that persists between the paroxysms of pain normally
associated with glossopharyngeal neuralgia is highly suggestive of a
space-occupying lesion and requires thorough evaluation.

Palatine tonsil
Posterior of
tongue

Testing
Magnetic resonance imaging (MRI) of the brain and brainstem
should be performed in all patients thought to have glossopharyngeal neuralgia. MRI of the brain provides the best information regarding the cranial vault and its contents. MRI is highly
accurate and helps identify abnormalities that may put the patient
48

Figure 19-1 The pain of glossopharyngeal neuralgia is in the distribution


of cranial nerve IX.

19 Glossopharyngeal Neuralgia 49

fossa and piriform sinuses, may mimic the pain of glossopharyngeal neuralgia, as may tumors at the cerebellopontine angle. Occasionally, demyelinating disease may produce a clinical syndrome
identical to glossopharyngeal neuralgia. The jaw claudication associated with temporal arteritis also sometimes confuses the clinical
picture, as does trigeminal neuralgia.

Treatment
Pharmacological Treatment
Carbamazepine
Carbamazepine is considered first-line treatment for glossopharyngeal neuralgia. Rapid response to this drug essentially confirms a clinical diagnosis of glossopharyngeal neuralgia. Despite
the safety and efficacy of carbamazepine compared with other
treatments for glossopharyngeal neuralgia, much confusion and
unfounded anxiety surround its use. This medication, which may
be the patients best chance for pain control, is sometimes discontinued because of laboratory abnormalities erroneously attributed
to it. Baseline screening laboratory tests, consisting of a complete
blood cell count, urinalysis, and automated chemistry profile,
should be obtained before starting the drug.
Carbamazepine should be started slowly, if the pain is not out
of control, at a starting dose of 100 to 200 mg at bedtime for
2 nights; the patient should be cautioned regarding side effects,
including dizziness, sedation, confusion, and rash. The drug is
increased in 100- to 200-mg increments, given in equally divided
doses over 2 days, as side effects allow, until pain relief is obtained

or a total dose of 1200 mg per day is reached. Careful monitoring


of laboratory parameters is mandatory to avoid the rare possibility
of life-threatening blood dyscrasia. At the first sign of blood count
abnormality or rash, this drug should be discontinued. Failure
to monitor patients started on carbamazepine can be disastrous
because aplastic anemia can occur. When pain relief is obtained,
the patient should be kept at that dosage of carbamazepine for at
least 6 months before considering tapering of this medication. The
patient should be informed that under no circumstances should
the dosage of drug be changed or the drug refilled or discontinued
without the physicians knowledge.
Gabapentin
In the uncommon event that carbamazepine does not control a
patients pain adequately, gabapentin may be considered. As with
carbamazepine, baseline blood tests should be obtained before
starting therapy. Gabapentin should be started with a 300-mg dose
at bedtime for 2 nights; the patient should be cautioned about
potential side effects, including dizziness, sedation, confusion, and
rash. The drug is increased in 300-mg increments, given in equally
divided doses over 2 days, as side effects allow, until pain relief is
obtained or a total dose of 2400 mg per day is reached. At this
point, if the patient has experienced partial pain relief, blood values are measured and the drug is carefully titrated using 100-mg
tablets. More than 3600 mg per day is rarely required.
Baclofen
Baclofen has been reported to be of value in some patients who
fail to obtain relief from carbamazepine and gabapentin. Baseline

B
Figure 19-2 Mixed cystic and solid acoustic nerve schwannoma in association with a solid schwannoma of the geniculate ganglion. A, Axial
enhanced image with fat saturation. A large mass with solid and cystic enhancing components is seen in the right cerebellopontine angle. A separate
solid erosive tumor is seen in the region of the right geniculate ganglion (arrowhead). B, Coronal enhanced image with fat saturation. The characteristic mushroom appearance of an intracanalicular acoustic schwannoma with extension into the adjacent cerebellopontine angle is well seen. This
more anterior section through the internal auditory canal does not show the cystic portion of the tumor, but it does show the compression of the
adjacent brainstem. (From Stark DD, Bradley WG Jr, editors: Magnetic resonance imaging, 3rd ed, St Louis, 1999, Mosby, p 1219.)

50 SECTION 1 Headache and Facial Pain Syndromes

laboratory tests should be obtained before starting baclofen. The


drug is started with a 10-mg dose at bedtime for 2 nights; the
patient should be cautioned about potential adverse effects, which
are the same as those of carbamazepine and gabapentin. The drug
is increased in 10-mg increments, given in equally divided doses
over 7 days, as side effects allow, until pain relief is obtained or
a total dose of 80 mg daily is reached. This drug has significant
hepatic and central nervous system side effects, including weakness and sedation. As with carbamazepine, careful monitoring of
laboratory values is indicated during the initial use of this drug.
When treating patients with any of the drugs mentioned, the
clinician should inform the patient that premature tapering or
discontinuation of the medication may lead to the recurrence of
pain. It becomes more difficult to control pain thereafter.
Interventional Treatment
Glossopharyngeal Nerve Block
The use of glossopharyngeal nerve block with local anesthetic and
a steroid serves as an excellent adjunct to drug treatment of glossopharyngeal neuralgia (Figure 19-3). This technique rapidly relieves
pain while medications are being titrated to effective levels. The
initial block is performed with preservative-free bupivacaine combined with methylprednisolone. Subsequent daily nerve blocks are
done in a similar manner, substituting a lower dose of methylprednisolone. This approach also may be used to obtain control of
breakthrough pain.
Radiofrequency Destruction of the
Glossopharyngeal Nerve
The destruction of the glossopharyngeal nerve can be carried out
by creating a radiofrequency lesion under biplanar fluoroscopic
guidance. This procedure is reserved for patients who have failed
to respond to all the treatments mentioned for intractable glossopharyngeal neuralgia and who are not candidates for microvascular decompression of the glossopharyngeal root. Gamma knife
ablation has also been used in this patient population.

Styloid
process

Glossopharyngeal
nerve
Figure 19-3 Proper needle placement for glossopharyngeal nerve
block. (From Waldman SD: In Waldman SD, editor: Atlas of interventional
pain management techniques, 3rd ed, Philadelphia, 2009, Saunders.)

Microvascular Decompression of the


Glossopharyngeal Root
Microvascular decompression of the glossopharyngeal root, also
referred to as the Jannetta procedure, is the major neurosurgical
procedure of choice for intractable glossopharyngeal neuralgia.
It is based on the theory that glossopharyngeal neuralgia is a
compressive mononeuropathy analogous to trigeminal neuralgia
(Figure 19-4). The operation consists of identifying the glossopharyngeal root close to the brainstem and isolating the offending
compressing blood vessel. A sponge is interposed between the vessel and nerve, relieving the compression and the pain.

Complications and Pitfalls


The pain of glossopharyngeal neuralgia is severe and can lead to
suicide; therefore it must be considered a medical emergency,
and strong consideration should be given to hospitalizing such
patients. If a dull ache remains after the intense, paroxysmal pain
of glossopharyngeal neuralgia subsides, this is highly suggestive of
persistent compression of the nerve by a structural lesion such as
a brainstem tumor or schwannoma. Glossopharyngeal neuralgia
is almost never seen in persons younger than 30 years unless it
is associated with multiple sclerosis, and all such patients should
undergo MRI to identify demyelinating disease.

Figure 19-4 Vascular relationship between trigeminal nerve and the


superior cerebellar artery in the cerebellopontine cistern. The distortion
of the trigeminal rootlets is evident from this intraoperative microscopic
photograph. (From Franzini A, Ferroli P, Messina G, Broggi G: Surgical
treatment of cranial neuralgias. In Bruyn G, Vinken P, editors: Handbook of
clinical neurology, vol 97, New York, 2010, Elsevier, pp 679692.)

The major complications associated with glossopharyngeal


nerve block are related to trauma to the internal jugular and
carotid artery. Hematoma formation and intravascular injection of local anesthetic with subsequent toxicity are significant
problems for the patient. Blockade of the motor portion of the

19 Glossopharyngeal Neuralgia 51

glossopharyngeal nerve can result in dysphagia secondary to weakness of the stylopharyngeus muscle. If the vagus nerve is inadvertently blocked, as it often is during glossopharyngeal nerve block,
dysphonia secondary to paralysis of the ipsilateral vocal cord may
occur. Reflex tachycardia secondary to vagal nerve block is also
observed in some patients. Inadvertent block of the hypoglossal
and spinal accessory nerves during glossopharyngeal nerve block
will result in weakness of the tongue and trapezius muscle.
The glossopharyngeal nerve is susceptible to trauma from the
needle, hematoma, or compression during injection procedures.
Such complications, although usually transitory, can be quite
upsetting to the patient. Although uncommon, risk for infection
is ever present, especially in patients who have cancer and are
immunocompromised. Early detection of infection is crucial to
avoiding potentially life-threatening sequelae.

Clinical Pearls
The pain of glossopharyngeal neuralgia is among the most
severe pain that humans can experience and must be considered a medical emergency. The uncontrolled pain of
glossopharyngeal neuralgia has led to suicide, and hospitalization of such patients should be strongly considered.
Between attacks of glossopharyngeal neuralgia, the patient
is relatively pain free. If a dull ache remains after the intense
pain subsides, this is highly suggestive of a persistent compression of the nerve by a structural lesion, such as a brainstem tumor or schwannoma. Glossopharyngeal neuralgia
is almost never seen in individuals younger than 30 years
unless it is associated with multiple sclerosis, and all such
patients should undergo MRI with sequences designed to
identify demyelinating disease.

SUGGESTED READINGS
Benoliel R, Eliav E: Neuropathic orofacial pain, Oral Maxillofac Surg Clin North
Am 20:237254, 2008.
Franzini A, Ferroli P, Messina G, Broggi G: Surgical treatment of cranial neuralgias. In Bruyn G, Vinken P, editors: Handbook of clinical neurology, vol 97,
New York, 2010, Elsevier, pp 679692.
Khan NU, Iyer A: Glossopharyngeal neuralgia associated with anomalous glossopharyngeal nerve, Otolaryngol Head Neck Surg 136:502503, 2007.
Waldman SD: Glossopharyngeal nerve block. In Waldman SD, editor: Atlas of
interventional pain management, ed 3, Philadelphia, 2009, Saunders, pp 9397.
The surgical treatment of microvascular compression syndromes, Operative Tech
Neurosurg 4:137141, 2001.

SECTION 2 Neck and Brachial Plexus Pain Syndromes

Chapter 20
CLIVAL CHORDOMA SYNDROME

ICD-9 CODE 213.0


ICD-10 CODE D16.4

The Clinical Syndrome


Clival chordoma is a rare neoplasm that arises from embryological remnants of the notochord along the spinal axis. Clival chordomas are usually benign, although aggressive clival chordomas
have been reported. Comprising one third of central nervous system chordomas, clival chordomas tend to be slow growing and
produce symptoms by compression of the adjacent brainstem
and cranial nerves. In spite of this, the long-term outcome of
patients diagnosed with clival chordoma remains poor because of
the location of these tumors and their tendency to recur regardless of the treatment method chosen. Clival chordomas can occur
at any age, further complicating the diagnosis. These uncommon
tumors occur slightly more commonly in men. Early diagnosis of clival chordoma is important to avoid acute neurological
disasters; however, because of the slow growth of these tumors,
the average time from onset of symptoms to diagnosis averages
2 years.

Testing
Magnetic resonance imaging (MRI) of the brain and brainstem
should be performed in all patients thought to have clival chordoma
(see Figure 20-1). MRI of the brain provides the best information
regarding the cranial vault and its contents. MRI is highly accurate
and helps identify abnormalities that may put the patient at risk for
neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease
(Figure 20-2). Magnetic resonance angiography (MRA) may be helpful in identifying aneurysms responsible for neurological symptoms.
In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice.
Clinical laboratory tests consisting of a complete blood cell
count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection, temporal arteritis, and
other malignancies that may mimic clival chordoma. Endoscopy
of the nasopharynx and hypopharynx with special attention to the
piriform sinuses also is indicated to rule out occult malignancy.

Differential Diagnosis
Clival chordoma is generally a straightforward clinical diagnosis in retrospect. Given that the time between the onset of
TABLE 20-1

Signs and Symptoms

Common Symptoms Associated with Clival Chordoma

Headache is the most common presenting complaint in patients


with clival chordoma. Other common symptoms associated with
clival chordoma reflect the propensity of this tumor to compress
adjacent neural structures, causing facial pain, facial numbness,
facial paresthesias, and diplopia (Table 20-1). Ataxia, dysphagia,
visual disturbance, hoarseness, and extremity weakness also commonly occur.
Findings on neurological examination (e.g., cranial nerve deficits, pyramidal tract dysfunction, hemiparesis, hyperreflexia, clonus, a positive Babinski sign, and cerebellar signs, including ataxia)
also reflect compression of neural structures by this slow-growing
tumor (Figure 20-1). Occasionally, papilledema and optic nerve
atrophy are identified.

Headache

52

Facial numbness
Facial pain
Facial paresthesias
Diplopia
Dysarthria
Dysphagia
Ataxia
Extremity weakness
Hoarseness
Visual disturbance

20 Clival Chordoma Syndrome 53

neurological signs and symptoms and definitive diagnosis is an


average of 2 years, a high index of clinical suspicion is necessary
to avoid misdiagnosis. Obtaining a targeted history and performing a careful physical examination are essential. Diseases of
the eye, ears, nose, throat, and teeth may mimic trigeminal neuralgia or may coexist and confuse the diagnosis. Tumors of the
nasopharynyx and hypopharynx, including the tonsillar fossa
and piriform sinus, may mimic the pain of clival chordoma, as
may tumors at the cerebellopontine angle. Occasionally, demyelinating disease may produce a clinical syndrome identical to
clival chordoma. The jaw claudication associated with temporal

arteritis also sometimes confuses the clinical picture, as does


trigeminal neuralgia.

Treatment
Treatment of clival chordoma requires surgery, radiation therapy, or both. Although clival chordomas are almost always
benign and rarely metastasize, the critical location of clival chordomas relative to adjacent neural structures makes both forms
of treatment challenging. Often, complete tumor resection is
impossible because of the location and postoperative radiation

Figure 20-1 Patients suffering from clival chordoma will often complain of headaches and associated facial pain, numbness, and diplopia.

Figure 20-2 Sagittal (A) and axial (B) T2-weighted magnetic resonance imaging of the clival chordoma showing significant compression of the
spinal cord and brainstem plus destruction of cervical vertebra. (From Chau T, Lazzaro A, Mobbs RJ, Teo C: Surgical treatment of cranial neuralgias:
combined endoscopic endonasal and posterior cervical approach to a clival chordoma, J Clin Neurosci 17:14631465, 2010.)

54 SECTION 2 Neck and Brachial Plexus Pain Syndromes

therapy, gamma knife stereotactic surgery, and implantation of


radioactive seeds may be required.

Complications and Pitfalls


Because of the slow-growing nature of clival chordomas, delayed
diagnosis is an ever-present possibility complicating an already
difficult treatment regimen. Further confusing the clinical presentation of this tumor of embryological origin is the fact that many
of the clinical syndromes that mimic the signs and symptoms of
clival chordoma are also difficult to diagnose.

Clinical Pearls
Clival chordoma is a rare neoplasm that is usually benign,
although aggressive clival chordomas have been reported.
Clival chordomas tend to be slow growing and produce
symptoms by compression of the adjacent brainstem and
cranial nerves. In spite of this fact, the long-term outcome
of patients diagnosed with clival chordoma remains poor
because of the location of these tumors and their tendency
to recur regardless of the treatment method chosen. Clival
chordomas can occur at any age.

SUGGESTED READINGS
Aminoff M, Boller F, Swaab D: Cytogenetic analysis of three variants of clival
chordoma, Cancer Genet Cytogenet 154:124130, 2004.
Chau T, Lazzaro A, Mobbs RJ, Teo C: Surgical treatment of cranial neuralgias:
combined endoscopic endonasal and posterior cervical approach to a clival chordoma, J Clin Neurosci 17:14631465, 2010.
Chugh R, Tawbi H, Lucas DR, etal: Chordoma: the nonsarcoma primary bone
tumor, Oncologist 12:13441350, 2007.
Feng K, Qiuhang Z, Qiuyi Q: Transclival cerebrospinal fluid rhinorrhea as the
initial presenting symptom of a tiny intradural chordoma, J Clin Neurosci
17:10831085, 2010.

Chapter 21
SPASMODIC TORTICOLLIS

ICD-9 CODE 33.83


ICD-10 CODE G24.8
The Clinical Syndrome

feature of the syndrome, and spasms of the cervical paraspinous


musculature, the strap muscles of the neck, and the sternocleidomastoid are often present. Hypertrophy of the affected muscles
may occur occasionally. Other than the dystonic movements, the
neurological examination is normal. As mentioned previously, the
patient may seem indifferent to the abnormal head movements
or position. Touching the opposite side of the face or chin often
causes the dystonia to cease momentarily.

Spasmodic torticollis is a rare condition characterized by involuntary movement of the head. It is classified as a focal or segmental dystonia and occurs in approximately 3 in 10,000 people. It
begins in early adult life. The three varieties of spasmodic torticollis are as follows:
Tonic, which involves involuntary turning of the head to
one side
Clonic, which involves involuntary shaking of the head
Tonic/clonic, which involves both types of involuntary
movement
Spasmodic torticollis also can be subclassified based on the
specific movement of the head: (1) rotation, which involves the
turning of the head to the side; (2) laterocollis, which involves
the leaning of the head against the shoulder; (3) retrocollis, which
involves the leaning of the head toward the back; and (4) anterocollis, which involves the leaning of the head toward the chest.
The disease occurs more commonly in women and often is initially diagnosed as a hysterical reaction or tic.
Thought to be due to dysfunction centrally, rather than a disease of the affected muscles, spasmodic torticollis often begins as a
subtle involuntary movement of the head. Early in the disease, the
dystonia is often intermittent. As the disease progresses, the symptoms become more severe and harder for the patient to hide. The
dystonic movements may become more sustained and associated
with constant, aching pain in the affected muscles. The pain often
becomes the primary reason for the patient to seek medical attention, with the patient almost indifferent to the dystonic movements. The dystonia often disappears with sleep and becomes less
pronounced on first awakening, with the dystonic movements
and pain worsening as the day progresses. Spontaneous recovery
has been reported, but, overall, treatment is difficult and of limited success.

Testing

Signs and Symptoms

Treatment

A patient with spasmodic torticollis exhibits involuntary, dystonic


movements of the head. In extreme cases, the dystonia is continuous and the laterocollis so marked that the patients ear rests on
the ipsilateral shoulder (Figure 21-1). Pain may be a predominant

In general, the treatment of spasmodic torticollis is disappointing.


Pharmacological treatment with skeletal muscle relaxants, including drugs that act at the spinal cord level, such as baclofen, and
centrally acting drugs, such as the anticonvulsants and levodopa,

Magnetic resonance imaging (MRI) of the brain and brainstem


should be performed in all patients thought to have spasmodic
torticollis. MRI of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and
helps identify abnormalities that may put the patient at risk for
neurological disasters secondary to intracranial and brainstem
pathological conditions, including tumors and demyelinating
disease. Magnetic resonance angiography (MRA) may be useful in identifying aneurysms responsible for neurological symptoms. In patients who cannot undergo MRI, such as patients with
pacemakers, computed tomography (CT) is a reasonable second
choice.
Clinical laboratory tests consisting of a complete blood cell
count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection and malignancy.

Differential Diagnosis
Spasmodic torticollis is generally a straightforward clinical diagnosis that can be made on the basis of a targeted history and physical
examination. The involuntary nature of this movement disorder
is the hallmark of the disease and helps distinguish it from tics
and habit spasms that are voluntary and worsen when the patient
is tense. Tics and habit spasms resemble volitional movement.
Behavioral abnormalities, such as hysterical conversion reactions,
also must be considered. Acute spasm and pain of the muscles
of the neck or wry neck can mimic spasmodic torticollis, but its
onset is acute, and the symptoms usually resolve within days to a
week. Occasionally, patients with clonic spasmodic torticollis are
initially diagnosed as having Parkinson disease.

55

56 SECTION 2 Neck and Brachial Plexus Pain Syndromes

Longus capitus
muscle

Scalene muscles:
Middle
Anterior
Posterior
Longus colli
muscle

Figure 21-1 The dystonia of spasmodic torticollis causes significant pain and functional disability.

may provide some symptomatic relief in mild cases. Trihexyphenidyl and diazepam also have been advocated.
In patients for whom pharmacological treatment fails, injection of the affected muscles with botulinum toxin is a reasonable
next step. Frequent injections may result in the development of
antibodies against the toxin, which makes the toxin less effective. By changing to different subtypes of toxin, efficacy may be
restored. For intractable cases, bilateral thalamotomy has been
advocated. The results of this radical treatment are variable at best.

Complications and Pitfalls


Although spasmodic torticollis is usually a straightforward clinical
diagnosis, the clinician must always rule out other pathological
processes involving the central nervous system. Treatment of this
syndrome is difficult, and treatment of concurrent depression is
often required.

Clinical Pearls
Spasmodic torticollis is a devastating disease that responds
poorly to treatment. Injection of the affected muscles with
botulinum toxin to effect chemodenervation is probably the
best therapeutic option for most patients. The diagnosis of
the disease is straightforward. MRI of the brain is indicated
in all patients thought to have spasmodic torticollis.

SUGGESTED READINGS
Maia FM, Kanashiro AK, Chien HF, Gonalves LR, Barbosa ER: Clinical changes
of cervical dystonia pattern in long-term botulinum toxin treated patients, Parkinsonism Relat Disord 16:811, 2010.
Ochudo S, Drzyzga K, Drzyzga LR, Opala G: Various patterns of gestes antagonistes in cervical dystonia, Parkinsonism Relat Disord 13:417420, 2007.
Takeuchi N, Chuma T, Mano Y: Phenol block for cervical dystonia: effects and
side effects, Arch Phys Med Rehabil 85:11171120, 2004.
Truong D, Brodsky M, Lew M, etal: Global Dysport Cervical Dystonia Study
Group: Long-term efficacy and safety of botulinum toxin type A (Dysport) in
cervical dystonia, Parkinsonism Relat Disord 16:316323, 2010.

Chapter 22
CERVICOTHORACIC INTERSPINOUS
BURSITIS

ICD-9 CODE 727.3


ICD-10 CODE M71.50
The Clinical Syndrome
Cervicothoracic interspinous bursitis is an uncommon cause of
pain in the lower cervical and upper thoracic spine. The interspinous ligaments of the lower cervical and upper thoracic spine
and their associated muscles are susceptible to the development
of acute and chronic pain symptoms after overuse. Bursitis is
believed to be responsible for this pain syndrome. Frequently, the
patient presents with midline pain after prolonged activity requiring hyperextension of the neck, such as painting a ceiling or prolonged use of a computer monitor with too high of a focal point.
The pain is localized to the interspinous region between C7 and
T1 and does not radiate. It is constant, dull, and aching. The
patient may attempt to relieve the constant ache by assuming a
posture of dorsal kyphosis with a thrusting forward of the neck
(Figure 22-1). The pain of cervicothoracic interspinous bursitis
often improves with activity and worsens with rest and relaxation.

Signs and Symptoms


A patient with cervicothoracic bursitis presents with the complaint of dull, poorly localized pain in the lower cervical and upper
thoracic region. The pain spreads from the midline to the adjacent
paraspinous area, but is nonradicular. The patient often holds the
cervical spine rigid, with the head thrust forward to splint the
affected ligament and bursae. Flexion and extension of the lower
cervical spine and upper thoracic spine tend to cause more pain
than rotation of the head.
The neurological examination of patients with cervicothoracic
bursitis should be normal. Focal or radicular neurological findings suggest a central or spinal cord origin of pain symptoms and
should be followed with magnetic resonance imaging (MRI) of
the appropriate anatomical regions.

Testing
MRI of the lower cervical and upper thoracic spine should be
performed in all patients thought to have cervicothoracic bursitis (Figure 22-2). Electromyography of the brachial plexus and
upper extremities is indicated if neurological findings or pain
that radiates into the arms are present. Clinical laboratory tests,

including a complete blood cell count, automated chemistry profile, antinuclear antibody testing, and erythrocyte sedimentation
rate, are indicated to rule out infection; collagen-vascular disease,
including ankylosing spondylitis; and malignancy that may mimic
the clinical presentation of cervicothoracic bursitis. Injection of
the affected interspinous bursae with local anesthetic and steroid
may serve as a diagnostic and therapeutic maneuver and may help
strengthen the diagnosis of cervicothoracic bursitis. Plain radiography of the sacroiliac joints is indicated if ankylosing spondylitis
is being considered in the differential diagnosis.

Differential Diagnosis
The diagnosis of cervicothoracic bursitis is usually made on clinical grounds as a diagnosis of exclusion. The clinician needs to rule
out intrinsic disease of the spinal cord, including syringomyelia
and tumor, which may mimic the clinical presentation of cervicothoracic bursitis. Ankylosing spondylitis also may manifest in a
manner similar to that of cervicothoracic bursitis. Fibromyalgia
may coexist with cervicothoracic bursitis and should be identifiable by its characteristic trigger points and positive jump sign.

Treatment
Initial treatment of the pain and functional disability associated with
cervicothoracic bursitis should include a combination of nonsteroidal
antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2)
inhibitors and physical therapy. The local application of heat and
cold also may be beneficial. For patients who do not respond to
these treatment modalities, the following injection technique with a
local anesthetic and steroid may be a reasonable next step.
The skin overlying the C7-T1 interspace is prepared with
antiseptic solution. A syringe containing 20 mL of 0.25% preservative-free bupivacaine and 40 mg of methylprednisolone is
attached to a 25-gauge, 1-inch needle. The needle is carefully
advanced through the supraspinal ligament into the interspinous
ligament (Figure 22-3). Care must be taken to keep the needle
in the midline and not to advance it too deeply, or inadvertent
epidural, subdural, or subarachnoid injection could occur. After
careful aspiration, a volume of 2 to 3 mL is gently injected into
the ligament. The patient should be informed that two to five
treatment sessions may be required to abolish the symptoms of
cervicothoracic bursitis completely.

Complications and Pitfalls


The proximity to the spinal cord and exiting nerve roots makes
it imperative that this procedure be performed only by clinicians
57

58 SECTION 2 Neck and Brachial Plexus Pain Syndromes

C7
T1

Figure 22-1 Patients with cervicothoracic interspinous bursitis attempt to relieve pain by assuming a position of dorsal kyphosis with a thrusting
forward of the neck.

well versed in the regional anatomy and experienced in performing injection techniques. The proximity to the vertebral artery
combined with the vascular nature of this anatomical region
makes the potential for intravascular injection high. Even small
amounts of a local anesthetic injected into the vertebral arteries
result in seizures. Given the proximity of the brain and brainstem,
ataxia after trigger point injection as a result of vascular uptake
of local anesthetic is common. Many patients also complain of a
transient increase in pain after injection in this anatomical area. If
long needles are used, pneumothorax also may occur.

Because of the proximity of the epidural, subdural, and subarachnoid space, placement of a needle too deeply could result
in inadvertent neuraxial block. Failure to recognize inadvertent
epidural, subdural, or dural puncture can result in significant
motor and sensory block with the potential for associated loss of
consciousness, hypotension, and apnea. If subdural placement is
unrecognized, and the previously mentioned doses of local anesthetics are administered, the signs and symptoms are similar to
those of subarachnoid injection, although the resulting motor and
sensory block may be spotty.

22 Cervicothoracic Interspinous Bursitis 59


HRP

Clinical Pearls
The aforementioned injection technique is extremely effective in the treatment of cervicothoracic bursitis. This technique is a safe procedure if careful attention is paid to the
clinically relevant anatomy in the areas to be injected. Care
must be taken to use sterile technique to avoid infection and
universal precautions to avoid risk to the operator. Most
side effects of the injection technique for cervicothoracic
bursitis are related to needle-induced trauma to the injection site and underlying tissues. The incidence of ecchymosis and hematoma formation can be decreased if pressure
is placed on the injection site immediately after injection.
The avoidance of overly long needles helps decrease the
incidence of trauma to underlying structures. Special care
must be taken to avoid pneumothorax given the proximity
to the underlying pleural space.
The use of physical modalities, including local heat and
gentle stretching exercises, should be introduced several
days after the patient undergoes this injection technique
for cervicothoracic bursitis. Vigorous exercises should be
avoided because they would exacerbate the symptoms.
Simple analgesics, NSAIDs, and antimyotonic agents such
as tizanidine may be used concurrently with this injection
technique.

AR

Figure 22-2 Magnetic resonance imaging (T2) of an interspinous bursa


measuring 2 2 2.5 cm between C6 and C7. (From Perka C, Schneider
SV, Buttgereit F, Matziolis G: Development of cervical interspinous bursitis
after prolonged sports trauma: a case report, Joint Bone Spine 73:118
120, 2006.)

Kyphosis

Figure 22-3 Proper needle placement for injection for treatment of cervicothoracic interspinous bursitis pain. (From Waldman SD: Atlas of pain
management injection techniques, Philadelphia, 2000, Saunders, p 33.)

SUGGESTED READINGS
Hull JJ, Tomaski SM: Osteomyelitis of the cervical spine: case report and literature
review, Otolaryngol Head Neck Surg 113:193, 1995.
Perka C, Schneider SV, Buttgereit F, Matziolis G: Development of cervical interspinous bursitis after prolonged sports trauma: a case report, Joint Bone Spine
73:118120, 2006.
Reckelhoff KE, Green MN, Kettner NW: Cervical spine osteochondroma: rare
presentation of a common lesion, J Manipulative Physiol Ther 33:711715,
2010.
Waldman SD: Cervicothoracic interspinous bursitis. In Waldman SD, editor:
Pain review, Philadelphia, 2009, Saunders, pp 238239.

Chapter 23
SCAPULOCOSTAL SYNDROME

ICD-9 CODE 726.2


ICD-10 CODE M75.80
The Clinical Syndrome
Scapulocostal syndrome is a clinical syndrome characterized by
pain and paresthesias over the medial border of the scapula that
radiate into the neck, upper triceps, chest wall, and distal upper
extremity. The pain is burning and aching. The intensity level of
pain associated with scapulocostal syndrome is moderate.
Also known as traveling salesman shoulder, the scapulocostal
syndrome is thought to be an overuse syndrome resulting from
repetitive use of the shoulder stabilizing muscles, including the
serratus anterior, levator scapulae, pectoralis minor, and rhomboid, when carrying out activities such as reaching backward over
a car seat for samples and prolonged use of the telephone cradled
between the shoulder and neck (Figure 23-1). Racquet sports also
have been implicated in the evolution of scapulocostal syndrome.

Signs and Symptoms


Physical examination reveals myofascial trigger points in the
rhomboid, infraspinatus, and subscapularis muscles. These trigger points are best shown by having the patient reach across the
chest and place his or her hand on the uninvolved shoulder. Palpation of trigger points along the medial border of the scapula
produces a positive jump sign and causes pain to radiate into the
ipsilateral upper extremity. The neurological examination of the
upper extremity is normal in scapulocostal syndrome. Untreated,
patients with scapulocostal syndrome develop decreased range of
motion of the shoulder and scapula, resulting in functional disability and pain.

Testing
Plain radiographs are indicated in all patients with scapulocostal syndrome. Based on the clinical presentation, additional tests,
including complete blood cell count, erythrocyte sedimentation
rate, and antinuclear antibody level, may be indicated. Magnetic
resonance imaging (MRI) of the shoulder is indicated if rotator
cuff tear is suspected. Radionuclide bone scanning is indicated
if metastatic disease or primary tumor involving the shoulder is

60

being considered. Chest radiographs with apical lordotic views


should be obtained if superior sulcus tumor of the lung is a possibility. Electromyography and nerve conduction velocity testing
help rule out radiculopathy, brachial plexopathy, and entrapment
neuropathy.

Differential Diagnosis
Scapulocostal syndrome is most commonly misdiagnosed as cervical radiculopathy. In contrast to cervical radiculopathy, however,
which is associated with numbness and weakness in the affected
dermatomes, the upper extremity neurological examination in
scapulocostal syndrome is normal. Osteoarthritis, rheumatoid
arthritis, posttraumatic arthritis, and rotator cuff tear arthropathy
also are common causes of shoulder pain secondary to arthritis
that may be confused with scapulocostal syndrome. Less common
causes of arthritis-induced shoulder pain include the collagenvascular diseases, infection, villonodular synovitis, and Lyme
disease. Acute infectious arthritis usually is accompanied by significant systemic symptoms, including fever and malaise, and should
be easily recognized by an astute clinician and treated appropriately with culture and antibiotics, rather than injection therapy.
The collagen-vascular diseases generally manifest as a polyarthropathy rather than a monarthropathy limited to the shoulder joint,
and the pain does not radiate into the upper extremity. Pancoast
tumor and brachial plexopathy also may mimic the clinical presentation of scapulocostal syndrome.

Treatment
Initial treatment of the pain and functional disability associated
with scapulocostal syndrome should include a combination of
nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also may be beneficial. Repetitive movements
that incite the syndrome should be avoided. For patients who do
not respond to these treatment modalities, injection of myofascial
trigger points with local anesthetic and steroid may be a reasonable next step.

Complications and Pitfalls


The major complication in the care of a patient thought to have
scapulocostal syndrome is misdiagnosis. Tumors of the superior
sulcus of the lung or primary or metastatic tumors of the shoulder
and scapula must be included in the differential diagnosis.

23 Scapulocostal Syndrome 61
SUGGESTED READINGS

Supraspinatus

Levator
scapulae

Infraspinatus

Rhomboids

Serratus
anterior

Figure 23-1 Scapulocostal syndrome is due to repetitive use of the


shoulder stabilizing muscles.

Clinical Pearls
Scapulocostal syndrome is a less common cause of shoulder
and upper extremity pain encountered in clinical practice,
with cervical radiculopathy occurring much more commonly. This painful condition must be separated from
other causes of shoulder pain, including rotator cuff tears.
Coexistent bursitis and tendinitis also may contribute to
shoulder pain and may require additional treatment with
more localized injection of local anesthetic and depot steroid. Trigger point injections are a safe procedure if careful attention is paid to the clinically relevant anatomy in
the areas to be injected. Care must be taken to use sterile
technique to avoid infection and universal precautions to
avoid risk to the operator. The incidence of ecchymosis and
hematoma formation can be decreased if pressure is placed
on the injection site immediately after injection. The use
of physical modalities, including local heat and gentle
range-of-motion exercises, should be introduced several
days after the patient undergoes trigger point injections for
scapulocostal syndrome. Avoidance of activities responsible
for the evolution of the disease must be considered or the
syndrome will recur. Vigorous exercises should be avoided
because they would exacerbate symptoms. Simple analgesics
and NSAIDs or a COX-2 inhibitor may be used concurrently with an injection technique.

Bradley LA: Pathophysiology of fibromyalgia, Am J Med 122(Suppl 1):S22S30,


2009.
Ge HU, Nie H, Madeleine P: Contribution of the local and referred pain from
active myofascial trigger points in fibromyalgia syndrome, Pain 147:233240,
2009.
Monach PA: Shoulder pain. In Mushlin SB, Greene HL II, editors: Decision making
in medicine, 3rd ed, New York, 2010, Elsevier, pp 522523.
Waldman SD: Scapulocostal syndrome. In Waldman SD, editor: Atlas of pain
management injection techniques, 2nd ed, Philadelphia, Saunders, pp 123126.

Chapter 24
PARSONAGE-TURNER SYNDROME

ICD-9 CODE 353.0


ICD-10 CODE G54.0

(e.g., Parsonage-Turner syndrome), and postradiation plexopathy. Cervical radiculopathy is a much more common cause of
upper extremity pain and weakness relative to Parsonage-Turner
syndrome. Table 24-1 differentiates these two painful conditions.
In patients in whom Parsonage-Turner syndrome affects only an

The Clinical Syndrome


Parsonage and Turner described the painful condition of the
shoulder and upper extremity that bears their name and was
first identified as a distinct clinical entity in 1948. The pain of
Parsonage-Turner syndrome is of acute onset and is severe in
intensity. The pain is burning and involves the shoulder and
upper arm, preceding the onset of muscle weakness by hours to
days (Figure 24-1). Sleep disturbance is common, and weakness
of the muscles of the shoulder and upper extremity, including
the deltoid, infraspinatus, supraspinatus, and biceps, occurs as
the syndrome progresses. In some patients, this weakness can be
severe, progressing to complete flaccidity. A viral cause of Parsonage-Turner syndrome has been suggested, as has the belief that
this painful condition is an immunological disease. Neither theory
has been proved.

Weakness in:
Supraspinatus
Deltoid
Infraspinatus

Signs and Symptoms


Patients with Parsonage-Turner syndrome first experience a sudden
onset of pain that begins in the shoulder and radiates down the arm.
The pain is severe and is followed by the development of weakness. The skin examination is normal, with no evidence of acute
herpes zoster. Range of motion of the cervical spine generally does
not affect the pain or numbness, in contrast to cervical radiculopathy. Weakness of the muscles of the shoulder and upper extremity, including the deltoid, infraspinatus, supraspinatus, and biceps,
increases as the syndrome progresses. Flaccidity of these muscles
may occur. Usually, more than one portion of the brachial plexus
is affected, although isolated single nerve involvement can occur.

Biceps

Differential Diagnosis
Brachial plexopathy has many causes. In common to all of them is
the constellation of symptoms consisting of neurogenic pain and
associated weakness that radiates into the supraclavicular region
and upper extremity. More common causes of brachial plexopathy include compression of the plexus by cervical ribs or abnormal muscles (e.g., thoracic outlet syndrome), invasion of the
plexus by tumor (e.g., Pancoast syndrome), direct trauma to the
plexus (e.g., stretch injuries and avulsions), inflammatory causes
62

Figure 24-1 The pain of Parsonage-Turner syndrome involves the


shoulder and upper arm, preceding the onset of muscle weakness by
hours to days.

24 Parsonage-Turner Syndrome 63

isolated nerve, the syndrome may be misdiagnosed as entrapment


neuropathy. Electromyography is the cornerstone in sorting out
the differential diagnosis in patients with the acute onset of shoulder and upper extremity pain.
Diseases of the cervical spinal cord, bony cervical spine, and
disc can mimic Parsonage-Turner syndrome. Appropriate testing,
including magnetic resonance imaging (MRI) and electromyography, helps sort out the myriad possibilities, but the clinician
also should be aware that more than one pathological process may
coexist and contribute to the patients symptoms. Syringomyelia,
tumors of the cervical spinal cord, and tumors of the cervical nerve
roots as they exit the spinal cord, such as schwannomas, can be of
insidious onset and quite difficult to diagnose. Pancoast tumor
should be high on the list of diagnostic possibilities in all patients
with brachial plexopathy in the absence of clear antecedent
trauma, especially in the presence of a history of tobacco abuse.
Lateral herniated cervical disc, metastatic tumor, or cervical spondylosis that results in significant nerve root compression also may
manifest as a brachial plexopathy. Rarely, infection involving the
apex of the lung may compress and irritate the plexus.

Testing
All patients presenting with Parsonage-Turner syndrome must
undergo MRI of the cervical spine and the brachial plexus (Figure
24-2). Computed tomography (CT) is a reasonable second choice
if MRI is contraindicated. Electromyography and nerve conduction velocity testing are extremely sensitive, and a skilled electromyographer can help delineate the specific portion of the plexus
that is abnormal. If an inflammatory basis for the plexopathy is
suspected, serial electromyography is indicated. If Pancoast tumor
or other tumors of the brachial plexus are suspected, chest radiographs with apical lordotic views may be helpful.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, antinuclear antibody testing, and automated blood chemistry testing should be performed
if the diagnosis of brachial plexopathy is in question, to help rule
out other causes of pain.

Treatment
Pharmacological Therapy
Gabapentin
Gabapentin is the first-line treatment for the neuritic pain of
Parsonage-Turner syndrome. The drug is started with a 300-mg
dose at bedtime for 2 nights; the patient should be cautioned about
potential side effects, including dizziness, sedation, confusion,

and rash. The drug is increased in 300-mg increments, given in


equally divided doses over 2 days, as side effects allow, until pain
relief is obtained or a total dose of 2400 mg per day is reached. At
this point, if the patient has experienced partial pain relief, blood
values are measured, and the drug is carefully titrated upward
using 100-mg tablets. More than 3600 mg daily rarely is required.
Carbamazepine
Carbamazepine is useful in patients with Parsonage-Turner syndrome who do not experience pain relief with gabapentin. Despite
the safety and efficacy of carbamazepine compared with other
treatments for Parsonage-Turner syndrome, much confusion and
unfounded anxiety surround its use. This medication, which may
be the best chance for pain control, is sometimes discontinued
because of laboratory abnormalities erroneously attributed to it.
Baseline screening laboratory tests, consisting of a complete blood
cell count, urinalysis, and automated chemistry profile, should be
obtained before starting the drug.
Carbamazepine should be started slowly if the pain is not out
of control. The drug is started with a 100- to 200-mg dose at bedtime for 2 nights; the patient should be cautioned regarding side
effects, including dizziness, sedation, confusion, and rash. The
drug is increased in 100- to 200-mg increments, given in equally
divided doses over 2 days, as side effects allow, until pain relief
is obtained or a total dose of 1200 mg daily is reached. Careful
monitoring of laboratory parameters is mandatory to avoid the
rare possibility of life-threatening blood dyscrasia. At the first sign
of blood count abnormality or rash, carbamazepine should be discontinued. Failure to monitor patients started on carbamazepine
can be disastrous because aplastic anemia can occur. When pain
relief is obtained, the patient should be kept at that dosage of
carbamazepine for at least 6 months before considering tapering
of this medication. The patient should be informed that under no
circumstances should the dosage of drug be changed or the drug
refilled or discontinued without the physicians knowledge.
Baclofen
Baclofen has been reported to be valuable in some patients who
fail to obtain relief from gabapentin and carbamazepine. Baseline
laboratory tests should be obtained before starting baclofen. The
drug is started with a 10-mg dose at bedtime for 2 nights; the
patient should be cautioned about potential adverse effects, which
are the same as those of carbamazepine and gabapentin.
Baclofen is increased in 10-mg increments, given in equally
divided doses over 7 days, as side effects allow, until pain relief is
obtained or a total dose of 80 mg per day is reached. This drug
has significant hepatic and central nervous system side effects,
including weakness and sedation. As with carbamazepine, careful

TABLE 24-1

Comparison of Parsonage-Turner Syndrome and Cervical Radiculopathy


Disease

History

Examination

Test Results

Parsonage-Turner
syndrome

Acute, intense burning pain that begins


spontaneously in shoulder and upper arm;
pain unaffected by neck movement

Neurological deficits that suggest


more than one nerve is involved;
weakness that may progress to
flaccidity

Electromyogram positive for


brachial plexopathy; MRI of
cervical spine noncontributory
to diagnosis

Cervical
radiculopathy

Pain begins in neck and radiates down


arm; the pain is increased by neck movement; pain and muscle weakness occur
spontaneously

Weakness and numbness in the


distribution of a single nerve root

MRI of cervical spine reveals


herniated disc or osteophyte
formation or both

MRI, Magnetic resonance imaging.

64 SECTION 2 Neck and Brachial Plexus Pain Syndromes

Figure 24-2 Pancoast tumor in a 46-year-old man. Sagittal T1-weighted (A) and sagittal T1-weighted gadolinium-enhanced with fat saturation (B)
sequences show left apical bronchogenic carcinoma (white arrow) invading the supraclavicular fossa, involving the brachial plexus, and encasing the
subclavian artery (black arrow). (From Knisely BL, Broderick LS, Kuhlman JE: MR imaging of the pleura and chest wall, MRI Clin North Am 8:125, 2000.)

monitoring of laboratory values is indicated during the initial use


of this drug.
When treating patients with any of the drugs mentioned, the
physician should inform the patient that premature tapering or
discontinuation of the medication may lead to the recurrence of
pain. The pain becomes more difficult to control thereafter.
Interventional Treatment
Brachial Plexus Block
The use of brachial plexus block with a local anesthetic and steroid
is an excellent adjunct to drug treatment of Parsonage-Turner
syndrome. This technique rapidly relieves pain while medications
are being titrated to effective levels. The initial block is performed
with preservative-free bupivacaine combined with methylprednisolone. Subsequent daily nerve blocks are done in a similar manner,
substituting a lower dose of methylprednisolone. This approach
also may be used to obtain control of breakthrough pain.
Physical Modalities
The use of physical and occupational therapy to maintain function and help palliate pain is a crucial part of the treatment plan
for patients with Parsonage-Turner syndrome. Shoulder abnormalities, including subluxation and adhesive capsulitis, must be
aggressively searched for and treated. Occupational therapy to

assist in activities of daily living also is important to avoid further


deterioration of function.

Complications and Pitfalls


The pain of Parsonage-Turner syndrome is difficult to treat. It
responds poorly to opioid analgesics and may respond poorly to
the previously mentioned medications. The uncontrolled pain
of Parsonage-Turner syndrome has led to suicide, and hospitalization of such patients should be strongly considered. Correct
diagnosis is crucial to successfully treat the pain and dysfunction
associated with brachial plexopathy because stretch injuries and
contusions of the plexus may respond with time, but plexopathy secondary to tumor or avulsion of the cervical roots requires
aggressive treatment.

Clinical Pearls
Brachial plexus block with a local anesthetic and steroid
represents an excellent stop-gap measure for patients with
the uncontrolled pain of Parsonage-Turner syndrome while
waiting for pharmacological treatments to take effect. As
mentioned, correct diagnosis is paramount to allow the clinician to design a logical treatment plan.

24 Parsonage-Turner Syndrome 65
SUGGESTED READINGS
Marshall GB, McKenna E, Mahallati H: ParsonageTurner syndrome, Eur J
Radiol Extra 6:5153, 2005.
Mileto A, Gaeta M: Calcific tendonitis of supraspinatus simulating acute brachial
neuritis (Parsonage-Turner syndrome), Clin Radiol 66:578581, 2011.

Stutz CM: Neuralgic amyotrophy: Parsonage-Turner syndrome, J Hand Surg


35:21042106, 2010.
Wendling D, Sevrin P, Bouchaud-Chabot A, etal: ParsonageTurner syndrome
revealing Lyme borreliosis, Joint Bone Spine 76:202204, 2009.

Chapter 25
HYOID SYNDROME

ICD-9 CODE 728.89


ICD-10 CODE M62.89
The Clinical Syndrome
Hyoid syndrome is caused by calcification and inflammation of
the attachment of the stylohyoid ligament to the hyoid bone. The
stylohyoid ligaments cephalad attachment is to the styloid process, and its caudal attachment is to the hyoid bone. Tendinitis
of the other muscular attachments to the hyoid bone also may
contribute to this painful condition. Hyoid syndrome also may be
seen in conjunction with Eagles syndrome (see Chapter 14). The
pain of hyoid syndrome is sharp and stabbing and occurs with
movement of the mandible, turning of the neck, or swallowing.

should be high on the list of diagnostic possibilities if the history


of trauma is weak or absent. Although clinically similar, glossopharyngeal neuralgia can be distinguished from hyoid syndrome
in that the pain of glossopharyngeal neuralgia is characterized by
paroxysms of shocklike pain in a manner analogous to trigeminal neuralgia, rather than the sharp, shooting pain that occurs on
movement associated with hyoid syndrome. Because glossopharyngeal neuralgia may be associated with serious cardiac bradyarrhythmias and syncope, the clinician must distinguish the two
syndromes.

Treatment

Magnetic resonance imaging (MRI) of the soft tissues of the neck


may reveal calcification, inflammation, or both of the caudad
attachment of the stylohyoid ligament at the hyoid bone. Injection of the ligament with local anesthetic can serve as a diagnostic
maneuver to help strengthen the diagnosis.

Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the
treatment of hyoid syndrome. The use of tricyclic antidepressants,
such as nortriptyline, at a single bedtime dose of 25 mg titrating
as side effects allow also is useful, especially if sleep disturbance is
present. If symptoms persist, injection of the caudad attachment
of the stylohyoid ligament is a reasonable next step.
To perform this injection, the patient is placed in the supine
position. The angle of the mandible on the affected side is identified. The greater cornu of the hyoid bone should lie approximately
1 inch inferior to the angle of the mandible. Gentle pressure at the
same point on the contralateral side of the neck steadies the hyoid
bone and makes identification of the greater cornu and subsequent
injection easier (Figure 25-2). The skin is prepared with antiseptic
solution. A 22-gauge, 112-inch needle attached to a 10-mL syringe
is advanced at this point 1 inch inferior to the angle of the mandible in a plane perpendicular to the skin. The greater cornu of
the hyoid bone should be encountered within 2.5 to 3 cm (Figure 25-3). After contact is made, the needle is withdrawn slightly
out of the periosteum or substance of the calcified ligament. After
careful aspiration reveals no blood or cerebrospinal fluid, 5 mL of
0.5% preservative-free lidocaine combined with 80 mg of methylprednisolone is injected in incremental doses. Subsequent daily
nerve blocks are done in a similar manner, substituting 40 mg of
methylprednisolone for the initial 80-mg dose.

Differential Diagnosis

Complications and Pitfalls

Soft tissue injuries to the region may mimic styloid syndrome.


Because trauma is invariably involved in the evolution of the painful condition, the strain and sprain of other soft tissues, such as
omohyoid syndrome, often exist concurrently with hyoid syndrome (see Chapter 26). Primary or metastatic tumors of the neck
and hypopharynx and mass effect from thyroglossal duct cyst
also may mimic the clinical presentation of hyoid syndrome and

The major complication in the treatment of patients thought to


have hyoid syndrome is wrong diagnosis. Occult cervical spine fracture or instability after trauma remains an ever-present possibility.
Failure to diagnose such injuries can put the patient at significant
risk for permanent neurological sequelae. As mentioned earlier, if
the patient is thought to have a history of trauma, the diagnosis of
hyoid syndrome should become one of exclusion. A careful search

Signs and Symptoms


The pain of hyoid syndrome starts below the angle of the mandible and radiates into the anterolateral neck. It is triggered or worsened with chewing, rotation of the cervical spine, and swallowing
(Figure 25-1). The pain of hyoid syndrome is sharp and stabbing
and often is referred to the ipsilateral ear. Some patients also may
complain of a foreign body sensation in the pharynx. Injection of
the attachment of the stylohyoid ligament to the greater cornu of
hyoid bone with local anesthetic and steroid serves as a diagnostic
and therapeutic maneuver.

Testing

66

25 Hyoid Syndrome 67

Styloid process

Hyoid bone

Inflamed and calcified


stylohyoid ligament

Figure 25-1 The pain of hyoid syndrome starts below the angle of the mandible and radiates into the anterolateral neck. It is triggered or worsened
with chewing, rotation of the cervical spine, or swallowing.

Hyoid
bone

Gentle pressure
on greater cornu

Internal External
carotid carotid
artery artery

Trachea

Internal
jugular
vein
Carrico & Shavell

Figure 25-3 Injection technique for relieving the pain of hyoid syndrome. (From Waldman SD: Atlas of pain management injection techniques, 2nd ed, Philadelphia, 2007, Saunders, p 17.)

Figure 25-2 Identification of the greater cornu of the hyoid bone. (From
Waldman SD: Atlas of pain management injection techniques, 2nd ed,
Philadelphia, 2007, Saunders, p 18.)

68 SECTION 2 Neck and Brachial Plexus Pain Syndromes

for tumors of the neck, apex of the lung, anterior triangle of the
neck, and hypopharynx is indicated. If a significant history of vomiting is ascertained, esophageal tear should be considered.
Although the injection technique for hyoid syndrome is safe,
complications can occur. In addition to the potential for complications involving the vasculature, if the needle is placed too
laterally, the proximity of the brachial plexus, the central neuraxial
structures, and the phrenic nerve can result in side effects and complications. Although these complications should be rare if proper
technique is observed, the potential for inadvertent epidural, subdural, or subarachnoid injection remains. Phrenic nerve block also
can occur when using this injection technique to treat hyoid syndrome if the needle placement is too posterolateral. In the absence
of significant pulmonary disease, unilateral phrenic nerve block
should rarely create respiratory embarrassment. Blockade of the
recurrent laryngeal nerve with its attendant vocal cord paralysis
combined with paralysis of the diaphragm may make the clearing
of pulmonary and upper airway secretions difficult. Because of the
proximity of the apex of the lung, pneumothorax is a distinct possibility, and the patient should be informed of this.

Clinical Pearls
The clinician should always evaluate a patient who has pain
in this anatomical region for occult malignancy. Tumors of
the larynx, hypopharynx, and anterior triangle of the neck
may manifest clinical symptoms identical to those of hyoid
syndrome. Given the low incidence of hyoid syndrome
relative to pain secondary to malignancy in this anatomical
region, hyoid syndrome must be considered a diagnosis of
exclusion.
The injection technique described for hyoid syndrome
is a simple technique that can produce dramatic relief for
patients with the previously mentioned pain problems.
As discussed earlier, the proximity of the greater cornu
of the hyoid bone to major vasculature makes postblock
hematoma and ecchymosis a distinct possibility. Although
these complications are usually transitory, their dramatic
appearance can be quite upsetting to the patient; therefore
the patient should be warned of this possibility before the
procedure. The vascularity of this region also increases the
incidence of inadvertent intravascular injection. Even small
amounts of local anesthetic injected into the carotid artery
at this level can result in local anesthetic toxicity and seizures. Incremental dosing while carefully monitoring the
patient for signs of local anesthetic toxicity helps avoid this
complication.

SUGGESTED READINGS
Auvenshine RC: Anatomy of the airway: an overview, Sleep Med Clin 5:4557,
2010.
Ernest EA III, Salter EG: Hyoid bone syndrome: a degenerative injury of the middle pharyngeal constrictor muscle with photomicroscopic evidence of insertion
tendinosis, J Prosthet Dentist 66:7883, 1991.
Nir D, Hefer T, Joachims HZ: Hyoid bone syndrome and its treatment with nonsteroidal anti-inflammatory drugs, Am J Otolaryngol 19:296300, 1998.
Waldman SD: Hyoid syndrome. In Atlas of pain management injection techniques,
2nd ed, Philadelphia, 2007, Saunders. pp 1619.

Chapter 26
OMOHYOID SYNDROME

ICD-9 CODE 726.2


ICD-10 CODE M79.7
The Clinical Syndrome
Trauma is the common denominator in patients with omohyoid
syndrome. The syndrome is most often seen in patients who have
recently experienced a bout of intense vomiting or sustained a flexion/extension injury to the cervical spine and the musculature of
the anterior neck. The pain of omohyoid syndrome is the result of
damage to the fibers of the inferior belly of the omohyoid muscle.
This pain manifests as myofascial. It is constant and exacerbated
with movement of the affected muscle. A trigger point in the inferior belly of the omohyoid muscle is often present and provides
a basis for treatment. The pain of omohyoid syndrome starts just
above the clavicle at the lateral aspect of the clavicular attachment
of the sternocleidomastoid muscle. The pain may radiate into the
anterolateral neck. Injection of the trigger point in the inferior
muscle of the omohyoid muscle with local anesthetic and steroid
serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis
Soft tissue injuries to the region may mimic omohyoid syndrome.
Because trauma is invariably involved in the evolution of the painful
condition, strain and sprain of other soft tissues often exist concurrently with omohyoid syndrome. Primary or metastatic tumors of
the neck and hypopharynx also may mimic the clinical presentation
of omohyoid syndrome and should be high on the list of diagnostic
possibilities if the history of trauma is weak or absent.

Treatment
Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase2 (COX-2) inhibitors represent a reasonable first step in the treatment
of omohyoid syndrome. The use of tricyclic antidepressants, such
as nortriptyline, at a single bedtime dose of 25 mg, titrating upward
as side effects allow also is helpful, especially if sleep disturbance

Signs and Symptoms


A patient with omohyoid syndrome presents with pain in the
supraclavicular region at a point just lateral and superior to the
attachment of the sternocleidomastoid muscle to the clavicle
(Figure 26-1). The pain often radiates into the anterolateral neck
and increases with movement of the omohyoid muscle. A baseline level of pain is present even without movement of the muscle. The pain intensity ranges from minor to moderate. A trigger
point in the belly of the omohyoid muscle is often present. The
pain of omohyoid syndrome is often exacerbated by swallowing.
The neurological examination of a patient with omohyoid syndrome is normal, unless trauma has occurred to the cervical nerve
roots or brachial plexus.

Testing
Magnetic resonance imaging (MRI) of the soft tissues of the neck
may reveal hematoma formation of the omohyoid muscle acutely
and calcification, fibrosis, or both as the syndrome becomes more
chronic. Injection of the belly of the omohyoid muscle with local
anesthetic can serve as a diagnostic maneuver to help strengthen
the diagnosis.

Figure 26-1 The pain of omohyoid syndrome is localized in the supraclavicular region at a point just lateral and superior to the attachment of
the sternocleidomastoid muscle to the clavicle.

69

70 SECTION 2 Neck and Brachial Plexus Pain Syndromes

Complications and Pitfalls

Site of
injection

Omohyoid muscle
(superior belly)
Sternocleidomastoid
muscle:
Sternal head

1"

Clavicular head
Clavicle
Omohyoid muscle
(inferior belly)
Figure 26-2 Injection site for the treatment of omohyoid syndrome.

is present. The injection of trigger points in the inferior belly of


the omohyoid muscle often produces dramatic improvement in
pain symptoms.
The key landmark for injecting when treating omohyoid
syndrome is the lateral aspect of the clavicular head of the sternocleidomastoid muscle (Figure 26-2). The omohyoid muscle
is located slightly lateral and deep to the clavicular head of the
sternocleidomastoid muscle approximately to 1 inch above the
superior margin of the clavicle. Given the relationship of the great
vessels of the neck to the omohyoid muscle, care must be taken
when placing needles in this anatomical area.
The patient is placed in the supine position, with the head
turned away from the side to be blocked. Using a 5-mL sterile syringe, 3 mL of local anesthetic is drawn up. When treating omohyoid syndrome, 80 mg of depot steroid is added to the
local anesthetic with the first block and 40 mg of depot steroid
is added with subsequent blocks. The patient is asked to raise the
head against the resistance of the pain specialists hand to aid in
identification of the posterior border of the sternocleidomastoid
muscle. The point at which the lateral border of the sternocleidomastoid attaches to the clavicle is identified. At this point, slightly
lateral and approximately 1 inch above the clavicle, after preparation of the skin with antiseptic solution, a 1-inch needle is
inserted directly perpendicular to the table top (see Figure 26-2).
The needle should be advanced slowly because of proximity of the
great vessels and brachial plexus. A pop often is felt as the fascia
of the omohyoid muscle is pierced; this should occur at a depth
of to of an inch. If strict attention to technique is observed,
and the needle is not placed or directed too laterally, the brachial
plexus should not be encountered. Because of the proximity of
the brachial plexus, the patient should be warned that a paresthesia could occur, however; the patient should be instructed to
say There! if a paresthesia is felt. The needle should never be
directed in a more inferior medial trajectory because pneumothorax is likely to occur.
After the muscle is identified, gentle aspiration is done to identify blood or cerebrospinal fluid. If the aspiration test is negative,
and no paresthesia into the distribution of the brachial plexus is
encountered, 3 mL of solution is slowly injected, with the patient
being monitored closely for signs of local anesthetic toxicity or
inadvertent neuraxial injection.

The major complication in the treatment of patients thought to


have omohyoid syndrome is wrong diagnosis. Occult cervical
spine fractures or instability after trauma remain an ever-present
possibility. Failure to diagnose such injuries can put the patient at
significant risk for permanent neurological sequelae. As mentioned
earlier, if the history of trauma is suspect, the diagnosis of omohyoid syndrome should become one of exclusion. A careful search for
tumors of the neck, apex of the lung, anterior triangle of the neck,
and hypopharynx is indicated. If a significant history of vomiting is
ascertained, esophageal tear also should be considered.
Although the injection technique for omohyoid syndrome is safe,
complications can occur. In addition to the potential for complications involving the vasculature, if the needle is placed too laterally,
the proximity of the brachial plexus, the central neuraxial structures,
and the phrenic nerve can result in side effects and complications.
Although these complications should be rare if proper technique is
observed, the potential for inadvertent epidural, subdural, or subarachnoid injection remains a possibility. Phrenic nerve block also
can occur when using this injection technique to treat omohyoid
syndrome if the needle placement is too far posterolaterally. In the
absence of significant pulmonary disease, unilateral phrenic nerve
block should rarely create respiratory embarrassment. Blockade of
the recurrent laryngeal nerve with its attendant vocal cord paralysis
combined with paralysis of the diaphragm may make the clearing of
pulmonary and upper airway secretions difficult, however. Because
of the proximity of the apex of the lung, pneumothorax is a distinct
possibility and the patient should be informed of this.

Clinical Pearls
Although an uncommon cause of pain, omohyoid syndrome
is a clinically distinct and easily recognizable pain syndrome.
Because of its excellent response to the injection technique
described, the diagnosis of omohyoid syndrome should be
considered in the presence of a history of trauma or after
prolonged or forceful vomiting. If the patient has severe,
acute pain after vomiting, esophageal tear is a more likely
diagnosis. More chronic pain after a significant episode of
vomiting is more likely to indicate omohyoid syndrome.
The key to performing this injection technique safely is a
clear understanding of the anatomy and careful identification
of the anatomical landmarks necessary to perform the block.
The brachial plexus is quite superficial at the level at which
this block is performed. The needle should rarely be inserted
deeper than of an inch in all but the most obese patients.
If strict adherence to technique is observed, and the needle is
never advanced medially from the lateral border of the insertion of the sternocleidomastoid muscle on the clavicle, the
incidence of pneumothorax should be less than 0.5%.
In the absence of well-documented trauma to the anterior neck, omohyoid syndrome is a diagnosis of exclusion.
The clinician should always evaluate a patient with pain in
this anatomical region for occult malignancy. Tumors of
the larynx, hypopharynx, and anterior triangle of the neck
may manifest with clinical symptoms identical to omohyoid syndrome. In the setting of flexion/extension injuries or
other forceful trauma to the soft tissues of the neck, cervical
spine, or both, the clinician also should evaluate the patient
for trauma to the brachial plexus by careful physical examination and electromyography.

26 Omohyoid Syndrome 71
SUGGESTED READINGS
Bradley LA: Pathophysiology of fibromyalgia, Am J Med 122(Suppl 1):S22S30,
2009.
Ge HU, Nie H, Madeleine P, et al: Contribution of the local and referred
pain from active myofascial trigger points in fibromyalgia syndrome, Pain
147:233240, 2009.

Waldman SD: Omohyoid syndrome. In Waldman SD, editor: Atlas of pain management injection techniques, ed 2, Philadelphia, 2007, Saunders, pp 2931.
Wong DSY, Li HJC: The omohyoid sling syndrome, Am J Otolaryngol 21:318322,
2000.

Chapter 27
NECK-TONGUE SYNDROME

ICD-9 CODE 729.2


ICD-10 CODE M79.2
The Clinical Syndrome
Neck-tongue syndrome is a rare condition characterized by pain
in the neck associated with numbness of the ipsilateral half of the
tongue that is aggravated by movement of the upper cervical spine.
This unusual constellation of symptoms is thought to be due to
compression of the C2 nerve root by compromise of the atlantoaxial joint. This compression can be caused by joint instability that allows subluxation of the lateral joint, bony abnormality
such as congenital fusions or stenosis, or tubercular infection. The
tongue numbness is thought to be due to damage or intermittent
compression of the lingual afferent fibers that pass via the hypoglossal nerve to innervate the tongue. The bulk of the fibers are
proprioceptive, and patients with neck-tongue syndrome also may
exhibit pseudoathetosis of the tongue. Neck-tongue syndrome
occurs most commonly in patients older than 50 years, although
the syndrome has been reported in a few pediatric patients.

Signs and Symptoms


The pain of neck-tongue syndrome is in the distribution of the C2
nerve root. It is intermittent, but is reproducible with certain neck
movements. The physical findings associated with this pain are ill
defined, with some patients with neck-tongue syndrome exhibiting a decreased range of motion of the cervical spine or tenderness
of the upper paraspinous musculature. The main objective finding
in neck-tongue syndrome is decreased sensation of the ipsilateral
half of the tongue (Figure 27-1). Often associated with this finding are pseudoathetoid movements of the tongue resulting from
an impairment of the proprioceptive fibers.

Testing
Magnetic resonance imaging (MRI) of the brain and brainstem
should be performed in all patients thought to have neck-tongue
syndrome. MRI of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and
helps identify abnormalities that may put the patient at risk for
neurological disasters secondary to intracranial and brainstem
pathology, including tumors and demyelinating disease. Magnetic
resonance angiography (MRA) may be useful to help identify
72

aneurysms responsible for the patients neurological symptoms.


In patients who cannot undergo MRI, such as patients with
pacemakers, computed tomography (CT) is a reasonable second
choice.
Clinical laboratory tests consisting of a complete blood cell
count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection, temporal arteritis, and
malignancy that may mimic neck-tongue syndrome. Endoscopy
of the hypopharynx with special attention to the piriform sinuses
also is indicated to rule out occult malignancy. Differential neural
blockade of the C2 nerve may help strengthen the diagnosis of
neck-tongue syndrome.

Differential Diagnosis
Neck-tongue syndrome is a clinical diagnosis that can be made on
the basis of a targeted history and physical examination. Because
of the rarity of this syndrome, the clinician must consider necktongue syndrome to be a diagnosis of exclusion. Diseases of the
eyes, ears, nose, throat, and teeth may coexist and confuse the
diagnosis. Tumors of the hypopharynx, including the tonsillar
fossa and piriform sinus, may mimic the pain of neck-tongue
syndrome, as may tumors at the cerebellopontine angle. Occasionally, demyelinating disease may produce a clinical syndrome
identical to neck-tongue syndrome. The jaw claudication associated with temporal arteritis also may confuse the clinical picture,
as may glossopharyngeal neuralgia.

Treatment
The initial treatment of neck-tongue syndrome should consist
of immobilization of the cervical spine with a soft cervical collar. A trial of nonsteroidal antiinflammatory drugs (NSAIDs) or
cyclooxygenase-2 (COX-2) inhibitors represents a reasonable next
step. Blockade of the atlantoaxial joint and C2 nerve root with a
local anesthetic and steroid also should be considered. For refractory cases, cervical fusion of the upper cervical segments may be
required.

Complications and Pitfalls


Because of the rarity of neck-tongue syndrome, it is often misdiagnosed. Further complicating the confusion surrounding the
diagnosis of this painful condition is the fact that many of the
pathological processes that mimic neck-tongue syndrome are also
difficult to diagnose, especially diseases of the hypopharynx. For
these reasons, the diagnosis of neck-tongue syndrome should be
approached with extreme caution.

27 Neck-Tongue Syndrome 73

C1 nerve root
C1 (Atlas)
Atlantoaxial joint
C2 nerve root

C2 (Axis)

Numbness

Hypoglossal nerve

Figure 27-1 The pain and numbness of the ipsilateral half of the tongue are aggravated by movement of the upper cervical spine.

Clinical Pearls
Neck-tongue syndrome is a unique and uncommon cause
of neck pain. The associated ipsilateral tongue numbness is
pathognomonic for the syndrome and is unusual in character. An analogous type of proprioceptive numbness is seen
in patients with Bells palsy. Given the rarity of this painful condition, the clinician should search carefully for other
causes of the symptoms before attributing them to necktongue syndrome.

SUGGESTED READINGS
Bogduk N: An anatomical basis for the neck-tongue syndrome, J Neurol Neurosurg
Psychiatry 44:202208, 1981.
Borody C: Neck-tongue syndrome, J Manipulative Physiol Ther 27:367, 2004.
Chedrawi AK, Fishman MA, Miller G: Neck-tongue syndrome, Pediatr Neurol
22:397399, 2000.
Orrell RW, Marsden CD: The neck-tongue syndrome, J Neurol Neurosurg Psychiatry 57:348352, 1994.

SECTION 3 Shoulder Pain Syndromes

Chapter 28
SUPRASPINATUS TENDINITIS

ICD-9 CODE 726.10


ICD-10 CODE M75.10
The Clinical Syndrome
Supraspinatus tendinitis can manifest as an acute or chronic painful condition of the shoulder. Acute supraspinatus tendinitis
usually occurs in younger patients after overuse or misuse of the
shoulder joint. Inciting factors may include carrying heavy loads
in front of and away from the body, throwing injuries, or the vigorous use of exercise equipment. Chronic supraspinatus tendinitis
tends to occur in older patients and to manifest in a more gradual
or insidious manner, without a single specific event of antecedent trauma. The pain of supraspinatus tendinitis is constant and
severe, with sleep disturbance often reported. The pain of supraspinatus tendinitis is felt primarily in the deltoid region. It is moderate to severe and may be associated with a gradual loss of range
of motion of the affected shoulder. The patient often awakens at
night when he or she rolls over onto the affected shoulder.

Signs and Symptoms


A patient with supraspinatus tendinitis may attempt to splint the
inflamed tendon by elevating the scapula to remove tension from
the ligament, giving the patient a shrugging appearance (Figure
28-1). Point tenderness is usually present over the greater tuberosity. The patient exhibits a painful arc of abduction and complains
of a catch or sudden onset of pain in the midrange of the arc
resulting from impingement of the humeral head onto the supraspinatus tendon. A patient with supraspinatus tendinitis exhibits
a positive Dawbarns sign, which is pain to palpation over the
greater tuberosity of the humerus when the arm is hanging down
that disappears when the arm is fully abducted. Early in the course
of the disease, passive range of motion is full and without pain.
As the disease progresses, patients often experience a gradual
decrease in functional ability with decreasing shoulder range of
74

motion, making simple everyday tasks, such as combing hair, fastening a brassiere, or reaching overhead, quite difficult. With continued
disuse, muscle wasting may occur and a frozen shoulder may develop.

Testing
Plain radiographs are indicated in all patients who present with
shoulder pain. Based on the patients clinical presentation, additional testing, including complete blood cell count, sedimentation rate, and antinuclear antibody testing, may be indicated.
Magnetic resonance imaging (MRI) of the shoulder is indicated
if rotator cuff tear is suspected and to confirm the diagnosis of
supraspinatus tendinitis (Figure 28-2). The injection technique
described here serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis
Because supraspinatus tendinitis may occur after seemingly minor
trauma or develop gradually over time, the diagnosis often is
delayed. Tendinitis of the musculotendinous unit of the shoulder
frequently coexists with bursitis of the associated bursae of the
shoulder joint, creating additional pain and functional disability.
This ongoing pain and functional disability can cause the patient
to splint the shoulder group with resultant abnormal movement
of the shoulder, which puts additional stress on the rotator cuff.
This stress can lead to further trauma to the entire rotator cuff.
With rotator cuff tears, passive range of motion is normal but
active range of motion is limited, in contrast to frozen shoulder,
in which passive and active range of motion are limited. Rotator
cuff tear rarely occurs before age 40 except in cases of severe acute
trauma to the shoulder. Cervical radiculopathy rarely may cause
pain limited to the shoulder, although in most instances, associated neck and upper extremity pain and numbness are present.

Treatment
Initial treatment of the pain and functional disability associated
with supraspinatus tendinitis should include a combination of
nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local

28 Supraspinatus Tendinitis 75
Supraspinatus
tendon

Figure 28-1 Patients with supraspinatus tendinitis exhibit point tenderness of the greater tuberosity and a painful arc of abduction.

Figure 28-2 Tendinosis or tendinopathy of the rotator cuff. Coronal


oblique protein densityweighted (TR/TE, 2000/25) spin echo magnetic
resonance imaging reveals increased signal intensity in the distal part of
the supraspinatus tendon (arrows). No further increase in signal intensity in T2-weighted spin echo MRI was seen. The peribursal fat plane is
intact. (From Kjellin I, Ho CP, Cervilla V, etal: Alterations in the supraspinatus tendon at MR imaging: correlation with histopathologic findings in
cadavers, Radiology 181:837, 1991.)

application of heat and cold also may be beneficial. For patients


who do not respond to these treatment modalities, the following injection technique may be a reasonable next step. The use
of physical therapy, including gentle range-of-motion exercises,
should be introduced several days after the patient undergoes this
injection technique for shoulder pain. Vigorous exercises should
be avoided because they would exacerbate the symptoms.
To inject the supraspinatus tendon, the patient is placed in
the supine position, with the forearm medially rotated behind the
back. This positioning of the upper extremity places the lateral
epicondyle of the elbow in an anterior position and makes its
identification easier. After identification of the lateral epicondyle
of the elbow, the humerus is traced superiorly to the anterior edge
of the acromion. A slight indentation just below the anterior edge

of the acromion marks the point of insertion of the supraspinatus tendon into the upper facet of the greater tuberosity of the
humerus. The point is marked with a sterile marker.
Proper preparation with antiseptic solution of the skin overlying the shoulder, subacromial region, and joint space is carried
out. A sterile syringe containing 1 mL of 0.25% preservative-free
bupivacaine and 40 mg of methylprednisolone is attached to a
25-gauge, 1-inch needle using strict aseptic technique. With
strict aseptic technique, the previously marked point is palpated,
and the indentation indicating the insertion of the supraspinatus
tendon is identified again with the gloved finger. The needle is
carefully advanced perpendicularly at this point through the skin
and subcutaneous tissues and through the joint capsule until it
impinges on bone (Figure 28-3). The needle is withdrawn 1 to
2 mm out of the periosteum of the humerus, and the contents
of the syringe are gently injected. Slight resistance to injection
should be felt. If no resistance is encountered, either the needle tip
is in the joint space itself or the supraspinatus tendon is ruptured.
If significant resistance to injection is detected, the needle tip is
probably in the substance of a ligament or tendon and should
be advanced or withdrawn slightly until the injection proceeds
without significant resistance. The needle is removed, and a sterile
pressure dressing and ice pack are placed at the injection site.

Complications and Pitfalls


The major complication of this injection technique is infection.
This complication should be exceedingly rare if strict aseptic technique is followed. The possibility of trauma to the supraspinatus
tendon from the injection itself remains an ever-present possibility. Tendons that are highly inflamed or previously damaged are
subject to rupture if they are directly injected. This complication
can be greatly decreased if the clinician uses gentle technique and
stops injecting immediately if significant resistance to injection
is encountered. Approximately 25% of patients complain of a
transient increase in pain after this injection technique; patients
should be warned of this possibility.

76 SECTION 3 Shoulder Pain Syndromes


SUGGESTED READINGS
Subdeltoid
synovial bursa

Supraspinatus
tendon

Deltoid muscle
Head of humerus
Periosteum

Posterior

Anterior

Figure 28-3 Correct needle placement for injection into the supraspinatus tendon.

Clinical Pearls
The musculotendinous unit of the shoulder joint is susceptible to the development of tendinitis for several reasons.
First, the joint is subjected to a wide range of repetitive
motions. Second, the space in which the musculotendinous unit functions is restricted by the coracoacromial
arch, making impingement a likely possibility with extreme
movements of the joint. Third, the blood supply to the
musculotendinous unit is poor, making healing of microtrauma more difficult. All of these factors can contribute
to tendinitis of one or more of the tendons of the shoulder
joint. Calcium deposition around the tendon may occur if
the inflammation continues, making subsequent treatment
more difficult. Tendinitis of the musculotendinous unit of
the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain
and functional disability.
The injection technique described is extremely effective
in the treatment of pain secondary to the causes of shoulder
pain mentioned earlier. Coexistent bursitis and arthritis also
may contribute to shoulder pain and may require additional
treatment with a more localized injection of local anesthetic
and depot steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in
the areas to be injected. Care must be taken to use sterile
technique to avoid infection and universal precautions to
avoid risk to the operator. The incidence of ecchymosis and
hematoma formation can be decreased if pressure is placed
on the injection site immediately after injection.

Chen SK, Chou PH, Lue YL, Lu YM: Treatment for frozen shoulder combined
with calcific tendinitis of the supraspinatus, Kaohsiung J Med Sci 2880;24:
78-84.
Gimblett PA, Saville J, Ebrall E: A conservative management protocol for calcific
tendinitis of the shoulder, J Manipulative Physiol Ther 22:622627, 1999.
Hsu HC, Wu JJ, Jim YF, Chang CY, etal: Calcific tendinitis and rotator cuff
tearing: a clinical and radiographic study, J Shoulder Elbow Surg 3:159164,
1994.
Waldman SD: Supraspinatus tendinitis. In Waldman SD, editor: Atlas of
pain management injection techniques, ed 3, Philadelphia, 2007, Saunders,
pp 6467.
Waldman SD: Functional anatomy of the shoulder joint. In Waldman SD, editor:
Pain review, Philadelphia, 2009, Saunders, pp 8081.

Chapter 29
INFRASPINATUS TENDINITIS

ICD-9 CODE 726.10


ICD-10 CODE M75.10

sedimentation rate, and antinuclear antibody testing, may be


indicated. Magnetic resonance imaging (MRI) of the shoulder
is indicated if rotator cuff tear is suspected and to confirm the
diagnosis of infraspinatus tendinitis (Figure 29-2). The injection technique discussed here serves as a diagnostic and therapeutic maneuver.

The Clinical Syndrome

Differential Diagnosis

Infraspinatus tendinitis can manifest as an acute or chronic painful condition of the shoulder. Acute infraspinatus tendinitis usually occurs in a younger group of patients after overuse or misuse
of the shoulder joint. Inciting factors include activities that require
repeated abduction and lateral rotation of the humerus, such as
installing brake pads during assembly line work. The vigorous use
of exercise equipment also has been implicated. The pain of infraspinatus tendinitis is constant, severe, and localized to the deltoid
area. Significant sleep disturbance is often reported. Patients with
infraspinatus tendinitis exhibit pain with lateral rotation of the
humerus and on active abduction. Chronic infraspinatus tendinitis tends to occur in older patients and to manifest in a more
gradual or insidious manner, without a single specific event of
antecedent trauma. The pain of infraspinatus tendinitis may be
associated with a gradual loss of range of motion of the affected
shoulder. The patient often awakens at night when he or she rolls
over onto the affected shoulder.

Because infraspinatus tendinitis may occur after seemingly


minor trauma or develop gradually over time, the diagnosis is
often delayed. Tendinitis of the musculotendinous unit of the
shoulder frequently coexists with bursitis of the associated bursae
of the shoulder joint, creating additional pain and functional
disability. This ongoing pain and functional disability can cause
the patient to splint the shoulder group, with resultant abnormal movement of the shoulder that puts additional stress on the
rotator cuff. This stress can lead to further trauma to the entire
rotator cuff. With rotator cuff tears, passive range of motion is
normal, but active range of motion is limited, in contrast to frozen shoulder, in which passive and active range of motion are
limited. Rotator cuff tear rarely occurs before age 40 except in
cases of severe acute trauma to the shoulder. Cervical radiculopathy rarely may cause pain limited to the shoulder, although
in most instances, associated neck and upper extremity pain and
numbness are present.

Signs and Symptoms

Treatment

The patient may attempt to splint the inflamed infraspinatus tendon by rotating the scapula anteriorly to remove tension from the
tendon (Figure 29-1). Point tenderness is usually present over the
greater tuberosity. The patient exhibits a painful arc of abduction
and complains of a catch or sudden onset of pain in the midrange of the arc. Early in the course of the disease, passive range
of motion is full and painless. As the disease progresses, patients
with infraspinatus tendinitis often experience a gradual decrease
in functional ability with decreasing shoulder range of motion,
making simple everyday tasks, such as combing hair, fastening a
brassiere, or reaching overhead, quite difficult. With continued
disuse, muscle wasting may occur and a frozen shoulder may
develop.

Initial treatment of the pain and functional disability associated


with rotator cuff tear should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2
(COX-2) inhibitors and physical therapy. The local application
of heat and cold also may be beneficial. For patients who do
not respond to these treatment modalities, the following injection technique may be a reasonable next step. The use of physical therapy, including gentle range-of-motion exercises, should be
introduced several days after the patient undergoes this injection
technique for shoulder pain. Vigorous exercises should be avoided
because they would exacerbate the symptoms.
To inject the infraspinatus tendon, the skin overlying the
posterior shoulder is prepared with antiseptic solution. A sterile
syringe containing 1 mL of 0.25% preservative-free bupivacaine
and 40 mg of methylprednisolone is attached to a 25-gauge,
1-inch needle using strict aseptic technique. With strict aseptic technique, the previously marked point is palpated, and the
insertion of the infraspinatus tendon is identified again with
the gloved finger. The needle is carefully advanced at this point

Testing
Plain radiographs are indicated in all patients with shoulder
pain. Based on the patients clinical presentation, additional
testing, including complete blood cell count, erythrocyte

77

78 SECTION 3 Shoulder Pain Syndromes


Infraspinatus
tendon

Figure 29-1 Patients with infraspinatus tendinitis exhibit posterior point tenderness and a painful arc of abduction.

through the skin and subcutaneous tissues and the margin of


the deltoid muscle and underlying infraspinatus muscle until
it impinges on bone (Figure 29-3). The needle is withdrawn 1
to 2 mm out of the periosteum of the humerus, and the contents of the syringe are gently injected. There should be slight
resistance to injection. If no resistance is encountered, either
the needle tip is in the joint space itself or the infraspinatus
tendon is ruptured. If significant resistance to injection is felt,
the needle tip is probably in the substance of a ligament or tendon and should be advanced or withdrawn slightly until the
injection proceeds without significant resistance. The needle is
removed, and a sterile pressure dressing and ice pack are placed
at the injection site.

Complications and Pitfalls


The major complication of this injection technique is infection.
This complication should be exceedingly rare if strict aseptic technique is followed. The possibility of trauma to the infraspinatus
tendon from the injection itself remains an ever-present possibility. Tendons that are highly inflamed or previously damaged are
subject to rupture if they are directly injected. This complication
can be greatly decreased if the clinician uses gentle technique and
stops injecting immediately if significant resistance to injection
is encountered. Approximately 25% of patients complain of a
transient increase in pain after this injection technique; patients
should be warned of this possibility.

29 Infraspinatus Tendinitis 79

C
B

Figure 29-2 Periarticular crystal deposition: shoulderinfraspinatus, teres minor, and subscapularis tendon calcification. A, In internal rotation,
calcific deposits in the infraspinatus and teres minor tendons appear lateral to the humeral head (arrow) and deposits in the subscapularis tendon are
located near the lesser tuberosity, overlying the joint space (arrowhead). B and C, Radiograph and photograph of a section of the humeral head outline these same calcifications (arrows, arrowhead). (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders.)

80 SECTION 3 Shoulder Pain Syndromes


SUGGESTED READINGS
Gimblett PA, Saville J, Ebrall P: A conservative management protocol for calcific
tendinitis of the shoulder, J Manipulative Physiol Ther 22:622627, 1999.
Hsu HC, Wu JJ, Jim YF, etal: Calcific tendinitis and rotator cuff tearing: a clinical
and radiographic study, J Shoulder Elbow Surg 3:159164, 1994.
Toriyama K, Fukuda H, Hamada K, Noguchi T: Calcifying tendinitis of the infraspinatus tendon simulating a bone tumor, J Shoulder Elbow Surg 3:165168,
1994.
Waldman SD: Functional anatomy of the shoulder joint. In Waldman SD, editor:
Pain review, Philadelphia, 2009, Saunders, pp 8081.
Waldman SD: Infraspinatus tendinitis. In Waldman SD, editor: Atlas of pain management injection techniques, ed 3, Philadelphia, 2007, Saunders, pp 7175.

Infraspinatus
tendon
Synovial bursa
Deltoid muscle
Head of humerus
Periosteum

Posterior

Anterior

Figure 29-3 Correct needle placement for injection into the infraspinatus tendon.

Clinical Pearls
The musculotendinous unit of the shoulder joint is susceptible to the development of tendinitis for several reasons. First, the joint is subjected to a wide range of often
repetitive motions. Second, the space in which the musculotendinous unit functions is restricted by the coracoacromial arch, making impingement a likely possibility with
extreme movements of the joint. Third, the blood supply
to the musculotendinous unit is poor, making healing of
microtrauma more difficult. These factors can contribute
to tendinitis of one or more of the tendons of the shoulder
joint. Calcium deposition around the tendon may occur if
the inflammation continues, making subsequent treatment
more difficult. Tendinitis of the musculotendinous unit of
the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain
and functional disability.
The injection technique described is extremely effective
in the treatment of pain secondary to the causes of shoulder pain mentioned. Coexistent bursitis and arthritis also
may contribute to shoulder pain and may require additional
treatment with a more localized injection of a local anesthetic and depot steroid. This technique is a safe procedure
if careful attention is paid to the clinically relevant anatomy
in the areas to be injected. Care must be taken to use sterile
technique to avoid infection and universal precautions to
avoid risk to the operator. The incidence of ecchymosis and
hematoma formation can be decreased if pressure is placed
on the injection site immediately after injection.

Chapter 30
SUBACROMIAL IMPINGEMENT
SYNDROME
ICD-9 CODE 719.41
ICD-10 CODE M25.519
The Clinical Syndrome
The subacromial space lies directly inferior to the acromion,
coracoid process, acromioclavicular joint, and coracoacromial
ligament (Figure 30-1). Lubricated by the subacromial bursa, the
subacromial space in health is narrow, and the anatomical structures surrounding it are responsible for maintaining static and
dynamic shoulder stability. The space between the acromion and
the superior aspect of the humeral head is called the impingement
interval, and abduction of the arm narrows the space further (Figure 30-2). Any pathological condition that further narrows this
space (e.g., osteophyte, abnormal acromial anatomy, ligamentous
calcification, or congenital defects of the acromion) increases the
incidence of impingement (Figure 30-3). The most common
causes of subacromial impingement are listed in Table 30-1.
Similar to the congenital anatomical variant of the trefoil spinal
canal being associated with a statistically significantly higher incidence of spinal stenosis, several common normal anatomical variants
of the acromion often contribute to development of subacromial
Coracoid process
Subacromial space
Acromion

Clavicle

Greater tuberosity
Lesser tuberosity

Scapula

Humerus

Figure 30-1 The subacromial space lies directly inferior to the acromion, the coracoid process, the acromioclavicular joint, and the coracoacromial ligament.

impingement syndrome. These include type 2 and type 3 acromions


(Figure 30-4). Although the normal type 1 acromion is relatively
flat, the type 2 acromion curves downward and the type 3 acromion
hooks downward in the shape of a scimitar. The downward curve of
the type 2 and type 3 acromions markedly narrow the subacromial
space (Figure 30-5). In addition to these anatomical variations, a
congenitally unfused acromial apophysis, the os acromiale, is often
associated with subacromial impingement syndrome (Figure 30-6).
Patients with subacromial impingement syndrome present
with diffuse shoulder pain, with an associated feeling of weakness
combined with loss of range of motion. Pain is often worse at
night, and patients often complain that they are unable to sleep on
the affected shoulder. Although subacromial impingement syndrome can occur as a result of acute trauma, the usual clinical presentation is more insidious, without a clear-cut history of trauma
to the affected shoulder. Untreated, subacromial impingement
syndrome can lead to progressive tendinopathy of the rotator cuff
and gradually increasing shoulder instability and functional disability. In patients older than 50 years, progression of impingement often leads to rotator cuff tear.

Signs and Symptoms


A patient with subacromial impingement syndrome reports
increasing shoulder pain with any activities that abduct or forward
flex the shoulder, such as putting in a light bulb or reaching for
dishes in a cabinet above shoulder height (Figure 30-7). Patients
with subacromial impingement syndrome have a positive Neers
test, which is performed by having the patient assume a sitting
position while the examiner applies firm forward pressure on the
patients scapula and simultaneously raises the patients arm to an
overhead position (Figure 30-8). Neers test is considered positive when the patient exhibits pain or apprehension when the arm
moves about 60 degrees. Although not completely diagnostic of
subacromial impingement syndrome, a positive Neers test should
prompt the examiner to order magnetic resonance imaging (MRI)
of the affected shoulder to clarify and strengthen the diagnosis.

Testing
MRI of the shoulder provides the best information regarding any
pathological process of the shoulder. MRI is highly accurate and
helps identify abnormalities that may put the patient at risk for
continuing damage to the rotator cuff and humeral head. MRI
of the shoulder also helps rule out unsuspected pathological conditions that may harm the patient, such as primary and metastatic tumors of the shoulder joint and surrounding structures.
In patients who cannot undergo MRI, such as patients with
pacemakers, computed tomography (CT) is a reasonable second
81

82 SECTION 3 Shoulder Pain Syndromes

Coraco-clavicular ligament

Subacromial
bursa

Acromioclavicular ligament
Coracoacromial ligament
Inflamed supraspinatus
tendon

Biceps
brachii m.

Subacromial
bursa
impinged

Long head
Short head

Subcapularis m.

Figure 30-2 The space between the acromion and the superior aspect of the humeral head is the impingement interval, and abduction of the arm
narrows the space further.

choice. Radionuclide bone scanning and plain radiography are


indicated if fracture or bony abnormality such as metastatic disease is considered in the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of subacromial impingement syndrome is in question. Arthrocentesis of the
glenohumeral joint may be indicated if septic arthritis or crystal
arthropathy is suspected.

Differential Diagnosis
Subacromial impingement syndrome is a clinical diagnosis supported by a combination of clinical history, physical examination, radiography, and MRI. Pain syndromes that may mimic
subacromial impingement syndrome include subacromial bursitis, tendinopathy and tendinitis of the rotator cuff, calcification
and thickening of coracoacromial ligament, and arthritis affecting
any of the shoulder joints. Adhesive capsulitis or frozen shoulder
may confuse the diagnosis, as may idiopathic brachial plexopathy
(Parsonage-Turner syndrome; see Chapter 24). Primary and metastatic tumors of the shoulder and surrounding structures remain
an ever-present possibility and should always be part of the differential diagnosis of patients presenting with shoulder pain.

Treatment
Initial treatment of the pain and functional disability associated with subacromial impingement syndrome should include a

combination of nonsteroidal antiinflammatory drugs (NSAIDs)


or cyclooxygenase-2 (COX-2) inhibitors and gentle physical
therapy. Local application of heat and cold also may be beneficial. For patients who do not respond to these treatment modalities, injection of the subacromial space with local anesthetic
and steroid is a reasonable next step while obtaining MRI and
other appropriate testing to clarify further the working clinical
diagnosis. The use of physical therapy, including gentle rangeof-motion exercises, should be introduced several days after the
patient undergoes this injection technique for shoulder pain.
Vigorous exercises should be avoided because they would exacerbate the patients symptoms. For patients who do not respond
to these treatment modalities or radiographically have shown
anatomical subacromial impingement that is producing ongoing
damage to the rotator cuff, open or arthroscopic acromioplasty
is required.

Complications and Pitfalls


Failure to diagnose subacromial impingement syndrome correctly puts the patient at risk for the missed diagnosis of other
syndromes that may result in ongoing damage to the shoulder
or lead to overlooked pathological processes in this anatomical
region that may harm the patient, such as Pancoasts tumor or
primary or metastatic tumors of the shoulder. MRI is indicated in
all patients thought to have subacromial impingement syndrome,
and aggressive treatment of surgically correctable causes of such
impingement is generally indicated sooner rather than later to
avoid ongoing irreversible shoulder damage.

30 Subacromial Impingement Syndrome 83

C
Figure 30-3 Shoulder external subacromial impingement syndrome: Magnetic resonance imaging abnormalities. A, On sagittal oblique T1-weighted
(TR/TE, 800/20) spin echo MRI, a subacromial enthesophyte (solid arrow) containing marrow projects from the anterior surface of the acromion (a)
toward the coracoid process (c). Note its relationship to the coracoacromial ligament (open arrows) and supraspinatus tendon (arrowhead). B, In a
second patient, sagittal oblique T1-weighted (TR/TE, 800/12) spin echo MRI shows a large subacromial enthesophyte (arrows). The acromion (a) is
indicated. C, In a third patient, coronal oblique intermediate-weighted (TR/TE, 2000/30) spin echo MRI image reveals the flattened contour and low
signal intensity characteristic of a subacromial enthesophyte (arrows). Also observe osteoarthritis of the acromioclavicular joint manifested as osteophytosis (arrowhead) and an elevated position of the humeral head, indicative of a rotator cuff tear. The tear was shown better on other MR images
(not shown). (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3084.)

84 SECTION 3 Shoulder Pain Syndromes


TABLE 30-1

Causes of Subacromial Impingement Syndrome


Subacromial osteophytes
Rotator cuff tears
Abnormal acromial anatomy (e.g., type 2 acromion, type 3 acromion)
Congenital acromial defect (e.g., os acromiale)
Acquired acromial defects (e.g., displaced fracture)
Inflammatory arthritis of the acromioclavicular joint
Abnormalities of the superior aspect of the humeral head
Glenohumeral joint instability
Crystal arthropathies of the acromioclavicular joint

LD

Frozen shoulder (adhesive capsulitis)


Tendinopathy of the coracoacromial ligament

Type I

Type II

Type III

B
Figure 30-5 A, Lateral downsloping (LD) of the anterior acromion as
seen on coronal section (arrow). B, Coronal T2-weighted MR image with
fat suppression revealing LD (arrow). Note the corresponding alterations
on the bursal surface of the rotator cuff and the thickened subdeltoid
bursa filled with fluid (arrowheads). (From Zlatkin MB: MRI of the shoulder,
2nd ed, Philadelphia, 2003, Lippincott Williams & Wilkins, p 1639.)

Figure 30-4 Anatomical variants of the acromion.

Figure 30-6 Os acromiale. Transaxial intermediate-weighted (TR/TE,


1000/20) spin echo MRI shows a triangular os acromiale (arrows) articulating with the clavicle and, in an irregular fashion (arrowhead), with the
acromion. (From Resnick D, editor: Diagnosis of bone and joint disorders,
4th ed, Philadelphia, 2002, Saunders, p 4577.)

30 Subacromial Impingement Syndrome 85

Acromioclavicular joint
Clavicle
Acromion

Subacromial
bursa
Supraspinatus
tendon

Figure 30-7 Patients with subacromial impingement syndrome typically complain of increasing shoulder pain with activities that abduct or forward
flex the shoulder.

Clinical Pearls

Figure 30-8 Neers test for subacromial impingement syndrome. (From


Waldman SD: Physical diagnosis of pain: An atlas of signs and symptoms, Philadelphia, 2006, Saunders, 2006.)

The musculotendinous unit of the shoulder joint is susceptible to the development of tendinitis for several reasons. First, the joint is subjected to a wide range of often
repetitive motions. Second, the space in which the musculotendinous unit functions is restricted by the coracoacromial arch, making impingement a likely possibility with
extreme movements of the joint. Third, the blood supply
to the musculotendinous unit is poor, making healing of
microtrauma more difficult. These factors can contribute
to tendinitis of one or more of the tendons of the shoulder
joint. Calcium deposition around the tendon may occur if
the inflammation continues, making subsequent treatment
more difficult. Tendinitis of the musculotendinous unit of
the shoulder frequently coexists with bursitis of the associated bursae of the shoulder joint, creating additional pain
and functional disability. Patients with untreated subacromial impingement syndrome continue to experience pain
and functional disability and may continue to cause ongoing irreversible shoulder damage culminating in damage to
the humeral head and rotator cuff tear.

SUGGESTED READINGS
Dickens VA, Williams JL, Bhamra MS: Role of physiotherapy in the treatment
of subacromial impingement syndrome: a prospective study, Physiotherapy
91:159164, 2005.
Michener LA, McClure PW, Karduna AR: Anatomical and biomechanical mechanisms of subacromial impingement syndrome, Clin Biomech 18:369379, 2003.
Neagle CE, Bennett JB: Subacromial anatomy and biomechanics related to the
impingement syndrome, Oper Tech Sports Med 2:8288, 1994.
Waldman SD: Functional anatomy of the shoulder joint. In Waldman SD, editor:
Pain review, Philadelphia, 2009, Saunders, pp 8081.

Chapter 31
OS ACROMIALE PAIN SYNDROME

ICD-9 CODE 719.41


ICD-10 CODE M25.519

diagnostic of os acromiale, a positive Neers or Hawkings test


should prompt the examiner to order magnetic resonance imaging (MRI) of the affected shoulder to clarify and strengthen the
diagnosis.

Testing
The Clinical Syndrome
The subacromial space lies directly inferior to the acromion,
the coracoid process, the acromioclavicular joint, and the coracoacromial ligament (see Figure 30-1). Lubricated by the subacromial bursa, the subacromial space in health is narrow and
the anatomical structures surrounding it are responsible for
maintaining static and dynamic shoulder stability. The space
between the acromion and the superior aspect of the humeral
head is the impingement interval, and abduction of the arm
narrows the space further (see Figure 30-2). Any pathological condition that further narrows this space (e.g., osteophyte,
abnormal acromial anatomy, ligamentous calcification, or
congenital defects of the acromion) increases the incidence of
impingement (see Figure 30-3 and Table 30-1).
One such congenital defect is caused by failure of the distal
ossification center of the acromion to fuse (Figure 31-1). This
failure to fuse is termed os acromiale and essentially results in a
second acromial joint. This second joint can lead to impingement syndromes and exacerbate shoulder instability.
Patients suffering from os acromiale have diffuse shoulder
pain, with an associated feeling of weakness combined with loss
of range of motion. Pain is often worse at night, and patients
often complain that they are unable to sleep on the affected
shoulder. The clinical presentation is usually insidious, without
a clear-cut history of trauma to the affected shoulder. Affected
patients tend to be younger than those with other causes of
shoulder impingement syndromes. Untreated, os acromiale can
lead to progressive tendinopathy of the rotator cuff and gradually increasing shoulder instability and functional disability. In
patients older than 50 years, progression of impingement often
leads to rotator cuff tear.

Signs and Symptoms


A patient with os acromiale reports increasing shoulder pain
with any activities that abduct or forward flex the shoulder,
such as putting in a light bulb or reaching for dishes in a cabinet
above shoulder height (Figure 31-2). Patients with os acromiale
have positive tests for shoulder impingement, such as Neers
and Hawkings tests (Figure 31-3). Although not completely
86

MRI of the shoulder provides the best information regarding


any pathological condition of the shoulder (Figure 31-4). MRI
is highly accurate and helps to identify abnormalities that may
put the patient at risk for continuing damage to the rotator cuff
and the humeral head. MRI of the shoulder also helps rule out
unsuspected pathology that may harm the patient, such as primary and metastatic tumors of the shoulder joint and surrounding
structures. In patients who cannot undergo MRI, such as patients
with pacemakers, computed tomography (CT) is a reasonable second choice. Radionuclide bone scanning and plain radiography
are indicated if fracture or bony abnormality such as metastatic
disease is considered in the differential diagnosis.
Screening laboratory tests consisting of complete blood cell
count, erythrocyte sedimentation rate, and automated blood
chemistry testing should be performed if the diagnosis of subacromial impingement syndrome is in question. Arthrocentesis of the
glenohumeral joint may be indicated if septic arthritis or crystal
arthropathy is suspected.

Differential Diagnosis
Os acromiale is a clinical diagnosis supported by a combination
of clinical history, physical examination, radiography, and MRI.
Pain syndromes that may mimic os acromiale include subacromial
impingement syndrome, subacromial bursitis, tendinopathy and
tendinitis of the rotator cuff, calcification and thickening of the
coracoacromial ligament, and arthritis affecting any of the shoulder joints. Adhesive capsulitis or frozen shoulder may confuse
the diagnosis, as may idiopathic brachial plexopathy (ParsonageTurner syndrome; see Chapter 24). Acromial stress fractures and
undiagnosed clavicular fractures also may mimic the clinical presentation of os acromiale, as may impingement syndromes caused
by aberrant subacromial blood vessels. Primary and metastatic
tumors of the shoulder and surrounding structures are an everpresent possibility and should remain as part of the differential
diagnosis of patients with shoulder pain.

Treatment
Initial treatment of the pain and functional disability associated
with os acromiale should include a combination of nonsteroidal

31 Os Acromiale Pain Syndrome 87

Os acromiale

Figure 31-1 Os acromiale is a congenital defect caused by failure of the distal ossification center of the acromion to fuse.

antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2)


inhibitors and gentle physical therapy. Local application of heat
and cold also may be beneficial. For patients who do not respond
to these treatment modalities, injection of the subacromial space
with local anesthetic and steroid is a reasonable next step while
obtaining MRI and other appropriate testing to clarify the working clinical diagnosis further. The use of physical therapy, including gentle range-of-motion exercises, should be introduced several
days after the patient undergoes this injection technique for
shoulder pain. Vigorous exercises should be avoided because they
would exacerbate the symptoms. For patients who do not respond
to these treatment modalities or have radiographically shown
anatomical subacromial impingement that is producing ongoing
damage to the rotator cuff, open or arthroscopic acromioplasty is
required.

Complications and Pitfalls


Failure to diagnose os acromiale correctly puts the patient at risk
for the missed diagnosis of other syndromes that may result in
ongoing damage to the shoulder or lead to an overlooked pathological condition in this anatomical region that may harm the
patient, such as Pancoasts tumor or primary or metastatic tumors
of the shoulder. MRI is indicated in all patients thought to have
os acromiale, and aggressive treatment of surgically correctable
causes of such impingement is generally indicated sooner rather
than later to avoid ongoing irreversible shoulder damage.

Clinical Pearls
The acromion has three distinct ossification centers: (1) the
basiacromion-metacromion, which is most proximal; (2)
the metacromion-mesoacromion, which is in the middle;
and (3) the mesoacromion-preacromion, which is most
distal. Lack of fusion of the mesoacromion-preacromion is
responsible for the development of os acromiale.
The musculotendinous unit of the shoulder joint is susceptible to the development of tendinitis for several reasons.
First, the joint is subjected to a wide range of often repetitive motions. Second, the space in which the musculotendinous unit functions is restricted by the coracoacromial
arch, making impingement a likely possibility with extreme
movements of the joint. Third, the blood supply to the musculotendinous unit is poor, making healing of microtrauma
more difficult. These factors can contribute to tendinitis of
one or more of the tendons of the shoulder joint. Calcium
deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult.
Tendinitis of the musculotendinous unit of the shoulder
frequently coexists with bursitis of the associated bursae of
the shoulder joint, creating additional pain and functional
disability. Patients with untreated os acromiale continue to
experience pain and functional disability and may continue
to cause ongoing irreversible shoulder damage culminating
in damage to the humeral head and rotator cuff tear.

Figure 31-3 Hawkings test for shoulder impingement. (From Waldman


SD: Physical diagnosis of pain: an atlas of signs and symptoms, Philadelphia, 2006, Saunders, p 72.)

Figure 31-2 Patients with os acromiale complain of increasing shoulder


pain with any activities that abduct or forward flex the shoulder.

Figure 31-4 Os acromiale. A, T2-weighted gradient echo axial image. A high signal intensity line (arrows) runs through the acromion and demarcates
the division between the anterior os acromiale and the remainder of the acromion. B, T2-weighted sagittal oblique image also shows a dividing line
crossing the acromion (arrow). A large, full-thickness rotator cuff tear also is visible (asterisk). (From Haaga JR, Lanzieri CF, Gilkeson RC, editors: CT and
MR imaging of the whole body, 4th ed, Philadelphia, 2003, Mosby, p 1955.)

31 Os Acromiale Pain Syndrome 89


SUGGESTED READINGS
Case DT, Burnett SE, Nielsen T: Os acromiale: population differences and their
etiological significance, HOMO 57:118, 2006.
Nicholson GP, Goodman DA, Flatow EL, Bigliani LU: The acromion: morphologic condition and age-related changesa study of 420 scapulas, J Shoulder
Elbow Surg 5:111, 1996.

Nissen CW: The acromion: fractures and os acromiale, Oper Tech Sports Med
12:3234, 2004.
Pagnani MJ, Mathis CE, Solman CG: Painful os acromiale (or unfused acromial
apophysis) in athletes, J Shoulder Elbow Surg 15:432435, 2006.

Chapter 32
GLOMUS TUMOR OF THE
SHOULDER

ICD-9 CODE 228.00


ICD-10 CODE D18.00
The Clinical Syndrome
Glomus tumor of the shoulder is an uncommon cause of shoulder pain. It is the result of tumor formation of the glomus body,
which is a neuromyoarterial apparatus whose function is to regulate peripheral blood flow in the dermis. Glomus tumors occur
most commonly in the subungual region of the fingers, but may
also occur in areas of the body that are not richly endowed with
glomus apparatus (e.g., muscle, bone, blood vessels, nerves). Glomus tumors tend to be solitary, small tumors, but occasionally can
become quite large.
Most patients with glomus tumor are women 30 to 50 years
of age. The pain associated with glomus tumor is severe, lancinating, and boring. Patients suffering from glomus tumor exhibit the
classic triad of intermittent, excruciating pain; cold intolerance;
and tenderness to palpation. If the tumor is located superficially, a
bluish discoloration under the skin may be visible and the patient
may experience an exacerbation of pain with exposure to cold.
Because of the rarity of glomus tumor in areas other than the digits, diagnosis is often delayed.

appears as a very high and homogeneous signal on T2-weighted


images (Figure 32-1). The bony changes associated with glomus
tumor of the shoulder also may appear on plain radiographs if
a careful comparison of the corresponding contralateral shoulder
is made. Radionuclide bone scan also may reveal localized bony
destruction. If the tumor is superficial, pain may be reproduced by
placing an ice pack over the affected area. Based on the patients
clinical presentation, additional tests, including complete blood
cell count, uric acid level, erythrocyte sedimentation rate, and
antinuclear antibody testing, may be indicated. Electromyography
is indicated if coexistent plexopathy or radiculopathy is suspected.
Surgical exploration of the affected area bed often is necessary to
confirm the diagnosis.

Differential Diagnosis
The triad of localized, intermittent, lancinating excruciating
pain, tenderness to palpation, and cold intolerance makes the
diagnosis apparent to an astute clinician. Glomus tumor of the
shoulder must be distinguished from other causes of localized
shoulder pain. If a history of trauma is present, fracture, osteomyelitis, tenosynovitis, and foreign body synovitis should be

Signs and Symptoms


The diagnosis of glomus tumor of the shoulder is based primarily
on three points in the patients clinical history: (1) excruciating
pain that is localized to the area of the tumor, (2) ability to trigger the pain by palpating the area (Loves test), and (3) marked
intolerance to cold (Posners cold induction test). Hildreths test
is also useful in the diagnosis of glomus tumor. It is performed
by placing a tourniquet proximal to the area of suspected tumor.
As the distal area becomes ischemic, the sharp lancinating pain
characteristic of glomus tumor will occur. If the tumor is superficial enough, the examiner may identify it beneath the skin. The
patient with glomus tumor of the shoulder often will guard or
protect the area of the tumor to avoid stimulating the pain.

Testing
Magnetic resonance imaging (MRI) of the affected area often
reveals the actual glomus tumor and may reveal erosion or a perforating lesion of the phalanx beneath the tumor. The tumor
90

Figure 32-1 Frontal T1 MRI shows the calcification at the distal insertion
of the deltoid muscle in the area of the glomus tumor. (From Boretto J-G,
Lazerges C, Coulet B, Baldet P, Chammas M: Calcified glomus tumor of the
shoulder: a case report, Chir Main 4:183186, 2008.)

32 Glomus Tumor Of The Shoulder 91

considered. If there is no history of trauma, tumors or diseases


of the glenohumoral joint and associated soft tissues should
be considered. Reflex sympathetic dystrophy should be distinguishable from glomus tumor of the shoulder because the pain
of reflex sympathetic dystrophy is less localized and is associated with distal trophic skin and nail changes and vasomotor
and sudomotor abnormalities.

Treatment
The mainstay of treatment of glomus tumor is surgical removal.
Medication management is uniformly disappointing. Injection of the affected area in the point of maximal tenderness
may provide temporary relief of the pain of glomus tumor and
blocks Posners cold induction test response, further strengthening the diagnosis.

Complications and Pitfalls


The main complication associated with glomus tumor of the
shoulder involves problems associated with delayed diagnosis,
mainly ongoing destruction of the bone and soft tissues adjacent to the glomus tumor. Although usually localized and well
encapsulated, rarely, these tumors can exhibit aggressive invasive

Clinical Pearls
The diagnosis of glomus tumor of the shoulder is usually
straightforward if the clinician identifies the unique nature
of its clinical presentation. Because of the rare potential for
aggressive, invasive behavior, complete excision and careful
follow-up are important.
tendencies, making complete excision of the tumor and careful
follow-up mandatory.
SUGGESTED READINGS
Abela M, Cole AS, Hill GA, Carr AJ: Glomus tumor of the scapular region,
JShoulder Elbow Surg 9:532533, 2000.
Boretto JG, Lazerges C, Coulet B, Baldet P, Chammas M: Calcified glomus tumor
of the shoulder: a case report, Chir Main 27:183186, 2008.
Ghaly RF, Ring AM: Supraclavicular glomus tumor, 20-year history of undiagnosed shoulder pain: a case report, Pain 83:379382, 1999.
Nebreda CL, Urban BJ, Taylor AE: Upper extremity pain of 10 years duration
caused by a glomus tumor, Reg Anesth Pain Med 25:6971, 2000.
Roberts SN, Carter C, Brown JN, Hayes MG, Saies A: Enormous glomus tumor
of the shoulder, J Shoulder Elbow Surg 8:365366, 1999.
Yoshikawa I, Murakami M, Ishizawa M, Matsumoto K, Hukuda S: Glomus
tumor of the musculotendinous junction of the rotator cuff, Clin Orthop
326:250253, 1996.

Chapter 33
PECTORALIS MAJOR TEAR
SYNDROME

PECTORALIS MUSCLE TEAR


ICD-9 CODE 840.9
ICD-10 CODE S46.919A
PECTORALIS MUSCLE TENDON RUPTURE
ICD-9 CODE 848.9
ICD-10 CODE T14.90
The Clinical Syndrome
The pectoralis major muscle is susceptible to trauma ranging
from microscopic tears of the muscle substance after heavy exertion to macroscopic partial tearing of the muscle or, in extreme
cases, full-thickness tearing with associated hematoma formation
and cosmetic deformity (Figures. 33-1 to 33-3). Additionally, the
pectoralis major tendon can rupture at its point of insertion into
the crest of the greater tubercle of the humerus (Figure 33-4). A
broad, thick, fanlike muscle, the pectoralis major arises from the
anterior surface of the proximal clavicle, the anterior surface of
the sternum, the cartilaginous attachments of the second through
sixth and occasionally seventh ribs, and the aponeurotic band of
the obliquus externus abdominis. These muscle fibers overlap,
with some running upward and laterally, others running horizontally, and others running downward and laterally, all ending in a
broad flat tendon that inserts into the crest of the greater tubercle
of the humerus.
The clinical presentation of pectoralis major tear syndrome is
varied because of its several causes, with the severity of symptoms
directly proportional to the amount of trauma sustained by the
muscle, its tendons, or both. Patients with pectoralis major tear
syndrome present with the acute onset of anterior chest wall pain
after trauma to the muscle sustained while performing activities
such as bench pressing or rappelling down cliffs (Figure 33-5).
The severity of pain is proportional to the amount of trauma sustained. A patient with pectoralis muscle tear syndrome also may
complain of varying degrees of weakness with internal rotation of
the humerus. If complete tear of the muscle or rupture of the tendon occurs, the anterior chest wall bulges acutely with contraction
92

of the muscle in a manner analogous to the Popeyes bulge of


Ludingtons sign associated with rupture of the biceps tendon
(Figures 33-6 and 33-7). If the rupture is not repaired promptly,
further muscle retraction and calcification occur, worsening the
functional disability and cosmetic deformity.

Signs and Symptoms


A patient with pectoralis major tear syndrome complains of the
acute onset of pain in the anterior chest after trauma to the pectoralis major muscle, tendon, or both. If the trauma is significant,
hematoma formation is clearly visible. With rupture of the tendon
at its insertion site into the humerus, impressive ecchymosis of the
arm and anterior chest wall that may seem out of proportion to
the amount of trauma perceived by the patient is present. Active
internal rotation of the humerus against examiner resistance may
reveal weakness. If significant disruption of the muscle or rupture
of the tendon occurs, the patient is unable to reach behind his
or her back (Figure 33-8). As mentioned previously, if complete
tear of the muscle or rupture of the tendon occurs, the anterior
chest wall bulges with contraction of the pectoralis major against
the unopposed torn distal muscle, tendon, or both. Although
not completely diagnostic of pectoralis major tear syndrome, this
physical finding should prompt the examiner to order magnetic
resonance imaging (MRI) of the affected proximal humerus and
shoulder and anterior chest wall to further clarify and strengthen
the diagnosis.

Testing
MRI of the shoulder, proximal humerus, and anterior chest
wall provides the best information regarding pathological processes of these anatomical regions. MRI is highly accurate and
helps identify abnormalities that may require urgent surgical
repair, such as large complete muscle tears, tendon rupture,
or both. MRI of the affected anatomy also helps the clinician
rule out unsuspected pathological conditions that may harm
the patient, such as primary and metastatic tumors. In patients
who cannot undergo MRI, such as patients with pacemakers,
computed tomography (CT) is a reasonable second choice.
Radionuclide bone scanning and plain radiography are indicated if fracture or bony abnormality such as metastatic disease
of the proximal humerus, shoulder, or anterior chest wall is
being considered in the differential diagnosis. Screening laboratory tests consisting of complete blood cell count, erythrocyte
sedimentation rate, and automated blood chemistry testing
should be performed if the diagnosis of pectoralis major tear
syndrome is in question.

33 Pectoralis Major Tear Syndrome 93


Microscopic muscle fiber tears

Mild bleeding and slight edema

Figure 33-1 Microscopic tear of the pectoralis muscle with mild bleeding and slight edema.

Pectoralis major muscle tear


Figure 33-2 Full-thickness tear of the pectoralis muscle with associated hematoma formation and cosmetic deformity.

Differential Diagnosis
Pectoralis major tear syndrome is a clinical diagnosis supported
by a combination of clinical history, physical examination, radiography, and MRI. Pain syndromes that may mimic pectoralis
major tear syndrome include injuries to the pectoralis minor, subscapularis, or latissimus dorsi muscles and inferior glenohumeral
ligament injuries. Dislocation of the manubrium from the body of

the sternum after acceleration/deceleration injuries also may confuse the diagnosis. Fractures of all of the bony origins of the pectoralis major muscles (e.g., the sternum and ribs and fractures of the
anatomical or surgical neck of the humerus) may mimic the clinical presentation of pectoralis major tear syndrome. Primary and
metastatic tumors of the shoulder, humerus, and anterior chest
wall and their surrounding structures remain an ever-present possibility and should be included as part of the differential diagnosis

94 SECTION 3 Shoulder Pain Syndromes

Complete rupture
of tendon

Figure 33-4 Pectoralis major tendon rupture at its point of insertion


into the crest of the greater tubercle of the humerus.
Figure 33-3 Clinical photograph shows retraction of the left pectoralis
major, abnormal contour of the axillary fold, and ecchymosis over the
medial arm in a patient with a complete pectoralis tendon rupture at
the insertion site. (From Hasegawa K, Schofer JM: Rupture of the pectoralis
major: a case report and review, J Emerg Med 38:196200, 2010.)

Pectoralis
major muscle

Figure 33-5 Patients with pectoralis major tear syndrome present with acute onset of anterior chest wall pain after trauma to the muscle sustained
while performing activities such as bench pressing.

of patients with symptoms thought to result from pectoralis major


tear syndrome.

Treatment
Although the pain and functional disability associated with mild
microscopic tears of the pectoralis major muscle may be treated
conservatively with a combination of the nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors
and gentle physical therapy, more extensive tears and rupture of
the pectoralis major tendon require urgent surgical repair if permanent cosmetic deformity and functional disability are to be
avoided.

Complications and Pitfalls


Failure to diagnose pectoralis major tear syndrome correctly
puts the patient at risk for the missed diagnosis of other syndromes that may result in ongoing damage to the shoulder or
lead to overlooked pathological processes in this anatomical
region that may harm the patient, such as Pancoasts tumor
or primary or metastatic tumors of the shoulder, humerus, or
anterior chest wall. MRI is indicated in all patients thought to
have pectoralis major tear syndrome, and aggressive treatment
of surgically correctable causes of the symptoms is indicated
on an urgent basis to avoid irreversible cosmetic deformity and
functional disability.

33 Pectoralis Major Tear Syndrome 95

Figure 33-8 Active internal rotation of the humerus may reveal weakness. If significant disruption of the muscle or rupture of the tendon
occurs, the patient is unable to reach behind his or her back. (From
Waldman SD: Physical diagnosis of pain: an atlas of signs and symptoms,
Philadelphia, 2006, Saunders, p 50.)

B
Figure 33-6 A, Resting axial gradient-recalled echo (GRE) MRI in
a patient with a known pectoralis tendon tear adjacent to the left
humerus at rest shows mild asymmetry of the pectoralis major muscles,
with apparent discontinuity of the left pectoralis major muscle at the
axillary line (arrow). B, Axial GRE MRI in the same patient with sustained
maximal contraction of the injured muscle shows a prominent bulge
in the medial aspect of the left pectoralis major muscle (arrow). (From
EdelmanRR, Hesselink JR, Zlatkin MB, etal, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2006, Saunders, p 3468.)

Biceps m.

Ruptured proximal tendon

Clinical Pearls
Pectoralis major tear syndrome is an uncommon but easily
recognized cause of anterior chest wall and shoulder pain. A
patient with complete pectoralis major muscle tear, tendon
rupture, or both may present with hematoma and ecchymosis formation that seems out of proportion to the patients
perception of the amount of trauma sustained; the patient
often requires reassurance that he or she will not bleed to
death. Such patients should undergo urgent surgical repair
and careful postoperative rehabilitation to avoid permanent
cosmetic deformity and functional disability.

SUGGESTED READINGS

Figure 33-7 Popeyes sign associated with rupture of the biceps tendon. (From Waldman SD: Physical diagnosis of pain: an atlas of signs and
symptoms, Philadelphia, 2006, Saunders, p 83.)

Beloosesky Y, Grinblat J, Katz M, Hendel D, Sommer R: Pectoralis major rupture in the elderly: clinical and sonographic findings, Clin Imaging 27:261264,
2003.
ElMaraghy AR, Devereaux MW: A systematic review and comprehensive classification of pectoralis major tears, J Shoulder Elbow Surg 21:412422, 2012.
Mellado JM, Calmet J, Gin J, Saur A: Pectoralis major muscle and tendon tears:
report of two cases with surgical correlation and postoperative follow-up, Eur
JRadiol Extra 50:101104, 2004.
Petilon J, Ellingson CI, Sekiya JK: Pectoralis major muscle ruptures, Oper Tech
Sports Med 13:162168, 2005.
Weaver JS, Jacobson JA, Jamadar DA, etal: Sonography of the pectoralis major
tear, Ultrasound Med Biol 29:S15, 374383, 2003.

Chapter 34
SUPRASCAPULAR NERVE
ENTRAPMENT
to the ipsilateral shoulder. Tenderness over the suprascapular
notch is usually present. Shoulder movement, especially reaching
across the chest, may increase the pain. Untreated, weakness and
atrophy of the supraspinatus and infraspinatus muscles occur.

ICD-9 CODE 355.9


ICD-10 CODE G58.9

Signs and Symptoms


The Clinical Syndrome
Suprascapular nerve entrapment is an uncommon cause of shoulder pain that is being encountered more frequently in clinical
practice with the increasing use of backpacks instead of briefcases. Suprascapular nerve entrapment syndrome is caused by
compression of the suprascapular nerve as it passes through the
suprascapular notch. The most common causes of compression
of the suprascapular nerve at this anatomical location include the
prolonged wearing of heavy backpacks and direct blows to the
nerve such as occur in football injuries and in falls from trampolines (Figure 34-1). Suprascapular nerve entrapment syndrome
also is seen in baseball pitchers and quarterbacks.
This entrapment neuropathy manifests most commonly as a
severe, deep, aching pain that radiates from the top of the scapula

The most important finding in patients with suprascapular nerve


entrapment is weakness of the supraspinatus and infraspinatus
muscles. This weakness manifests itself as weakness of abduction
and external rotation of the ipsilateral shoulder. With significant
compromise of the suprascapular nerve, atrophy of the infraspinatus muscle is apparent as it lies superficially. The pain of
suprascapular nerve entrapment can be exacerbated by abducting
the ipsilateral scapula by reaching across the chest and simultaneously rotating the neck away from the involved shoulder. Tenderness to palpation of the suprascapular notch is often present.

Testing
Electromyography helps to distinguish cervical radiculopathy and
Parsonage-Turner syndrome from suprascapular nerve entrapment
Suprascapular nerve

Supraspinatus m.
Infraspinatus m. (cut)

Figure 34-1 Suprascapular nerve entrapment is caused by compression of the suprascapular nerve as it passes through the suprascapular notch.
m,Muscle.

96

34 Suprascapular Nerve Entrapment 97

Trapezius

Supraspinatus

Suprascapular
nerve
Infraspinatus m.
Suprascapular
notch

Transverse
suprascapular
ligament

Suprascapular
nerve

Figure 34-2 Transverse sonogram of the scapular notch (arrowheads),


the transverse suprascapular ligament, and the suprascapular nerve
(SSN). (From Herring AA, Stone MB, Nagdev A: Ultrasound-guided suprascapular nerve block for shoulder reduction and adhesive capsulitis in the
ED, Am J Emerg Med 8:e1-e3, 2011.

syndrome. Plain radiographs are indicated in all patients who


present with suprascapular nerve entrapment syndrome to rule
out occult bony pathology. Ultrasound imaging may also aid
in the identification of this uncommon cause of shoulder pain
(Figure 34-2). Based on the patients clinical presentation, additional testing, including complete blood cell count, uric acid
level, erythrocyte sedimentation rate, and antinuclear antibody
testing, may be indicated. Magnetic resonance imaging (MRI) of
the shoulder is indicated if a primary joint pathological process
or space-occupying lesion is suspected. The injection technique
described here is a diagnostic and therapeutic maneuver.

Differential Diagnosis
Suprascapular nerve entrapment syndrome is often misdiagnosed as
bursitis, tendinitis, or arthritis of the shoulder. Cervical radiculopathy of the C5 nerve root also may mimic the clinical presentation
of suprascapular nerve entrapment syndrome. Parsonage-Turner
syndrome, also known as idiopathic brachial neuritis, may manifest as sudden onset of shoulder pain and can be confused with
suprascapular nerve entrapment. Tumor involving the superior
scapular nerve, shoulder, or both also should be considered in the
differential diagnosis of suprascapular nerve entrapment syndrome.

Treatment
Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the
treatment of suprascapular nerve entrapment syndrome. The use
of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg titrating upward as side effects allow also is
useful, especially if sleep disturbance also is present. Avoidance of

Infraspinatus m.

Figure 34-3 Injection of suprascapular nerve for relief of pain. (From


Waldman SD: Atlas of pain management injection techniques, Philadelphia, 2000, Saunders, p 169.)

repetitive trauma thought to be contributing to this entrapment


neuropathy also is important, especially in professional athletes. If
these maneuvers fail to produce rapid symptomatic relief, injection of the suprascapular nerve with local anesthetic and steroid is
a reasonable next step (Figure 34-3). If symptoms persist, surgical
exploration and release of the suprascapular nerve are indicated.

Complications and Pitfalls


The proximity to the suprascapular artery and vein suggests the
potential for inadvertent intravascular injection or local anesthetic
toxicity from intravascular absorption or both. The clinician should
carefully calculate the total milligram dosage of local anesthetic
that may be given safely when performing this injection technique.
Because of proximity of the lung, if the needle is advanced too
deeply through the suprascapular notch, pneumothorax is possible.
Care must be taken if surgical decompression of the suprascapular
nerve is undertaken to avoid inadvertent trauma to the spinal accessory nerve as it runs along the ventral surface of the trapezius.

Clinical Pearls
Avoidance techniques of the repetitive movements responsible for suprascapular nerve entrapment are often forgotten in the rush to treatment. The use of rolling briefcases
instead of backpacks may help avoid continued trauma to
the nerve. This injection technique renders the shoulder
joint insensate. It is important that the clinician ensure that
the physical and occupational therapists caring for a patient
who has undergone suprascapular nerve block understand
that the shoulder girdle as well as the shoulder joint have
been rendered insensate after this injection technique.
Deep heat modalities and range-of-motion exercises must
be monitored carefully to avoid burns or damage to the
shoulder.

98 SECTION 3 Shoulder Pain Syndromes


SUGGESTED READINGS
Fehrman DA, Orwin JF, Jennings RM: Suprascapular nerve entrapment by ganglion cysts: a report of six cases with arthroscopic findings and review of the
literature, Arthroscopy 11:727734, 1995.
Moore TP, Hunter RE: Suprascapular nerve entrapment, Oper Tech Sports Med
4:814, 1996.

Toussaint CP, Zager EL: Whats new in common upper extremity entrapment
neuropathies, Neurosurg Clin North Am 19:573581, 2008.
Waldman SD: Suprascapular nerve block. In Waldman SD, editor: Pain review,
Philadelphia, 2009, Saunders, pp 439440.

Chapter 35
QUADRILATERAL SPACE SYNDROME

ICD-9 CODE 355.9


ICD-10 CODE G58.9
The Clinical Syndrome
Quadrilateral space syndrome is an uncommon cause of shoulder and posterior upper arm pain first described by Cahill and
Palmer in 1983. It is now being encountered more frequently
in clinical practice because magnetic resonance imaging (MRI)
makes confirmation of the clinical diagnosis much easier than
the previously required arteriography of the shoulder and upper
extremity. Quadrilateral space syndrome is caused by compression of the axillary nerve as it passes through the quadrilateral
space (Figures 35-1 and 35-2).
The onset of quadrilateral space syndrome is usually insidious,
with the patient often not reporting any obvious antecedent trauma.
A patient suffering from quadrilateral space syndrome complains of
ill-defined pain in the shoulder and paresthesias radiating into the
posterior upper arm and lateral shoulder. This pain and associated
paresthesias frequently are worsened with abduction and external
rotation of the affected upper extremity. As the syndrome progresses, the patient may note increasing weakness of the affected
arm and difficulty with abduction and external rotation. Most cases
of quadrilateral space syndrome have occurred in young athletes
in their early second decade to third decade who are involved in
throwing activities (Figure 35-3). The syndrome may be seen occasionally in older patients as a result of other causes of compression
of the axillary nerve as it travels through the quadrilateral space,
such as glenolabral cysts or tumor. Mild cases of quadrilateral space
syndrome resolve over time, but more severe cases, if left untreated,
result in permanent atrophy of the deltoid and teres minor muscles
(Figure 35-4).

Signs and Symptoms


The most important finding in patients with quadrilateral space
syndrome is weakness of the supraspinatus and infraspinatus
muscles. This manifests as weakness of abduction and external
rotation of the ipsilateral shoulder. With significant compromise
of the axillary nerve, atrophy of the deltoid and teres minor muscle is apparent on physical examination. The pain of quadrilateral
space syndrome can be exacerbated by abducting and externally
rotating the ipsilateral upper extremity. Tenderness to palpation
of the quadrilateral space often is present.

Testing
Electromyography may help identify entrapment of the axillary nerve, although the test may be normal in mild cases even
though significant neurapraxia is present. Electromyography
helps distinguish cervical radiculopathy and Parsonage-Turner
syndrome from quadrilateral space syndrome. Plain radiographs
are indicated in all patients who present with quadrilateral
space syndrome to rule out occult bony pathological processes.
Based on the patients clinical presentation, additional testing,
including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may
be indicated. MRI of the shoulder is indicated in all patients
thought to have quadrilateral space syndrome because this test
is highly specific for this disorder. In the rare patient in whom
MRI is nondiagnostic, subclavian arteriography to show occlusion of the posterior humeral circumflex artery may be considered because this finding is highly suggestive of a diagnosis of
quadrilateral space syndrome.

Differential Diagnosis
Quadrilateral space syndrome is often initially misdiagnosed as
bursitis, tendinitis, or arthritis of the shoulder. Cervical radiculopathy of the lower nerve roots also may mimic the clinical presentation of quadrilateral space syndrome.
Parsonage-Turner syndrome, or idiopathic brachial neuritis,
also may manifest as sudden onset of shoulder pain and can be
confused with quadrilateral space syndrome. Tumor involving
this anatomical region also should be considered in the differential diagnosis of quadrilateral space syndrome, as should occult
fractures of the proximal humerus and other mass lesions, such
as cysts and lipomas, that may compress the axillary nerve as it
traverses the quadrilateral space.

Treatment
Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the
treatment of mild, self-limited quadrilateral space syndrome. The
use of tricyclic antidepressants, such as nortriptyline, at a single
bedtime dose of 25 mg, titrating upward as side effects allow also
is useful, especially if sleep disturbance is present. Gabapentin or
carbamazepine also can be considered. Avoidance of repetitive
trauma thought to be contributing to this entrapment neuropathy also is important, especially in professional athletes. If these
maneuvers fail to produce rapid symptomatic relief, surgical
exploration and release of the axillary nerve are indicated.
99

100 SECTION 3 Shoulder Pain Syndromes

Inflamed and flattened


axillary nerve

Figure 35-1 Anatomy of axillary nerve as it travels through the quadrilateral space.

Figure 35-2 Quadrilateral space: normal anatomy. Coronal oblique section (A) and T1-weighted (TR/TE, 600/20) spin echo magnetic resonance
imaging (B). The posterior humeral circumflex artery and the axillary nerve (51) are located in the quadrilateral space. Other identified structures are
the humeral diaphysis (7), infraspinatus muscle (13), teres minor muscle (15), deltoid muscle (16), teres major muscle (17), long head of the triceps
muscle (35), and lateral head of the triceps muscle (35). (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002,
Saunders, p 3145.)

35 Quadrilateral Space Syndrome 101

Axillary nerve
Teres minor muscle
Teres major muscle
Triceps muscle

Figure 35-3 If left untreated, quadrilateral space syndrome may result in permanent atrophy of the deltoid and teres minor muscles.

Figure 35-4 Quadrilateral space syndrome: Magnetic resonance imaging (MRI). Coronal oblique (A) and transaxial (B) intermediate-weighted (TR/
TE, 2000/35) spin echo MRI show selective atrophy with fatty replacement of the teres minor muscle (arrows). The infraspinatus muscle (13), deltoid
muscle (16), teres major muscle (17), and long (35) and lateral (35) heads of the triceps muscle are identified and are not involved. The humeral
diaphysis (7) also is seen. (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3145.)

Complications and Pitfalls


Failure to diagnose quadrilateral space syndrome correctly puts
the patient at risk for the missed diagnosis of other syndromes
that may result in ongoing damage to the shoulder or lead to
overlooked pathological processes in this anatomical region that

may harm the patient, such as Pancoasts tumor or primary or


metastatic tumors of the shoulder. MRI is indicated in all patients
thought to have quadrilateral space syndrome, and aggressive
treatment of surgically correctable causes is generally indicated
sooner rather than later to avoid ongoing irreversible shoulder
damage.

102 SECTION 3 Shoulder Pain Syndromes

Clinical Pearls
Avoidance techniques of the repetitive movements responsible for quadrilateral space syndrome often are forgotten in
the rush to treatment. Mild cases of quadrilateral space syndrome are usually self-limited, but more severe cases require
urgent surgical intervention. As with other uncommon pain
syndromes, quadrilateral space syndrome should be considered a diagnosis of exclusion and the clinician should ensure
that no potentially harmful occult space-occupying lesions
are present before attributing symptoms to other benign
causes.

SUGGESTED READINGS
Chautems RC, Glauser T, Waeber-Fey MC, Rostan O, Barraud GE: Quadrilateral space syndrome: case report and review of the literature, Ann Vasc Surg
14:673676, 2000.
McClelland D, Paxinos A: The anatomy of the quadrilateral space with reference
to quadrilateral space syndrome, J Shoulder Elbow Surg 17:162164, 2008.
Nishimura M, Kobayashi M, Hamagashira K, etal: Quadrilateral space syndrome:
a rare complication of thoracic surgery, Ann Thorac Surg 86:13501351, 2008.
Sanders TG, Tirman PFJ: Paralabral cyst: an unusual cause of quadrilateral space
syndrome, Arthroscopy 15:632637, 1999.

SECTION 4 Elbow Pain Syndromes

Chapter 36
PRONATOR SYNDROME

ICD-9 CODE 354.0


ICD-10 CODE G56.00
The Clinical Syndrome
Several sites of entrapment of the median nerve exist in the forearm. The median nerve may be entrapped at the lacertus fibrosus,
at the lateral edge of the flexor digitorum superficialis, by fibrous
bands of the superficial head of the pronator teres muscle, or,
most commonly, by the pronator teres muscle itself. Pronator
syndrome is the compression of the median nerve by the pronator teres muscle. The onset of symptoms is usually after repetitive
elbow motions, such as chopping wood, sculling, and cleaning
fish, although occasionally the onset is more insidious, without apparent antecedent trauma. Clinically, pronator syndrome
manifests as a chronic aching sensation localized to the forearm
with pain occasionally radiating into the elbow. Patients with
pronator syndrome may complain of a tired or heavy sensation in
the forearm with minimal activity and clumsiness of the affected
extremity. The sensory symptoms of pronator syndrome are
identical to symptoms of carpal tunnel syndrome. In contrast to
carpal tunnel syndrome, nighttime symptoms are unusual with
pronator syndrome.

Median nerve

Pronator teres
muscle (cut):
Pronator teres
muscle (cut)

Humeral head
Ulnar head

Flexor digitorum
superficialis muscle

Signs and Symptoms


The physical findings in pronator syndrome include tenderness
over the forearm in the region of the pronator teres muscle.
Unilateral hypertrophy of the pronator teres muscle may be
identified. A positive Tinels sign over the median nerve as it
passes beneath the pronator teres muscle also may be present
(Figure 36-1). Weakness of the intrinsic muscles of the forearm
and hand that are innervated by the median nerve may be identified with careful manual muscle testing. A positive pronator
syndrome test, which is pain on forced pronation of the patients
fully supinated arm, is highly suggestive of compression of the
median nerve by the pronator teres muscle (Figure 36-2).

Figure 36-1 The symptoms of pronator syndrome are due to compression of the median nerve by the pronator teres muscle.

103

104 SECTION 4 Elbow Pain Syndromes

Testing
Electromyography helps to distinguish cervical radiculopathy,
thoracic outlet syndrome, and carpal tunnel syndrome from pronator syndrome. Plain radiographs are indicated in all patients
who present with pronator syndrome to rule out occult bony
pathological processes. Based on the patients clinical presentation, additional testing, including complete blood cell count,
uric acid level, erythrocyte sedimentation rate, and antinuclear
antibody testing, may be indicated. Magnetic resonance imaging
(MRI) of the forearm is indicated if a primary elbow pathological
condition or space-occupying lesion is suspected. The injection
of the median nerve at the elbow may serve as a diagnostic and
therapeutic maneuver.

Differential Diagnosis
Median nerve entrapment by the ligament of Struthers manifests
clinically as unexplained persistent forearm pain caused by compression of the median nerve by an aberrant ligament that runs
from a supracondylar process to the medial epicondyle. Clinically,
it is difficult to distinguish from pronator syndrome. The diagnosis
is made by electromyography and nerve conduction velocity testing
that show compression of the median nerve at the elbow combined
with the radiographic finding of a supracondylar process.

Both of these entrapment neuropathies can be differentiated


from isolated compression of the anterior interosseous nerve that
occurs approximately 6 to 8 cm below the elbow. These syndromes also should be differentiated from cervical radiculopathy involving the C6 or C7 roots, which sometimes may mimic
median nerve compression. Cervical radiculopathy and median
nerve entrapment may coexist as the double crush syndrome.
The double crush syndrome is seen most commonly with median
nerve entrapment at the wrist or carpal tunnel syndrome. Thoracic outlet syndrome also may cause forearm pain to be confused
with pronator syndrome. The pain of thoracic outlet syndrome
radiates into the ulnar rather than the median portion of the hand,
however.

Treatment
Nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in the
treatment of pronator syndrome. The use of tricyclic antidepressants, such as nortriptyline, at a single bedtime dose of 25 mg,
titrating upward as side effects allow, also is useful, especially
if sleep disturbance is present. Avoidance of repetitive trauma
thought to be contributing to this entrapment neuropathy also is
important. If these maneuvers fail to produce rapid symptomatic
relief, injection of the median nerve at the elbow with a local anesthetic and steroid is a reasonable next step. If symptoms persist,
surgical exploration and release of the median nerve are indicated.

Complications and Pitfalls


Median nerve block at the elbow is a safe block, with the major
complications being inadvertent intravascular injection and persistent paresthesia secondary to needle trauma to the nerve. This
technique can be performed safely in the presence of anticoagulation by using a 25- or 27-gauge needle, although at increased risk
for hematoma, if the clinical situation dictates a favorable riskto-benefit ratio. These complications can be decreased if manual pressure is applied to the area of the block immediately after
injection. Application of cold packs for 20-minute periods after
the block also decreases the amount of postprocedure pain and
bleeding.

Clinical Pearls

Figure 36-2 A positive pronator syndrome test is highly indicative of


pronator syndrome.

Avoidance techniques of the repetitive movements responsible for pronator syndrome are often forgotten in the rush
to treatment. Median nerve block at the elbow is a simple
and safe technique in the evaluation and treatment of the
aforementioned painful conditions. Careful neurological
examination to identify preexisting neurological deficits
that may later be attributed to the nerve block should be
performed in all patients before beginning median nerve
block at the elbow.
Median nerve compression by the ligament of Struthers
manifests clinically as unexplained persistent forearm pain
caused by compression of the median nerve by an aberrant
ligament that runs from a supracondylar process to the
medial epicondyle. The diagnosis is made by electromyography and nerve conduction velocity testing that show
compression of the median nerve at the elbow combined

36 Pronator Syndrome 105

with the radiographic finding of a supracondylar process.


The pronator syndrome is characterized by unexplained
persistent forearm pain with tenderness to palpation over
the pronator teres muscle. A positive Tinels sign also may
be present. Median nerve compression by the ligament
of Struthers and pronator syndrome must be differentiated from isolated compression of the anterior interosseous nerve that occurs approximately 6 to 8 cm below the
elbow. These syndromes also should be differentiated from
cervical radiculopathy involving the C6 or C7 roots that
may sometimes mimic median nerve compression. Cervical
radiculopathy and median nerve entrapment may coexist as
the double crush syndrome. The double crush syndrome is
seen most commonly with median nerve entrapment at the
wrist or carpal tunnel syndrome.

SUGGESTED READINGS
Horak BT, Kuz JT: An unusual case of pronator syndrome with ipsilateral supracondylar process and abnormal muscle mass, J Hand Surg 33:7982, 2008.
Lacey SH, Soldatis JJ: Bilateral pronator syndrome associated with anomalous
heads of the pronator teres muscle: a case report, J Hand Surg 18:349351,
1993.
Presciutti S, Rodner CM: Pronator syndrome, J Hand Surg 36:907909, 2011.
Rehak DC: Pronator syndrome, Clin Sports Med 20:531540, 2001.

Chapter 37
CUBITAL BURSITIS

ICD-9 CODE 726.33


ICD-10 CODE M70.20
The Clinical Syndrome
An uncommon cause of elbow pain, cubital bursitis is being seen
in clinical practice more frequently because of the increasing
number of people using exercise equipment. The cubital bursa
lies in the anterior aspect of the elbow and produces anterior
elbow pain when inflamed. Also known as the bicipitoradial
bursa, the cubital bursa may exist as a single bursal sac or in some
patients may exist as a multisegmented series of sacs that may be
loculated. The cubital bursa is vulnerable to injury from acute
trauma and repeated microtrauma. Acute injuries frequently take
the form of direct trauma to the anterior aspect of the elbow.
Repetitive movements of the elbow, including repeated bicepsstrengthening exercises and throwing of javelins and baseballs,
may result in inflammation and swelling of the cubital bursa
(Figure 37-1). Gout or rheumatoid arthritis rarely may precipitate acute cubital bursitis. If the inflammation of the cubital
bursa becomes chronic, calcification of the bursa may occur.

nerve entrapment syndromes at the elbow. Injection of the cubital


bursa with a local anesthetic and steroid is a diagnostic and therapeutic maneuver.

Differential Diagnosis
The most common causes of elbow pain are arthritis of the elbow
joint, tennis elbow, golfers elbow, and olecranon bursitis. Arthritis of the elbow joint may mimic cubital bursitis because both
painful conditions are associated with movement of the joint.
The anterior point tenderness seen in cubital bursitis is absent in
arthritis of the elbow, however. Tennis elbow and golfers elbow
are distinct clinical entities that should not be confused with
cubital bursitis because the point tenderness seen in these painful
conditions is identified over the lateral and medial epicondyles,
rather than at the midline, as is seen with cubital bursitis. Acute
gout affecting the elbow manifests as a diffuse acute inflammatory condition that may be difficult to distinguish from infection of the joint, rather than as a localized musculoskeletal pain
syndrome.

Signs and Symptoms


A patient with cubital bursitis frequently reports pain and swelling with any movement of the elbow (see Figure 37-1). The pain
is localized to the cubital area, with referred pain often noted in
the forearm and hand. Physical examination reveals point tenderness in the anterior aspect of the elbow over the cubital bursa and
swelling of the bursa. Passive extension and resisted flexion of the
elbow reproduce the pain, as does any pressure over the bursa.

Testing
The diagnosis of cubital bursitis usually can be made on clinical
grounds. Plain radiographs of the elbow may reveal calcification
of the bursa and associated structures consistent with chronic
inflammation. Magnetic resonance imaging (MRI) is indicated
the patient is thought to have a joint mouse or primary pathological process of the elbow joint. Ultrasound imaging also may aid in
the diagnosis of cubital bursitis (Figure 37-2).
Laboratory testing to rule out hyperuricemia and collagenvascular disease also should be considered in appropriate patients.
Electromyography and nerve conduction velocity testing rule out
106

Figure 37-1 A patient with cubital bursitis reports pain and swelling on
any movement of the elbow.

37 Cubital Bursitis 107

Figure 37-2 Cubital or bicipitoradial bursitis. Longitudinal extended


FOV image demonstrating a sausage-shaped heterogeneous distended
cubital bursa (arrowheads). (From James JJ: Ultrasound of the elbow. In
Allan PL, Baxter GM, Weston MJ, editors: Clinical ultrasound, 3rd ed, New
York, 2011, Elsevier, pp 10431054.)

Treatment
Initial treatment of the pain and functional disability associated
with cubital bursitis should include a combination of nonsteroidal
antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2)
inhibitors and physical therapy. Local application of heat and cold
also may be beneficial. The repetitive movements that incite the
syndrome should be avoided. For patients who do not respond to
these treatment modalities, injection of the cubital bursa with a local
anesthetic and steroid may be a reasonable next step (Figure 37-3).
To inject the cubital bursa, the patient is placed in the supine
position, with the arm fully adducted at the patients side and the
elbow extended and the dorsum of the hand resting on a folded
towel. Using a 5-mL sterile syringe, 2 mL of local anesthetic and
40 mg of methylprednisolone is drawn.
After sterile preparation of skin overlying the anterior aspect
of the joint, the clinician identifies the pulsations of the brachial
artery at the crease of the elbow. After preparation of the skin
with antiseptic solution, a 25-gauge, 1-inch needle is inserted just
lateral to the brachial artery at the crease and slowly advanced in a
slightly medial and cephalad trajectory through the skin and subcutaneous tissues. If bone is encountered, the needle is withdrawn
back into the subcutaneous tissue. The contents of the syringe
are gently injected. Little resistance to injection should be felt. If
resistance is encountered, the needle is probably in the tendon and
should be withdrawn back until the injection proceeds without
significant resistance. The needle is removed, and a sterile pressure
dressing and ice pack are placed at the injection site.

Complications and Pitfalls


The major complication associated with cubital diagnosis is misdiagnosis. Failure of the clinician to recognize an acute inflammatory or infectious arthritis of the elbow may result in permanent
damage to the joint and chronic pain and functional disability.
Injection of the cubital bursa at the elbow is a safe block, with
the major complications being inadvertent intravascular injection and persistent paresthesia secondary to needle trauma to
the median nerve. This technique can be performed safely in the
presence of anticoagulation by using a 25- or 37-gauge needle,
although at increased risk for hematoma, if the clinical situation
dictates a favorable risk-to-benefit ratio. These complications
can be decreased if manual pressure is applied to the area of the
block immediately after injection. Application of cold packs for
20-minute periods after the block also decreases the amount of
postprocedure pain and bleeding.

Cubital bursa

Figure 37-3 Proper needle placement for injection for treatment of


cubital bursitis.

Clinical Pearls
Bursae are formed from synovial sacs whose purpose is
to allow easy sliding of muscles and tendons across one
another at areas of repeated movement. These synovial sacs
are lined with a synovial membrane invested with a network
of blood vessels that secrete synovial fluid. Inflammation of
the bursa results in an increase in the production of synovial fluid with swelling of the bursal sac. With overuse or
misuse, these bursae may become inflamed, enlarged, and,
rarely, infected. Coexistent tendinitis and epicondylitis also
may contribute to elbow pain and may require additional
treatment with more localized injection of local anesthetic
and depot steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in
the areas to be injected, in particular avoiding the median
nerve by keeping the needle lateral to the brachial artery.
Care must be taken to use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation
can be decreased if pressure is placed on the injection site
immediately after injection. The use of physical modalities,
including local heat and gentle range-of-motion exercises,
should be introduced several days after the patient undergoes this injection technique for elbow pain. Vigorous
exercises should be avoided because they exacerbate the
patients symptoms. Simple analgesics and NSAIDs may be
used concurrently with this injection technique.
SUGGESTED READINGS
Chung CB, Kim HJ: Sports injuries of the elbow, Magn Res Imaging Clin N Am
11:239253, 2003.
Hayter CL, Giuffre BM: Overuse and traumatic injuries of the elbow, Magn Res
Imaging Clin N Am 17:617638, 2009.
Howard TM, Shaw JL, Phillips J: Physical examination of the elbow. In Seidenberg PH, Beutler AI, editors: The sports medicine resource manual. Philadelphia,
2008, Saunders, pp 7178.
Sellards R, Kuebrich C: The elbow: diagnosis and treatment of common injuriesprimary care, Clin Office Pract 32:116, 2005.
Waldman SD: Injection technique for cubital bursitis pain. In Waldman SD,
editor: Pain review, Philadelphia, 2009, Saunders, pp 463464.

Chapter 38
ANCONEUS EPITROCHLEARIS

ICD-9 CODE 354.2


ICD-10 CODE G56.20
The Clinical Syndrome
Anconeus epitrochlearis is an uncommon cause of lateral forearm
pain and weakness that can be quite distressing to the patient.
Anconeus epitrochlearis is caused by entrapment and compression
of the ulnar nerve at the elbow by an accessory anconeus muscle
(Figure 38-1). This entrapment neuropathy manifests as pain and
associated paresthesias in the lateral forearm that radiates to the
wrist and ring and little fingers in a manner analogous to tardy
ulnar palsy. The symptoms often are aggravated by prolonged

flexion of the elbow. The pain of anconeus epitrochlearis has been


characterized as unpleasant and dysesthetic. The onset of symptoms is usually after repetitive elbow motions or from repeated
pressure on the elbow, such as using the elbows to arise from bed.
Anconeus epitrochlearis also is seen in throwing athletes such as
baseball pitchers and quarterbacks. Direct trauma to the ulnar
nerve as it enters the cubital tunnel may result in a similar clinical
presentation, as can compression of the ulnar nerve as it passes
through the cubital tunnel by osteophytes, lipomas, ganglions,
and aponeurotic bands. Untreated, progressive motor deficit and
ultimately flexion contracture of the affected fingers can result.

Signs and Symptoms


Physical findings include tenderness over the ulnar nerve at the
elbow. A positive Tinels sign over the ulnar nerve as it passes
beneath the aponeuroses is usually present. Weakness of the

Anconeus
epitrochlearis
Anconeus m.

Inflamed and
compressed
ulnar nerve

Figure 38-1 Anconeus epitrochlearis is caused by entrapment and compression of the ulnar nerve at the elbow by an accessory anconeus muscle.
m, Muscle.

108

38 Anconeus Epitrochlearis 109

Figure 38-2 Eliciting Froments sign. (From Waldman SD: Physical diagnosis of pain: an atlas of signs and symptoms, Philadelphia, 2006, Saunders, p 126.)

intrinsic muscles of the forearm and hand that are innervated


by the ulnar nerve may be identified with careful manual muscle
testing, although early in the course of the evolution of anconeus
epitrochlearis, the only physical finding other than tenderness
over the nerve may be the loss of sensation on the ulnar side of
the little finger. As the syndrome progresses, the affected hand
may have a clawlike appearance (see Figure 32-1). A positive
Wartenbergs sign indicative of weakness of the adduction of the
fifth digit is often present. A positive Froments sign also may be
present (Figure 38-2).

Testing
Electromyography helps to distinguish cervical radiculopathy and
anconeus epitrochlearis from golfers elbow. Plain radiographs are
indicated in all patients who present with anconeus epitrochlearis
to rule out occult bony pathology, such as osteophytes impinging
on the ulnar nerve. Based on the patients clinical presentation,
additional testing, including complete blood cell count, uric acid
level, erythrocyte sedimentation rate, and antinuclear antibody
testing, may be indicated. Magnetic resonance imaging (MRI) of
the elbow is indicated if joint instability is suspected and clearly
identifies whether the compression of the ulnar nerve is caused by
an accessory anconeus muscle (see Figure 38-3). Injection of the
ulnar nerve serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis
Anconeus epitrochlearis is often misdiagnosed as golfers elbow,
and this fact accounts for the many patients with golfers elbow
who fail to respond to conservative measures. In anconeus epitrochlearis, the maximal tenderness to palpation is over the ulnar
nerve 1 inch below the medial epicondyle, whereas in golfers
elbow, the maximal tenderness to palpation is directly over the
medial epicondyle. Anconeus epitrochlearis also should be differentiated from cervical radiculopathy involving the C7 or C8
roots and golfers elbow. Cervical radiculopathy and ulnar nerve
entrapment may coexist as the double crush syndrome. The
double crush syndrome is seen most commonly with median
nerve entrapment at the wrist or carpal tunnel syndrome.

Treatment
Initial treatment of the pain and functional disability associated
with anconeus epitrochlearis should include a combination of

Figure 38-3 Anconeus epitrochlearis muscle replacing the cubital tunnel retinaculum. A T2-weighted axial image reveals the ulnar nerve
(white arrow) deep to an anomalous anconeus epitrochlearis muscle
(black arrow) and superficial to the posterior bundle of the medial collateral ligament (curved arrow). (From Edelman RR, Hesselink JR, Zlatkin MB,
etal, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia,
2006, Saunders, p 3303.)

nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application
of heat and cold also may be beneficial. The repetitive movements
that incite the syndrome should be avoided. For patients who do
not respond to these treatment modalities, injection of the ulnar
nerve at the elbow with a local anesthetic and steroid may be a
reasonable next step. If the symptoms of anconeus epitrochlearis
persist, surgical exploration, resection of the accessory anconeus
muscle, and decompression of the ulnar nerve are indicated.

Complications and Pitfalls


The major complications associated with anconeus epitrochlearis
fall into two categories: (1) iatrogenically induced complications
resulting from persistent and overaggressive treatment of resistant
golfers elbow and (2) the potential for permanent neurological
deficits resulting from prolonged untreated entrapment of the ulnar
nerve. Failure of the clinician to recognize an acute inflammatory or
infectious arthritis of the elbow may result in permanent damage to
the joint and chronic pain and functional disability.

Clinical Pearls
An accessory anconeus muscle is present in approximately
11% of the adult population. Anconeus epitrochlearis is a
distinct clinical entity that is often misdiagnosed as golfers
elbow, and this fact accounts for the many patients with
golfers elbow who fail to respond to conservative measures. With anconeus epitrochlearis, the maximal tenderness to palpation is over the ulnar nerve and a positive
Tinels sign is present, whereas with golfers elbow, the
maximal tenderness to palpation is over the medial epicondyle. If anconeus epitrochlearis is suspected, injection

110 SECTION 4 Elbow Pain Syndromes

of the radial nerve at the elbow with a local anesthetic and


steroid gives almost instantaneous relief. Careful examination to identify preexisting neurological deficits that may
later be attributed to the nerve block should be performed
on all patients before beginning ulnar nerve block at the
elbow.

SUGGESTED READINGS
Dellon AL: Musculotendinous variations about the medial humeral epicondyle,
JHand Surg 11:175181, 1985.
Kojima T: Ulnar compression neuropathy secondary to the anconeus epitrochlearis muscle, J Hand Surg 14:918919, 1989.
Masear VR, Hill JJ Jr, Cohen SM: Ulnar compression neuropathy secondary to
the anconeus epitrochlearis muscle, J Hand Surg 13:720724, 1988.
Waldman SD: The ulnar nerve. In Waldman SD, editor: Pain review, Philadelphia, 2009, Saunders, pp 7677.

Chapter 39
OS SUPRATROCHLEARE-RELATED
ELBOW PAIN

ICD-9 CODE 733.99


ICD-10 CODE M89.8x9
The Clinical Syndrome
Elbow pain secondary to os supratrochleare is being seen with
increasing frequency in clinical practice owing to the increased
interest in physical fitness and the use of exercise machines. Os
supratrochleare is the name given to an accessory ossicle occasionally found in the posterior elbow. This accessory ossicle often is
found adjacent to the proximal aspect of the olecranon process. It
is thought that accessory ossicles such as os supratrochleare bones
serve to decrease the friction and pressure of tendons as they pass
in proximity to a joint. Similar accessory ossicles are found in the
feet, hands, and wrists.
Elbow pain secondary to os supratrochleare is characterized by tenderness and pain over the posterior elbow. Patients
often describe a feeling of having gravel in their elbow and may
report a grating sensation with flexion and extension of the
elbow (Figure 39-1). The pain of os supratrochleare worsens
with activities that require repeated flexion and extension of the

elbow or with forceful overhead throwing. Os supratrochleare


is often associated with loose bodies in the elbow joint and may
coexist with olecranon bursitis.

Signs and Symptoms


On physical examination, pain can be reproduced by pressure
on the os supratrochleare. In contrast to olecranon bursitis, in
which the tender area remains over the olecranon bursa, with os
supratrochleare, the area of maximal tenderness is just above the
olecranon process. A creaking or grating sensation may be appreciated by the examiner, and locking or catching on extension and
flexion of the elbow occasionally may be present.

Testing
Plain radiographs are indicated in all patients with os supratrochleare to rule out fractures and identify accessory ossicles that may
have become inflamed. Plain radiographs also often identify loose
bodies or joint mice frequently seen in patients with elbow pain
secondary to os supratrochleare. Based on the patients clinical presentation, additional testing, including complete blood cell count,
erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the
elbow joint is indicated if joint instability, occult mass, or tumor
is suspected and to clarify the diagnosis further (Figure 39-2).
Radionucleotide bone scanning may be useful in identifying stress
fractures or tumors of the elbow and distal humerus that may be
missed on plain radiographs.

Differential Diagnosis
Primary pathological processes of the elbow, including gout and
occult fractures, may mimic the pain and disability associated
with os supratrochleare. Entrapment neuropathies, such as ulnar
tunnel syndrome, also may confuse the diagnosis, as may bursitis,
tendinitis, and epicondylitis of the elbow, which may coexist with
os supratrochleare. Osteochondritis dissecans, Panners disease,
and synovial chondromatosis also may mimic the pain associated
with os supratrochleare. Primary and metastatic tumors of the
elbow may manifest in a manner similar to elbow pain secondary
to os supratrochleare.

Treatment
Figure 39-1 Elbow pain secondary to os supratrochleare is characterized by tenderness and pain over the posterior elbow.

Initial treatment of the pain and functional disability associated


with os supratrochleare should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2
111

112 SECTION 4 Elbow Pain Syndromes

Figure 39-2 Accessory ossicles. A, Os vesalianum. B, Os intermetatarseum. C, Os supratrochleare posterius (dorsale). (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 4570.)

(COX-2) inhibitors and physical therapy. The local application


of heat and cold also may be beneficial. Avoidance of repetitive
activities that aggravate the patients symptoms also may provide
relief. For patients who do not respond to these treatment modalities, injection of the os supratrochleare with a local anesthetic and
steroid may be a reasonable next step. For pain that persists, or if
the os supratrochleare is causing damage to the elbow joint, surgical removal is indicated.

Clinical Pearls
Pain emanating from the elbow is a common problem
encountered in clinical practice. Os supratrochleare must be
distinguished from fractures of the elbow, fractures of the os
supratrochleare itself, entrapment neuropathies of the ulnar
nerve, bursitis, tendinitis, and epicondylitis. Less common
causes of posterior elbow pain are osteochondritis dissecans,
Panners disease, and synovial chondromatosis.

Complications and Pitfalls


The major complication of injection of os supratrochleare is infection. This complication should be exceedingly rare if strict aseptic
technique is followed. Approximately 25% of patients report a
transient increase in pain after injection of the os supratrochleare
and should be warned of this possibility. Another potential risk of
this injection technique is trauma to the extensor tendons from
the injection itself.

SUGGESTED READINGS
Gudmundsen E, stensen H: Accessory ossicles in the elbow, Acta Orthop Scand
58:130132, 1987.
McFarland EG, Gill HS, Laporte DM, Streiff M: Miscellaneous conditions about
the elbow in athletes [review], Clin Sports Med 23:743763, 2004.
Waldman SD: Functional anatomy of the elbow. In Waldman SD, editor: Pain
review, Philadelphia, 2009, Saunders, pp 7677.
Wood VE, Campbell GS: The supratrochleare dorsale accessory ossicle in the
elbow, J Shoulder Elbow Surg 3:395398, 1994.

Chapter 40
OSTEONECROSIS OF
THE ELBOW JOINT

ICD-9 CODE 733.43


ICD-10 CODE M87.03
The Clinical Syndrome
Osteonecrosis of the elbow joint is an often missed diagnosis. Like
the scaphoid bone of the wrist, the elbow joint is extremely susceptible to this disease because of the tenuous blood supply of the
articular cartilage. This blood supply is easily disrupted, often leaving the proximal portion of the bone without nutrition and leading
to osteonecrosis (Figure 40-1). A disease of the fourth and fifth
decades, with the exception of patients with osteonecrosis of the
elbow joint secondary to collagen-vascular disease, osteonecrosis
of the elbow joint is more common in men. In younger patients,
sickle cell disease is the most common cause of osteonecrosis of the
elbow. The disease is bilateral in 45% to 50% of cases.
Factors predisposing to osteonecrosis of the elbow joint are
listed in Table 40-1. They include trauma to the joint; corticosteroid use; Cushings disease; alcohol abuse; connective tissue

diseases, especially systemic lupus erythematosus; osteomyelitis;


human immunodeficiency virus infection; organ transplantation;
hemoglobinopathies, including sickle cell disease; hyperlipidemia; gout; renal failure; pregnancy; sickle cell disease; and radiation therapy involving the femoral head.
Patients with osteonecrosis of the elbow joint report pain over
the affected elbow joint or joints that may radiate into the upper
extremity. The pain is deep and aching, and patients often report
a catching sensation with range of motion of the affected elbow
joint or joints. Range of motion decreases as the disease progresses.

Signs and Symptoms


Physical examination of patients with osteonecrosis of the elbow
joint reveals pain to deep palpation of the elbow joint. The pain
can be worsened by passive and active range of motion. A click or
crepitus also may be appreciated by the examiner when ranging
the elbow joint. A decreased range of motion is invariably present.

Testing
Plain radiographs are indicated in all patients with osteonecrosis
of the elbow joint to rule out underlying occult bony pathological processes and identify sclerosis and fragmentation of
the osseous support of the articular surface. However, early in
TABLE 40-1

Predisposing Factors for Osteonecrosis of the Elbow Joint


Normal
Cell death
Ischemia
Hyperemia

Trauma to the elbow joint


Steroids
Cushings disease
Alcohol abuse
Connective tissue diseases, especially systemic lupus erythematosus
Osteomyelitis
Human immunodeficiency virus
Organ transplantation
Hemoglobinopathies, including sickle cell disease
Hyperlipidemia
Gout
Renal failure
Pregnancy

Figure 40-1 The blood supply to the elbow is easily disrupted, often
leaving the proximal portion of the bone without nutrition and leading
to osteonecrosis.

Radiation therapy
Sickle cell disease

113

114 SECTION 4 Elbow Pain Syndromes

the course of the disease, plain radiographs can be notoriously


unreliable; magnetic resonance imaging (MRI) reveals articular
changes before significant changes are evident on plain radiographs (Figure 40-2). Based on the patients clinical presentation, additional testing, including complete blood cell count,
uric acid level, erythrocyte sedimentation rate, and antinuclear
antibody testing, also may be indicated. MRI of the elbow joint
is indicated in all patients thought to have osteonecrosis of the
elbow joint; if other causes of joint instability, infection, or
tumor are suspected; or if plain radiographs are nondiagnostic.
Computed tomography (CT) may be useful in early diagnosis,
especially with three-dimensional reconstruction (Figure 40-3).
Administration of gadolinium followed by postcontrast imaging
may help delineate the adequacy of blood supply, with contrast
enhancement of the elbow joint being a good prognostic sign.
Electromyography is indicated if coexistent cervical radiculopathy or brachial plexopathy is suspected. A very gentle intraarticular injection of the elbow joint with small volumes of local
anesthetic will provide immediate improvement of the pain and
help demonstrate the nidus of the pain is in fact the elbow joint.
Ultimately, total joint replacement will be required in most
patients with osteonecrosis of the elbow joint, although newer
joint preservation techniques are becoming more popular in
younger, more active patients given the short life expectancy of
total shoulder prosthesis.

Differential Diagnosis
Coexistent arthritis and gout of the elbow joint, bursitis, and
tendinitis may coexist with osteonecrosis of the elbow joints and
exacerbate the pain and disability. Tears of the ligaments, bone

cysts, bone contusions, and fractures may mimic the pain of osteonecrosis of the elbow joint, as can occult metastatic disease.

Treatment
Initial treatment of the pain and functional disability associated
with osteonecrosis of the elbow joint should include a combination of the nonsteroidal antiinflammatory drugs (NSAIDs) or
cyclooxygenase-2 (COX-2) inhibitors and decreased weight bearing of the affected elbow joint or joints. Local application of heat
and cold may be beneficial. For patients who do not respond to
these treatment modalities, an injection of a local anesthetic into
the elbow joint may be a reasonable next step to provide palliation
of acute pain. Vigorous exercises should be avoided because they
will exacerbate the symptoms. Ultimately, surgical repair in the
form of total joint arthroplasty is the treatment of choice.

Complications and Pitfalls


Failure to surgically treat significant osteonecrosis of the elbow
joint usually will result in continued pain and disability and in
most patients will lead to ongoing damage to the elbow joint
(see Figure 40-2). Injection of the joint with local anesthetic is
a relatively safe technique if the clinician is attentive to detail,
specifically using small amounts of local anesthetic and avoiding
high injection pressures, which may further damage the joint.
Another complication of this injection technique is infection.
This complication should be exceedingly rare if strict aseptic
technique is followed. Approximately 25% of patients report
a transient increase in pain after this injection technique and
should be warned of this possibility.

Figure 40-2 A, Coronal T2-weighted with fat suppression (FST2W) MRI demonstrating an area of high-signal intensity marrow edema in the
capitellum (solid arrow) of an adolescent with elbow pain. An area of low SI is seen in the subchondral bone plate (dashed arrow), suggestive of an
osteochondral defect. B, The sagittal FST2W MRI more clearly shows the low-SI osteochondral defect (curved arrow), with a linear area of high SI
at its base, indicating that the lesion is likely to be unstable. These appearances are typical of Panners disease (osteochondritis dissecans). (From
Waldman SD: Osteonecrosis of the elbow. In Waldman SD, Campbell RSD, editors: Imaging of pain, New York, 2011, Elsevier, p 282.)

40 Osteonecrosis of the Elbow Joint 115

Po

255/128

255/128

Figure 40-3 Multifocal aspect of elbow osteonecrosis. A, Anterior; P, posterior. (From Mukaza MM, Manicom O, Fillipini P, Hernigou P: Elbow osteonecrosis in sickle cells anemia: a study of six cases, Orthop Traumatol 95:8284, 2009.)

Clinical Pearls
Osteonecrosis of the elbow joint is a diagnosis that is often
missed, leading to considerable unnecessary pain and disability. The clinician should include osteonecrosis of the
elbow joint in the differential diagnosis in all patients with
shoulder joint pain, especially if any of the predisposing factors listed in Table 40-1 are present. Coexistent arthritis,
tendinitis, and gout may contribute to the pain and may
require additional treatment. The use of physical modalities, including local heat and cold and decreased weight
bearing may provide symptomatic relief. Vigorous exercises
should be avoided because they will exacerbate the symptoms and may cause further damage to the wrist. Simple
analgesics and NSAIDs may be used concurrently with this
injection technique.

SUGGESTED READINGS
Henderson AB: Sickle cell anemia: clinical study of fifty-four cases (review), Am J
Med 9:757765, 1950.
Mukaza MM, Manicom O, Fillipini P, Hernigou P: Elbow osteonecrosis in sickle
cells anemia: a study of six cases, Orthop Traumatol 95:8284, 2009.
Savini CJ, James CW: HIV infection and osteonecrosis, J Assoc Nurse AIDS Care
12:8385, 2001.
Waldman SD: Functional anatomy of the elbow. In Waldman SD, editor: Pain
review, Philadelphia, 2009, Saunders, pp 7677.
Waldman SD: Osteonecrosis of the elbow. In Waldman SD, Campbell RSD, editors: Imaging of pain, New York, 2011, Elsevier, pp 281283.
Watanabe R, Sato K, Nakamura T, etal: Steroid-induced osteonecrosis of bilateral distal humerus treated by arthroplasty using costal osteochondral graft: case
report, J Hand Surg 36:816819, 2011.

Chapter 41
TRICEPS TENDINITIS

Signs and Symptoms

ICD-9 CODE 727.09


ICD-10 CODE M65.80
The Clinical Syndrome
Triceps tendinitis is being seen with increasing frequency in
clinical practice as exercising and the use of exercise equipment
have increased in popularity. The triceps tendon is susceptible
to the development of tendinitis at its distal portion and its
insertion on the ulna. The triceps tendon is subject to repetitive motion that may result in microtrauma, which heals poorly
because of the tendons avascular nature. Exercise is often implicated as the inciting factor of acute triceps tendinitis. Tendinitis
of the triceps tendon frequently coexists with bursitis of the associated bursae of the tendon and elbow joint, creating additional
pain and functional disability. Calcium deposition around the
tendon may occur if the inflammation continues, making subsequent treatment more difficult (Figure 41-1). Continued trauma
to the inflamed tendon ultimately may result in tendon rupture
(Figure 41-2).

The onset of triceps tendinitis is usually acute, occurring after overuse


or misuse of the elbow joint. Inciting factors include playing tennis
and aggressive use of exercise machines. Improper stretching of triceps
muscle and triceps tendon before exercise also has been implicated in
the development of triceps tendinitis and acute tendon rupture. Injuries ranging from partial to complete tears of the tendon can occur
when the distal tendon sustains direct trauma while it is fully flexed
under load or when the elbow is forcibly flexed while the arm is fully
extended. The pain of triceps tendinitis is constant and severe and is
localized in the posterior elbow (Figure 41-3). Significant sleep disturbance is often reported. Patients with triceps tendinitis exhibit pain
with resisted extension of the elbow. A creaking or grating sensation
may be palpated when passively extending the elbow. As mentioned,
a chronically inflamed triceps tendon may rupture suddenly with
stress or during vigorous injection procedures inadvertently injected
into the substance of the tendon. With triceps tendon rupture, the
patient is unable to fully and forcefully extend the affected arm.

Testing
Plain radiographs and magnetic resonance imaging (MRI) are
indicated for all patients who present with posterior elbow pain

ST

Figure 41-1 Tendon and soft tissue calcification. Calcified deposits are visualized in the triceps tendon (T) and soft tissues (ST) around the proximal
end of the radius. (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 1581.)

116

41 Triceps Tendinitis 117

Figure 41-2 Triceps tendon rupture imaged in flexion. This patient was unable to extend the elbow because of discomfort. The images were obtained
on a high-field scanner with the patient prone and the arm flexed overhead. Proton density (A) and fat-suppressed T2-weighted (B) coronal images
reveal a fluid-filled tear of the distal triceps tendon (arrows) from the olecranon (O). (From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors: Clinical
magnetic resonance imaging, 3rd ed, Philadelphia, 2006, Saunders, p 3302.)

is useful to identify stress fractures of the elbow not seen on plain


radiographs.

Differential Diagnosis
Triceps tendinitis generally is easily identified on clinical grounds,
but coexistent bursitis may confuse the diagnosis. Stress fractures
of the olecranon also may mimic triceps tendinitis and may be
identified on plain radiographs or radionuclide bone scanning.

Treatment
Initial treatment of the pain and functional disability associated with
triceps tendinitis should include a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also
may be beneficial. Patients should be encouraged to avoid repetitive
activities responsible for the evolution of the tendinitis. For patients
who do not respond to these treatment modalities, injection with
local anesthetic and steroid may be a reasonable next step.

Figure 41-3 The pain of triceps tendinitis is constant and severe and is
localized in the posterior elbow.

(see Figures 41-1 and 41-2). Based on the patients clinical presentation, additional tests, including complete blood count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be
indicated. MRI of the elbow is indicated if joint instability is suspected and to confirm the diagnosis. Radionuclide bone scanning

Complications and Pitfalls


Trauma to the triceps tendon from the injection itself is possible. Tendons that are highly inflamed or previously damaged are
subject to rupture if they are directly injected. This complication
can be greatly decreased if the clinician uses gentle technique and
stops injecting immediately if significant resistance to injection is
encountered. Approximately 25% of patients report a transient
increase in pain after this injection technique, and patients should
be warned of this possibility.

118 SECTION 4 Elbow Pain Syndromes

Clinical Pearls
The triceps tendon is a very strong tendon, but it is also very
susceptible to rupture. Coexistent bursitis and arthritis also
may contribute to posterior elbow pain and may require
additional treatment with a more localized injection of local
anesthetic and methylprednisolone acetate.
Injection of the triceps tendon is a safe procedure if
careful attention is paid to the clinically relevant anatomy
in the areas to be injected. The use of physical modalities,
including local heat and gentle range-of-motion exercises,
should be introduced several days after the patient undergoes this injection technique for elbow pain. Vigorous exercises should be avoided because they would exacerbate the
patients symptoms. Simple analgesics and NSAIDs may be
used concurrently with this injection technique.

SUGGESTED READINGS
Badia A, Stennett C: Sports-related injuries of the elbow, J Hand Ther 19:
206227, 2006.
Jafarnia K, Gabel GT, Morrey BF: Triceps tendinitis, Oper Tech Sports Med
9:217221, 2001.
Potter HG, Schachar J, Jawetz S: Imaging of the elbow, Oper Tech Orthop 19:
199208, 2009.
Waldman SD: Functional anatomy of the elbow. In Waldman SD, editor: Pain
review, Philadelphia, 2009, Saunders, pp 7677.

Chapter 42
RADIAL TUNNEL SYNDROME

ICD-9 CODE 354.9


ICD-10 CODE G56.90

include aberrant fibrous bands in front of the radial head, anomalous blood vessels that compress the nerve, extrinsic masses, or a
sharp tendinous margin of the extensor carpi radialis brevis. These
entrapments may exist alone or in combination.

Signs and Symptoms


The Clinical Syndrome
Radial tunnel syndrome is an uncommon cause of lateral elbow
pain that has the unique distinction among entrapment neuropathies of almost always being initially misdiagnosed. The incidence
of misdiagnosis of radial tunnel syndrome is so common that it is
often incorrectly referred to as resistant tennis elbow (Table 42-1).
As seen from the following discussion, the only major similarity
that radial tunnel syndrome and tennis elbow share is the fact that
both clinical syndromes produce lateral elbow pain.
The lateral elbow pain of radial tunnel syndrome is aching
and localized to the deep extensor muscle mass. The pain may
radiate proximally and distally into the upper arm and forearm
(Figure 42-1). The intensity of the pain of radial tunnel syndrome is mild to moderate, but it may produce significant functional disability.
In radial tunnel syndrome, the posterior interosseous branch of
the radial nerve is entrapped by a variety of mechanisms that have
in common a similar clinical presentation. These mechanisms

Regardless of the mechanism of entrapment of the radial nerve,


the common clinical feature of radial tunnel syndrome is pain just
below the lateral epicondyle of the humerus. The pain of radial
tunnel syndrome may develop after an acute twisting injury or
direct trauma to the soft tissues overlying the posterior interosseous branch of the radial nerve, or the onset may be more insidious, without an obvious inciting factor. The pain is constant and
worsens with active supination of the wrist. Patients often note
the inability to hold a coffee cup or hammer. Sleep disturbance
is common. On physical examination, elbow range of motion is
normal. Grip strength on the affected side may be diminished.
In the classic text on entrapment neuropathies, Dawson and
colleagues note three important signs that allow the clinician to
distinguish radial tunnel syndrome from tennis elbow: (1) tenderness to palpation distal to the radial head in the muscle mass of the
extensors, rather than over the more proximal lateral epicondyle,
as in tennis elbow; (2) increasing pain on active resisted supination of the forearm owing to compression of the radial nerve by
the arcade of Frohse as a result of contraction of the muscle mass;

TABLE 42-1

Characteristics of Radial Tunnel Syndrome and Lateral Epicondylitis


Characteristic

Radial Tunnel Syndrome

Lateral Epicondylitis(Tennis Elbow)

Frequency

Rare (2% of all peripheral nerve compressions of the upper


limb)

Common cause of lateral elbow pain

Cause

Compression of the radial nerve

Caused by overuse of the extensor and supinator muscles

Characteristic patient

Anybody with repetitive, stressful pronation and supination


(e.g., tennis players, Frisbee players, swimmers, powerlifters)

Tennis players

Pain location

Pain over the neck of the radius and lateral aspect of the
proximal forearm over the extensor muscles themselves
(distal to where the pain is located in LE)

Pain and tenderness over the lateral epicondyle and immediately distal to it (at the
origin of the extensor muscles)

Pain radiation

Pain can radiate proximally and (more commonly) distally

Usually localized without radiation

Provocative tests (much overlap


between the two entities)

Pain with resisted extension of the middle finger with the


forearm pronated and the elbow extended. Pain with resisted
forearm supination with the elbow fully extended

Pain with resisted wrist extension or elbow


supination with the elbow extended. Pain with
forceful wrist flexion or forearm pronation

Modified from Mileti J, Largacha M, ODriscoll SW. Radial tunnel syndrome caused by ganglion cyst: treatment by arthroscopic cyst decompression, Arthroscopy
20:e39e44, 2004.

119

120 SECTION 4 Elbow Pain Syndromes

Differential Diagnosis

Radial nerve

Cervical radiculopathy and tennis elbow can mimic radial tunnel syndrome. Radial tunnel syndrome can be distinguished from
tennis elbow because with radial tunnel syndrome, the maximal
tenderness to palpation is distal to the lateral epicondyle over the
posterior interosseous branch of the radial nerve, whereas with
tennis elbow, the maximal tenderness to palpation is over the
lateral epicondyle. Increased pain with active supination and a
positive middle finger test (see earlier discussion) helps strengthen
the diagnosis of radial tunnel syndrome. Acute gout affecting the
elbow manifests as a diffuse acute inflammatory condition that
may be difficult to distinguish from infection of the joint, rather
than a localized nerve entrapment.

Treatment
Extensor carpi
radialis brevis
muscle

Figure 42-1 The pain of radial tunnel syndrome is localized to the deep
extensor muscle mass and may radiate proximally and distally into the
upper arm and forearm.

and (3) a positive result on the middle finger test. The middle
finger test is performed by having the patient extend the forearm,
wrist, and middle finger and sustain this action against resistance.
Patients with radial tunnel syndrome exhibit increased lateral
elbow pain secondary to fixation and compression of the radial
nerve by the extensor carpi radialis brevis muscle.

Testing
Because of the ambiguity and confusion surrounding this clinical syndrome, testing is important to help confirm the diagnosis
of radial tunnel syndrome. Electromyography helps to distinguish cervical radiculopathy and radial tunnel syndrome from
tennis elbow. Plain radiographs are indicated in all patients who
present with radial tunnel syndrome to rule out occult bony
pathology. Based on the patients clinical presentation, additional testing, including complete blood cell count, uric acid,
erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated.
Magnetic resonance imaging (MRI) of the elbow is indicated
if internal derangement of the joint is suspected and may help
identify the factors responsible for the nerve entrapment, such as
ganglion cysts or lipomas (Figure 42-2). The injection technique
of the radial nerve at the elbow with a local anesthetic and steroid
may help confirm the diagnosis and treat the syndrome.

Initial treatment of the pain and functional disability associated


with radial tunnel syndrome should include a combination of
nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. The local application of heat and cold also may be beneficial. Patients should avoid
the repetitive movements that incite the syndrome. For patients
who do not respond to these treatment modalities, injection of the
radial nerve at the elbow with a local anesthetic and steroid may be
a reasonable next step. If the symptoms of radial tunnel syndrome
persist, surgical exploration and decompression of the radial nerve
are indicated.

Complications and Pitfalls


The major complications associated with radial tunnel syndrome
fall into two categories: (1) iatrogenically induced complications
resulting from persistent and overaggressive treatment of resistant
tennis elbow and (2) the potential for permanent neurological
deficits as a result of prolonged untreated entrapment of the radial
nerve. Failure of the clinician to recognize an acute inflammatory
or infectious arthritis of the elbow may result in permanent damage to the joint and chronic pain and functional disability.

Clinical Pearls
Radial tunnel syndrome is a distinct clinical entity that is
often misdiagnosed as tennis elbow, and this fact accounts
for the many patients with tennis elbow who fail to
respond to conservative measures. Radial tunnel syndrome
can be distinguished from tennis elbow because with radial
tunnel syndrome, the maximal tenderness to palpation is
over the radial nerve, whereas with tennis elbow, the maximal tenderness to palpation is over the lateral epicondyle.
If radial tunnel syndrome is suspected, injection of the
radial nerve at the humerus with a local anesthetic and steroid gives almost instantaneous relief. Careful neurological
examination to identify preexisting neurological deficits
that may later be attributed to the nerve block should be
performed on all patients before beginning radial nerve
block at the humerus.

42 Radial Tunnel Syndrome 121

Post-op

Pre-op

Pre-op

C
Pre-op

Post-op

E
Post-op

Figure 42-2 Preoperative and postoperative magnetic resonance imaging (MRI) (T2-weighted fast spin echo sequences with fat saturation). A,
Sagittal MRI shows cystic mass anterior to the capitellum. B, Postoperative sagittal MRI shows decompression of the cyst. C, Preoperative coronal
MRI shows cyst communication with the proximal radioulnar joint. D, Postoperative coronal MRI after decompression. E, Series of preoperative axial
MRIs showing the cyst from the anterior to the capitellum distally into the proximal radioulnar joint. F, Postoperative axial MRIs after decompression.
(From Mileti J, Largacha M, ODriscoll SW: Radial tunnel syndrome caused by ganglion cyst: treatment by arthroscopic cyst decompression, Arthroscopy
20:e39e44, 2004.)
SUGGESTED READINGS
Clavert P, Lutz JC, Adam P: Frohses arcade is not the exclusive compression site
of the radial nerve in its tunnel, Orthop Traumatol Surg Res 95:114118, 2009.
Lee JT, Azari K: Ford Jones N: Long term results of radial tunnel release: the effect
of co-existing tennis elbow, multiple compression syndromes and workers
compensation, J Plast Reconstr Aesthet Surg 61:10951099, 2008.
Huisstede B, Miedema HS, van Opstal T: Interventions for treating the radial tunnel syndrome: a systematic review of observational studies, J Hand Surg 33:72,
2008, e1-72.e10.

Tennent TD, Woodgate A: Posterior interosseous nerve dysfunction in the radial


tunnel, Curr Orthop 22:226232, 2008.
Waldman SD: Radial tunnel syndrome. In Waldman SD, editor: Pain review,
Philadelphia, 2009, Saunders, pp 268269.
Waldman SD: Radial tunnel syndrome. In Waldman SD, Campbell RSD, editors:
Imaging of pain, Philadelphia, 2011, Saunders, pp 287288.

Chapter 43
CUBITAL TUNNEL SYNDROME

ICD-9 CODE 354.2


ICD-10 CODE G56.2
The Clinical Syndrome
Cubital tunnel syndrome is an uncommon cause of lateral forearm pain and weakness that can be quite distressing to the patient.
This entrapment neuropathy manifests as pain and associated
paresthesias in the lateral forearm that radiates to the wrist and
ring and little fingers. The symptoms are often aggravated by prolonged flexion of the elbow. The pain of cubital tunnel syndrome
has been characterized as unpleasant and dysesthetic. The onset
of symptoms is usually after repetitive elbow motions or from
repeated pressure on the elbow, such as using the elbows to arise
from bed. Direct trauma to the ulnar nerve as it enters the cubital tunnel may result in a similar clinical presentation. Untreated,
progressive motor deficit and ultimately flexion contracture of
the affected fingers can result. Cubital tunnel syndrome is most
often caused by compression of the ulnar nerve by an aponeurotic
band that runs from the medial epicondyle of the humerus to the
medial border of the olecranon.

Signs and Symptoms


Physical findings include tenderness over the ulnar nerve at the
elbow. A positive Tinels sign over the ulnar nerve as it passes beneath
the aponeuroses is usually present. Weakness of the intrinsic muscles
of the forearm and hand that are innervated by the ulnar nerve may
be identified with careful manual muscle testing, although early in
the course of the evolution of cubital tunnel syndrome, the only
physical finding other than tenderness over the nerve may be the loss
of sensation on the ulnar side of the little finger. As the syndrome
progresses, the affected hand may take on a clawlike appearance
(Figure 43-1). A positive Wartenbergs sign indicative of weakness
of the adduction of the fifth digit is often present (Figures 43-2 and
43-3). A positive scratch collapse test is often present (Figure 43-4).

Testing
Electromyography helps distinguish cervical radiculopathy and
cubital tunnel syndrome from golfers elbow. Plain radiographs are
indicated in all patients with cubital tunnel syndrome to rule out
occult bony pathological processes, such as osteophytes impinging
on the ulnar nerve. Based on the patients clinical presentation,
122

additional tests, including complete blood cell count, uric acid


level, erythrocyte sedimentation rate, and antinuclear antibody
testing, may be indicated. Magnetic resonance imaging (MRI)
of the elbow is indicated if joint instability is suspected and to
identify the cause of ulnar nerve entrapment (Figure 43-5). Ultrasound evaluation is also useful if the diagnosis is in question
(Figure 43-6). Injection of the ulnar nerve serves as a diagnostic
maneuver and a therapeutic maneuver.

Differential Diagnosis
Cubital tunnel syndrome is often misdiagnosed as golfers elbow,
which accounts for the many patients with golfers elbow who
fail to respond to conservative measures. Cubital tunnel syndrome
can be distinguished from golfers elbow, because in cubital tunnel syndrome, the maximal tenderness to palpation is over the
ulnar nerve 1 inch below the medial epicondyle, whereas with
golfers elbow, the maximal tenderness to palpation is directly
over the medial epicondyle. Cubital tunnel syndrome also should
be differentiated from cervical radiculopathy involving the C7
or C8 roots and golfers elbow. Cervical radiculopathy and ulnar
nerve entrapment may coexist as the double crush syndrome.
The double crush syndrome is seen most commonly with median
nerve entrapment at the wrist or carpal tunnel syndrome.

Treatment
Initial treatment of the pain and functional disability associated
with cubital tunnel syndrome should include a combination of
nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of
heat and cold also may be beneficial. The repetitive movements that
incite the syndrome should be avoided. For patients who do not
respond to these treatment modalities, injection of the ulnar nerve
at the elbow with a local anesthetic and steroid may be a reasonable
next step. If the symptoms of cubital tunnel syndrome persist, surgical exploration and decompression of the ulnar nerve are indicated.

Complications and Pitfalls


The major complications associated with cubital tunnel syndrome
fall into two categories: (1) iatrogenically induced complications
resulting from persistent and overaggressive treatment of resistant
golfers elbow and (2) the potential for permanent neurological
deficits as a result of prolonged untreated entrapment of the ulnar
nerve. Failure of the clinician to recognize an acute inflammatory
or infectious arthritis of the elbow may result in permanent damage to the joint and chronic pain and functional disability.

Ulnar nerve

Medial epicondyle

Ulnar
nerve

Flexor carpi
ulnaris muscle

Ulnar collateral
ligament

Medial
epicondyle

Figure 43-1 Patients with cubital tunnel syndrome exhibit weakness of the intrinsic muscles of the forearm, and the hand may take on a clawlike
appearance.

Figure 43-2 Adduction of the fifth digit is often present in cubital tunnel syndrome. (From Waldman SD: Physical diagnosis of pain: an atlas of
signs and symptoms, Philadelphia, 2006, Saunders, p 127.)

Figure 43-3 A positive Wartenbergs sign is indicative of cubital tunnel


syndrome. (From Waldman SD: Physical diagnosis of pain: an atlas of
signs and symptoms, Philadelphia, 2006, Saunders, p 128.)

124 SECTION 4 Elbow Pain Syndromes


Patient

Examiner

Figure 43-4 The scratch collapse test. The patient faces the examiner with arms adducted, elbows flexed, and hands outstretched with the wrists at
neutral. A, The patient resists bilateral shoulder adduction and internal rotation as the examiner applies these forces to the forearm. B, The examiner
scratches or swipes the fingertips over the course of the compressed ulnar nerve. C, The force is reapplied to the forearm. A positive result occurs
when the patient has a temporary loss of external rotation resistance tone (as seen in the diagram). (From Cheng CJ, Mackinnon-Patterson B, Beck JL,
Mackinnon SE: Scratch collapse test for evaluation of carpal and cubital tunnel syndromes, J Hand Surg 33A:15181524, 2008.)

Figure 43-5 Thickening of the cubital tunnel retinaculum. A, T1-weighted axial MR image reveals the ulnar nerve (white arrow) deep to a thickened
cubital tunnel retinaculum (arrowheads) and superficial to the posterior bundle of the medial collateral ligament (curved arrow). B, Axial image further
distally in the same patient reveals the ulnar nerve (white arrow) deep to a normal, thin aponeurosis of the flexor carpi ulnaris (small black arrows) and
superficial to a mildly thickened medial joint capsule (open arrow). (From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors: Clinical magnetic resonance
imaging, 3rd ed, Philadelphia, 2006, Saunders, p 3303.)

Ulna
ME

Tunnel

Figure 43-6 In this patient with UCT the ulnar nerve has a cross sectional anatomy (CSA) of 0.29 cm2. The cross-section of the ulnar nerve is
depicted by arrows outlining its periphery. The nerve also is hypoechoic,
a finding that can be seen with increased edema. ME, Medial epicondyle; Tunnel, ulnar tunnel. (From Wiesler ER, Chloros GD, Cartwright MS,
Shin HW, Walker FO: Ultrasound in the diagnosis of ulnar neuropathy at the
cubital tunnel, J Hand Surg 31:10881093, 2006.)

Clinical Pearls
Cubital tunnel syndrome is a distinct clinical entity that is
often misdiagnosed as golfers elbow, which accounts for
the many patients with golfers elbow who fail to respond
to conservative measures. Cubital tunnel syndrome can be
distinguished from golfers elbow because in cubital tunnel
syndrome, the maximal tenderness to palpation is over the
ulnar nerve and a positive Tinels sign is present, whereas
with golfers elbow, the maximal tenderness to palpation
is over the medial epicondyle. If cubital tunnel syndrome
is suspected, injection of the radial nerve at the elbow with
a local anesthetic and steroid gives almost instantaneous
relief. Careful neurological examination to identify preexisting neurological deficits that may later be attributed to
the nerve block should be performed on all patients before
beginning ulnar nerve block at the elbow.

43 Cubital Tunnel Syndrome 125


SUGGESTED READINGS
Hariri S, McAdams TR: Nerve injuries about the elbow, Clin Sports Med 29:655
675, 2010.
Heithoff SJ: Cubital tunnel syndrome: ulnar nerve subluxation, J Hand Surg
35:1556, 2010.

Palmer BA, Hughes TB: Cubital tunnel syndrome, J Hand Surg 35:153163,
2010.
Rich BC, McKay MP: The cubital tunnel syndrome: a case report and discussion,
J Emerg Med 23:347350, 2002.

Chapter 44
DRIVERS ELBOW

ICD-9 CODE 354.2


ICD-10 CODE G56.20
The Clinical Syndrome
The ulnar nerve is susceptible to compression when a driver or
passenger rests his or her elbow on the lower sill of the vehicle
window while the shoulder is abducted and the elbow flexed.
When the elbow is flexed, the proximal edge of the arcuate ligament becomes taut and the total volume of the cubital tunnel
is decreased, resulting in increased intratunnel pressure further
compromising the ulnar nerve. Vibration transmitted from the
car body to the elbow also may further contribute to compromise
of the ulnar nerve. This entrapment neuropathy presents as pain
and associated paresthesias in the lateral forearm that radiate to
the wrist and ring and little finger. Untreated, progressive motor
deficit and, ultimately, flexion contracture of the affected fingers
can result.

Signs and Symptoms


Physical findings associated with drivers elbow include tenderness
over the ulnar nerve at the elbow. A positive Tinels sign over the
ulnar nerve as it passes beneath the aponeuroses is usually present
(Figure 44-1). Weakness of the intrinsic muscles of the forearm

and hand that are innervated by the ulnar nerve may be identified with careful manual muscle testing (Table 44-1). It should be
noted that the possibility always exists that a patient with drivers
elbow also may have an coexistent ulnar, median, or radial nerve
lesion distal to the elbow that may confuse the clinical picture.
Furthermore, it should be remembered that cervical radiculopathy
and ulnar nerve entrapment may coexist as the double crush
syndrome. The double crush syndrome is seen most commonly
with median nerve entrapment at the wrist or with carpal tunnel
syndrome. The clinician should be aware that early in the course
of the evolution of drivers elbow the only physical finding other
than tenderness over the nerve may be the loss of sensation on the
ulnar side of the little finger.

Testing
Drivers elbow should be differentiated from cervical radiculopathy involving the C7 or C8 roots and golfers elbow. Electromyography helps distinguish cervical radiculopathy and drivers
elbow from golfers elbow. Ultrasound imaging of the elbow
may be useful in assessing the status of the ulnar nerve and can
provide important anatomic information when combined with
the neurophysiological data obtained from electromyography.
Plain radiographs and magnetic resonance imaging (MRI) are
indicated in all patients with drivers elbow to rule out intrinsic
pathological conditions of the elbow joint (Figure 44-2). Based
on the patients clinical presentation, additional testing, including complete blood count, uric acid level, sedimentation rate,
and antinuclear antibody testing, may be indicated. The injection
technique described in this chapter serves as both a diagnostic and
therapeutic maneuver.

Differential Diagnosis
Drivers elbow is an entrapment neuropathy resulting from external compression of the ulnar nerve that clinically mimics cubital
tunnel syndrome. It is often is misdiagnosed as golfers elbow,
which accounts for the many patients with golfers elbow who
fail to respond to conservative measures. Drivers elbow can be distinguished from golfers elbow in that in drivers elbow, the maximal tenderness to palpation is over the ulnar nerve 1 inch below
the medial epicondyle, whereas with golfers elbow, the maximal
tenderness to palpation is directly over the medial epicondyle.

Treatment
Figure 44-1 Tinels sign at elbow. (From Waldman SD: Atlas of pain management injection techniques, 3rd ed, Philadelphia, 2013, Saunders, p 129.)

126

Initial treatment of the pain and functional disability associated


with drivers elbow should include a combination of nonsteroidal

44 Drivers Elbow 127


TABLE 44-1

Summary of Ulnar Nerve Motor Signs and Tests Grouped by Affected Musculature
Test Name

Description

Positive Result

Motor signs involving the adductor pollicis muscle


Froments sign

The patient holds a piece of paper using a lateral pinch.


The examiner then pulls the paper distally along the
thumbs longitudinal axis and assesses the patients
method of stabilization.

Thumb IP flexion compensates for a weak adductor


pollicis muscle.

Jeannes sign

The patient holds a piece of paper using a lateral pinch.


The examiner then pulls the paper distally along the
thumbs longitudinal axis and assesses the patients
method of stabilization.

Thumb MP hyperextension compensates for a weak


adductor pollicis muscle.

Motor signs and tests involving the interosseous muscles


Finger flexion sign

Performed bilaterally at the same time. Both forearms and


wrists are in neutral. Examiner first places a piece of paper
between the middle and ring fingers in both hands and
then pulls the paper distally.

The involved side will use MP flexion to compensate


for interossei weakness.

Crossed finger test

Examiner asks the patient to cross the middle finger over


the index finger.

Inability to cross the fingers. Compare with uninvolved side.

Egawas sign

Examiner then asks the patient to flex the middle finger


MP joint and then to abduct it to both sides. This can
be difficult to perform; therefore bilateral assessment is
recommended.

Inability to perform this action in contrast to uninvolved side.

Motor signs involving the ulnar nerveinnervated lumbrical muscles


Duchennes sign

Sign is identified by observing the posture of the small


and ring fingers on the involved side.

Clawing posture (MP hyperextension and IP flexion)


present in the ring and small fingers.

Andr-Thomas sign

Sign is identified by observing the compensatory pattern


used in the ring and small fingers during actions involving EDC use.

Wrist tends to flex with ring and small finger EDC


activation.

Motor signs involving the hypothenar musculature


Wartenbergs sign

Patient actively abducts the fingers with the forearm in


pronation and the wrist in neutral. Observe the small
fingers ability to fully adduct.

Inability of the small finger to fully adduct and


touch the ring finger. Compare with the uninvolved
side.

Masses sign

Observe the metacarpal arch as compared with the uninvolved side. The convex nature of the ulnar aspect of the
hand is altered by hypothenar atrophy.

Flattened metacarpal arch.

Pitres-Testut sign

Noted after the examiner asks the patient to shape the


hand in the form of a cone. Although present in the literature, this sign is not commonly used in clinical practice
settings.

Inability to shape the hand in the form of a cone.

Palmaris brevis sign

A rarely observed sign in lower ulnar nerve palsy in which


the lesion selectively affects the deep branch. Determine
the presence of this sign by observing and evaluating the
palmaris brevis muscle in contrast to the uninvolved side.

The sparing of the palmaris brevis muscle in contrast to the uninvolved side.

Motor signs involving the extrinsic ulnar nerveinnervated muscles


Nail file sign

Patient attempts to make a hook fist. Examiner places an


index finger along the volar surface of the patients small
and ring fingers, leaving the DIPs free to contract.

Decreased small and ring finger FDP strength in


contrast to the uninvolved side.

Modified from Goldman SB, Brininger TL, Schrader JW, Koceja DM: A review of clinical tests and signs for the assessment of ulnar neuropathy, J Hand Ther 22:209220,
2009.
DIP, Distal interphalangeal; EDC, extensor digitorum communis; FDP, flexor digitorum profundus; IP, interphalangeal; MP, metacarpophalangeal.

anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX2) inhibitors and physical therapy. Local application of heat
and cold also may be beneficial. The repetitive movements that
incite the syndrome should be avoided. For patients who do not
respond to these treatment modalities, injection of the ulnar
nerve at the elbow with a local anesthetic and steroid may be a
reasonable next step. If the symptoms of cubital tunnel syndrome
persist, surgical exploration and decompression of the ulnar nerve
are indicated.

Complications and Pitfalls


The major complications associated with the diagnosis and treatment
of patients with drivers elbow fall into two categories: (1) iatrogenically induced complications resulting from persistent and overaggressive treatment of resistant golfers elbow and (2) the potential for
permanent neurological deficits as a result of prolonged untreated
entrapment of the ulnar nerve. Failure of the clinician to recognize
acute inflammatory or infectious arthritis of the elbow may result in
permanent damage to the joint, chronic pain, or functional disability.

128 SECTION 4 Elbow Pain Syndromes

LE
ME
O

Figure 44-2 A, Axial T1-weighted magnetic resonance imaging (MRI) of a patient with symptoms of ulnar nerve compression. Soft tissue is seen
within the region of the cubital tunnel (white arrow); it is isointense, with normal muscle and represents an accessory anconeus muscle. The ulnar
nerve is not clearly visible. B, Compare this axial T1-weighted image of a normal elbow with high signal intensity fat suppression (FS) within the
cubital tunnel around the ulnar nerve (broken black arrow) and no accessory muscle tissue. The axial (C) and sagittal FS T2-weighted MRI demonstrate
high signal intensity within the nerve (white arrows) resulting from compression neuritis. LE, Lateral epicondyle; ME, medial epicondyle; O, olecranon.
(From Waldman SD, Campbell RSD, editors: Imaging of pain, Philadelphia, 2011, Saunders, p 290.)

44 Drivers Elbow 129

Clinical Pearls
Drivers elbow is a distinct clinical entity often misdiagnosed as golfers elbow, which accounts for the many
patients with golfers elbow who fail to respond to conservative measures. Drivers elbow can be distinguished
from golfers elbow because, with cubital tunnel syndrome,
the maximal tenderness to palpation is over the ulnar nerve
and a positive Tinels sign is present, whereas with golfers
elbow, the maximal tenderness to palpation is over the
medial epicondyle. Drivers elbow also should be differentiated from cervical radiculopathy involving the C8 spinal
root, which may at times mimic ulnar nerve compression.
Furthermore, it should be remembered that cervical radiculopathy and ulnar nerve entrapment may coexist in double
crush syndrome. The double crush syndrome is seen most
commonly with median nerve entrapment at the wrist or
with carpal tunnel syndrome. Pancoasts tumor invading
the medial cord of the brachial plexus may also mimic an
isolated ulnar nerve entrapment and should be ruled out by
apical lordotic chest radiograph.
Careful neurological examination to identify preexisting neurological deficits that may later be attributed to
the nerve block should be performed on all patients before
beginning ulnar nerve block at the elbow.
Ulnar nerve entrapment at the elbow is often misdiagnosed as golfers elbow, and this fact accounts for the many
patients whose golfers elbow fails to respond to conservative measures. Drivers elbow can be distinguished from
golfers elbow in that in drivers elbow, the maximal tenderness to palpation is over the ulnar nerve 1 inch below the
medial epicondyle, whereas with golfers elbow, the maximal tenderness to palpation is directly over the medial epicondyle. If cubital tunnel syndrome is suspected, injection
of the ulnar nerve at the elbow with local anesthetic and
steroid gives almost instantaneous relief.

SUGGESTED READINGS
Abdel-Salam A, Eyres KS, Cleary J: Drivers elbow: a cause of ulnar neuropathy,
JHand Surg 16:436437, 1991.
Palmer BA, Hughes TB: Cubital tunnel syndrome, J Hand Surg 35:153163,
2010.
Szabo RM, Kwak C: Natural history and conservative management of cubital tunnel syndrome, Hand Clin 23:311318, 2007.
Waldman SD: Golfers elbow. In Waldman SD, editor: Pain review, Philadelphia,
2009, Saunders, pp 267268.
Waldman SD: The ulnar nerve. In Waldman SD, editor: Pain review, Philadelphia, 2009, Saunders, pp 76.
Waldman SD: Ulnar nerve entrapment at the elbow. In Waldman SD, editor:
Pain review, Philadelphia, 2009, Saunders, pp 270271.

Chapter 45
ANTERIOR INTEROSSEOUS
SYNDROME
ICD-9 CODE 354.9
ICD-10 CODE G56.90

uric acid level, erythrocyte sedimentation rate, and antinuclear


antibody testing, may be indicated. Magnetic resonance imaging
(MRI) of the forearm is indicated to help clarify the diagnosis
and if a primary elbow pathological process or a space-occupying
lesion is suspected (Figure 45-2). Injection of the median nerve at
the elbow serves as a diagnostic and therapeutic maneuver.

The Clinical Syndrome

Differential Diagnosis

Anterior interosseous syndrome is an uncommon cause of forearm and wrist pain. The onset of symptoms in patients with
anterior interosseous syndrome is usually after acute trauma to
the forearm or after repetitive forearm and elbow motions, such
as using an ice pick. In this setting, the pain and muscle weakness
of anterior interosseous syndrome are thought to be secondary
to median nerve compression of the nerve just below the elbow
by the tendinous origins of the pronator teres muscle and flexor
digitorum superficialis muscle of the long finger or by aberrant
blood vessels. In some patients, no antecedent trauma is identified, and an inflammatory cause analogous to Parsonage-Turner
syndrome has been suggested as the cause of anterior interosseous syndrome in the absence of trauma.
Clinically, anterior interosseous syndrome manifests as acute
pain in the proximal forearm and deep in the wrist. As the syndrome progresses, patients with anterior interosseous syndrome
may report a tired or heavy sensation in the forearm with minimal
activity and the inability to pinch items between the thumb and
index finger because of paralysis of the flexor pollicis longus and
the flexor digitorum profundus (Figure 45-1).

The anterior interosseous syndrome also should be differentiated


from cervical radiculopathy involving the C6 or C7 roots, which
sometimes may mimic median nerve compression. Cervical radiculopathy and median nerve entrapment may coexist as the double
crush syndrome. The double crush syndrome is seen most commonly with median nerve entrapment at the wrist or carpal tunnel
syndrome. Anterior interosseous syndrome can be distinguished
from pronator syndrome and median nerve compression by the
ligament of Struthers, because the pain of anterior interosseous syndrome occurs more distally and is accompanied by the characteristic
loss of ability to pinch items between the thumb and index finger.

Signs and Symptoms


Physical findings include the inability to flex the interphalangeal
joint of the thumb and the distal interphalangeal joint of the index
finger resulting from paralysis of the flexor pollicis longus and the
flexor digitorum profundus. Tenderness over the forearm in the
region of the pronator teres muscle is seen in some patients with
anterior interosseous syndrome. A positive Tinels sign over the
anterior interosseous branch of the median nerve approximately 6
to 8 cm below the elbow also may be present.

Testing
Electromyography helps distinguish cervical radiculopathy, thoracic outlet syndrome, and carpal tunnel syndrome from anterior interosseous syndrome. Plain radiographs are indicated in all
patients who present with anterior interosseous syndrome to rule
out occult bony pathology. Based on the patients clinical presentation, additional tests, including complete blood cell count,
130

Treatment
Nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represent a reasonable first step in
the treatment of anterior interosseous syndrome. The use of the
tricyclic antidepressants, such as nortriptyline, at a single bedtime
dose of 25 mg, titrating upward as side effects allow, also is useful, especially if sleep disturbance is present. It is important for
the patient to avoid repetitive trauma thought to be contributing
to this entrapment neuropathy. If these maneuvers fail to produce rapid symptomatic relief, injection of the median nerve at
the elbow with a local anesthetic and steroid is a reasonable next
step. If symptoms persist, surgical exploration and release of the
anterior interosseous branch of the median nerve are indicated.

Complications and Pitfalls


Median nerve block below the elbow is a relatively safe block, with
major complications being inadvertent intravascular injection and
persistent paresthesia secondary to needle trauma to the nerve.
This technique can be performed safely in the presence of anticoagulation by using a 25- or 27-gauge needle, albeit at increased
risk for hematoma, if the clinical situation dictates a favorable
risk-to-benefit ratio. These complications can be decreased if
manual pressure is applied to the area of the block immediately
after injection. Application of cold packs for 20-minute periods
after the block also decreases the amount of postprocedure pain
and bleeding the patient may experience.

45 Anterior Interosseous Syndrome 131

Muscle paralysis:
Nerve compression:
Normal

Pronator teres
muscle

Median nerve
Muscle paralysis

Pronator digitorum
superficialis muscle
Anterior interosseous
branch of median nerve

Flexor pollicis
longus muscle
Flexor digitorum
profundus muscle

Figure 45-1 Patients with anterior interosseous syndrome exhibit acute forearm pain and progressive weakness of pinch.

132 SECTION 4 Elbow Pain Syndromes

C
Figure 45-2 A, Axial T1-weighted magnetic resonance imaging (MRI) of the mid-forearm in a patient with weakness in muscles in the distribution of
the anterior interosseous nerve. The forearm appears normal on the T1-weighted image, but the axial FST2-weighted image (B) shows high signal
intensity within the muscles of the flexor pollicis longus (FPL), index finger tendon (FDP2) and middle finger tendon (FDP3) (arrows), which are significantly reduced in bulk. This pattern is typical of denervation edema and atrophy. C, The axial FST2-weighted image of the distal forearm shows
similar high signal intensity denervation edema in the pronator quadratus muscle (arrows). (Waldman SD:. In Waldman SD, Campbell RSD, editors:
Imaging of pain, Philadelphia, 2011, Saunders, p 291)

Clinical Pearls
Avoidance techniques for the repetitive movements responsible for pronator syndrome are often forgotten in the rush
to treatment. Median nerve block at the elbow is a simple
and safe technique in the evaluation and treatment of the
aforementioned painful conditions. Careful neurological
examination to identify preexisting neurological deficits
that may later be attributed to the nerve block should be
performed in all patients before beginning median nerve
block at the elbow.

SUGGESTED READINGS
Chi Y, Harness NG: Anterior interosseous nerve syndrome, J Hand Surg 35:2078
2080, 2010.
Douglas H, Chin CL, Meals RA: Anterior interosseous nerve syndrome, J Am Soc
Surg Hand 1:249257, 2001.
Feldman MI, Muhammad K, Beltran J: Preoperative diagnosis of anterior interosseous nerve syndrome resulting in complete recovery, Eur J Radiol Extra
69:e73e76, 2009.
Waldman SD: Anterior interosseous syndrome. In Waldman SD, editor: Pain
review, Philadelphia, 2009, Saunders, pp 271272.
Waldman SD: Anterior interosseous syndrome. In Waldman SD, Campbell RSD,
editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 291293.

SECTION 5 Wrist and Hand Pain Syndromes

Chapter 46
ULNAR TUNNEL SYNDROME
ICD-9 CODE 354.2

neuropathy. Clinically, this mixed neuropathy manifests as pain


and the previously described motor deficits.

ICD-10 CODE G56.20

Signs and Symptoms

The Clinical Syndrome


Ulnar tunnel syndrome is an entrapment neuropathy of the ulnar
nerve characterized by pain, numbness, and paresthesias of the
wrist that radiate into the ulnar aspect of the palm and dorsum
of the hand and the little finger and the ulnar half of the ring
finger. These symptoms also may radiate proximal to the nerve
entrapment into the forearm. The pain of ulnar tunnel syndrome
is often described as aching or burning, with associated pins and
needles paresthesias.
Similar to carpal tunnel syndrome, ulnar tunnel syndrome
occurs more commonly in women than in men. Also similar to
carpal tunnel syndrome, the pain of ulnar tunnel syndrome is
frequently worse at night and worsened by vigorous flexion and
extension of the wrist. The onset of symptoms usually follows
repetitive wrist motions or from direct trauma to the wrist, such
as wrist fractures, or direct trauma to the proximal hypothenar
eminence, such as may occur when the hand is used to hammer
on hubcaps or from handlebar compression during long-distance
cycling. Ulnar tunnel syndrome also is seen in patients with
rapid weight gain, rheumatoid arthritis, or Dupuytrens disease
or during pregnancy. Untreated, progressive motor deficit and
ultimately flexion contracture of the affected fingers can result.
Ulnar tunnel syndrome is caused by compression of the ulnar
nerve as it passes through Guyons canal at the wrist (Figure 46-1).
The most common causes of compression of the ulnar nerve at
this anatomical location include space-occupying lesions, such
as ganglion cysts and ulnar artery aneurysms; fractures of the
distal ulna and carpals; and repetitive motion injuries that compromise the ulnar nerve as it passes through this closed space.
This entrapment neuropathy manifests most commonly as a
pure motor neuropathy without pain, which is due to compression of the deep palmar branch of the ulnar nerve as it passes
through Guyons canal. This pure motor neuropathy manifests
as painless paralysis of the intrinsic muscles of the hand. Ulnar
tunnel syndrome also may manifest as a mixed sensory and motor

Physical findings include tenderness over the ulnar nerve at the


wrist. A positive Tinels sign over the ulnar nerve as it passes
beneath the transverse carpal ligament is usually present. If the
sensory branches are involved, decreased sensation occurs into the
ulnar aspect of the hand and the little finger and the ulnar half of
the ring finger. Depending on the location of neural compromise,
the patient may have weakness of the intrinsic muscles of the hand
as evidenced by the inability to spread the fingers, weakness of the
hypothenar eminence, or both.

ery

Ar t

Moto

Sens

ory

Figure 46-1 The ulnar nerve can be divided into sensory (palmar) and
motor (dorsal) branches. Note the fibrous arch of the hypothenar muscles under which the deep motor branch passes on its way out of the
ulnar tunnel. The ulnar artery travels along the radial side of the nerve
through the tunnel, after which it splits and becomes the deep and
superficial palmar arches. Blue tag, Sensory branch; black tag, motor
branch; red tag, ulnar artery. (From Waugh RP, Pellegrini Jr VD: Ulnar tunnel syndrome, Hand Clin 23:301310, 2007.)

133

134 SECTION 5 Wrist and Hand Pain Syndromes

Figure 46-2 Entrapment of the ulnar nerve: Guyons canal syndrome (ulnar tunnel syndrome). A, Ganglion cyst. Transverse T2-weighted (TR/TE,
2000/80) spin echo magnetic resonance imaging shows a ganglion cyst (arrow) adjacent to the ulnar nerve and vessels (arrowhead). B and C, Anomalous muscle. This accessory muscle (i.e., accessory abductor digiti minimi muscle) (arrows) is well shown in transverse T1-weighted (TR/TE, 550/12)
spin echo (B) and fat-suppressed fast spin echo (TR/TE, 3000/11) (C) images. Note the abnormal high signal intensity in the muscle and subjacent
Guyons canal in C. (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3527.)

Testing
Electromyography helps distinguish cervical radiculopathy, diabetic
polyneuropathy, and Pancoasts tumor from ulnar tunnel syndrome.
Plain radiographs are indicated in all patients who present with ulnar
tunnel syndrome to rule out occult bony pathological processes.
Based on the patients clinical presentation, additional tests, including
complete blood cell count, uric acid level, erythrocyte sedimentation
rate, and antinuclear antibody testing, may be indicated. Magnetic
resonance imaging (MRI) of the wrist is indicated to help confirm
the diagnosis and whether joint instability or a space-occupying
lesion is suspected (Figures 46-2 and 46-3). The injection technique
described here serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis
Ulnar tunnel syndrome often is misdiagnosed as arthritis of the
carpometacarpal joints, cervical radiculopathy, Pancoasts tumor,

and diabetic neuropathy. Patients with arthritis of the carpometacarpal joint usually have radiographic evidence and physical findings suggestive of arthritis. Most patients with a cervical
radiculopathy have reflex, motor, and sensory changes associated
with neck pain, whereas patients with ulnar tunnel syndrome have
no reflex changes, and motor and sensory changes are limited to
the distal ulnar nerve.
Diabetic polyneuropathy generally manifests as symmetrical
sensory deficit involving the entire hand, rather than limited in the
distribution of the ulnar nerve. Cervical radiculopathy and ulnar
nerve entrapment may coexist as the double crush syndrome.
Because ulnar tunnel syndrome is commonly seen in patients with
diabetes, diabetic polyneuropathy usually occurs in patients with
diabetes with ulnar tunnel syndrome. Pancoasts tumor invading
the medial cord of the brachial plexus also may mimic an isolated
ulnar nerve entrapment and should be ruled out by apical lordotic
chest radiographs.

46 Ulnar Tunnel Syndrome 135

P
S
T

C
Figure 46-3 A, Axial T2-weighted magnetic resonance imaging through the level of the proximal carpal row in a patient with symptoms of ulnar
nerve compression. A high signal intensity lesion (white arrow) adjacent to the ulnar artery and vein (broken white arrows) displaces the ulnar nerve
(curved white arrow). B, The postcontrast (obtained after administration of a contrast agent) T1-weighted image shows low SI within the lesion (white
arrow) without enhancement, and the displaced ulnar nerve is again demonstrated. The appearances are consistent with a ganglion within Guyons
canal. C, The cystic nature of the lesion is further confirmed on the transverse Doppler ultrasound image, on which the ganglion can be seen as an
anechoic mass (white arrow) with flow evident in the ulnar artery and vein (black arrows). L, Lunate; P, pisiform; S, scaphoid; T, triquetrum. (Reproduced with permission from Spratt JD, etal: The role of diagnostic radiology in compressive and entrapment neuropathies, Eur Radiol 12:23522364, 2002.)

Treatment
Initial treatment of the pain and functional disability associated
with ulnar tunnel syndrome should include a combination of
nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application
of heat and cold also may be beneficial. The repetitive movements
that incite the syndrome should be avoided. For patients who do
not respond to these treatment modalities, injection of the ulnar
nerve at the ulnar tunnel with a local anesthetic and steroid may
be a reasonable next step. If the symptoms of ulnar tunnel syndrome persist, surgical exploration and decompression of the
ulnar nerve are indicated.

Clinical Pearls
Ulnar tunnel syndrome should be differentiated from cervical radiculopathy involving the C8 spinal root, which
sometimes may mimic ulnar nerve compression. Cervical
radiculopathy and ulnar nerve entrapment may coexist in
the double crush syndrome. The double crush syndrome is
seen most commonly with ulnar nerve entrapment at the
wrist or carpal tunnel syndrome. Pancoasts tumor invading the medial cord of the brachial plexus also may mimic
isolated ulnar nerve entrapment and should be ruled out by
apical lordotic chest radiographs.

Complications and Pitfalls

SUGGESTED READINGS

The major complication associated with ulnar tunnel syndrome is


due to delayed diagnosis and treatment of the disease. This delay
can cause permanent neurological deficits resulting from prolonged untreated entrapment of the ulnar nerve. Failure of the
clinician to recognize an acute inflammatory or infectious arthritis
of the wrist may result in permanent damage to the joint and
chronic pain and functional disability.

Moghtaderi A, Ghafarpoor M: The dilemma of ulnar nerve entrapment at wrist in


carpal tunnel syndrome, Clin Neurol Neurosurg 111:151155, 2009.
Waldman SD: The ulnar tunnel. In Waldman SD, editor: Pain review, Philadelphia, 2009, Saunders, pp 104.
Waldman SD: Injection technique for ulnar tunnel syndrome. In Waldman SD,
editor: Pain review, Philadelphia, 2009, Saunders, pp 469470.
Ulnar tunnel syndrome. In Waldman SD, Campbell RSD, editors: Imaging of
pain, Philadelphia, 2011, Saunders, pp 323324.
Waugh RP, Pellegrini VD Jr: Ulnar tunnel syndrome, Hand Clin 23:301310, 2007.

Chapter 47
CHEIRALGIA PARESTHETICA

ICD-9 CODE 355.9


ICD-10 CODE G58.9
The Clinical Syndrome
Cheiralgia paresthetica is an uncommon cause of wrist and hand
pain and numbness. It also is known as handcuff neuropathy and
Wartenbergs syndrome. The onset of symptoms usually occurs
after compression of the sensory branch of the radial nerve.
Radial nerve dysfunction secondary to compression by tight
handcuffs, wristwatch bands, or casts is a common cause of cheiralgia paresthetica. Direct trauma to the nerve may result in a
similar clinical presentation. Fractures or lacerations frequently
disrupt the nerve completely, resulting in sensory deficit in the
distribution of the radial nerve. The sensory branch of the radial
nerve also may be damaged during surgical treatment of de Quervains tenosynovitis.
Cheiralgia paresthetica manifests as pain and associated paresthesias and numbness of the radial aspect of the dorsum of the
hand to the base of the thumb (Figure 47-1). Because significant
interpatient variability exists in the distribution of the sensory
branch of the radial nerve owing to overlap of the lateral antebrachial cutaneous nerve, the signs and symptoms of cheiralgia
paresthetica may vary from patient to patient.

have cheiralgia paresthetica. Plain radiographs are indicated in all


patients who present with cheiralgia paresthetica to rule out occult
bony pathological processes. Based on the patients clinical presentation, additional tests, including complete blood cell count,
uric acid level, erythrocyte sedimentation rate, and antinuclear
antibody testing, may be indicated. Magnetic resonance imaging
(MRI) of the elbow is indicated if joint instability is suspected.
Injection of the sensory branch of the radial nerve at the wrist
serves as a diagnostic and therapeutic maneuver and may be used
as an anatomical differential neural blockade to distinguish lesions
of the sensory branch of the radial nerve from lesions involving the
lateral antebrachial cutaneous nerve.

Differential Diagnosis
Cheiralgia paresthetica is often misdiagnosed as lateral antebrachial cutaneous nerve syndrome. Cheiralgia paresthetica also
should be differentiated from cervical radiculopathy involving the C6 or C7 roots, although patients with cervical radiculopathy generally present not only with pain and numbness
but also with reflex and motor changes. Cervical radiculopathy
and radial nerve entrapment may coexist as the double crush
syndrome. The double crush syndrome is seen most commonly
Superficial radial nerve

Dorsal digital nerves

Signs and Symptoms


Physical findings include tenderness over the radial nerve at the
wrist. A positive Tinels sign over the radial nerve at the distal
forearm is usually present (Figure 47-2). Decreased sensation
in the distribution of the sensory branch of the radial nerve is
often present, although, as mentioned, the overlap of the lateral
antebrachial cutaneous nerve may result in a confusing clinical
presentation. A positive wristwatch sign also may be present
(Figure 47-3). Flexion and pronation of the wrist and ulnar
deviation often cause paresthesias in the distribution of the
sensory branch of the radial nerve in patients with cheiralgia
paresthetica.

Testing
Electromyography can help identify the exact source of neurological dysfunction and clarify the differential diagnosis; this should
be the starting point of the evaluation of all patients thought to
136

Figure 47-1 Cheiralgia paresthetica manifests as pain, paresthesias, and


numbness of the radial aspect of the dorsum of the hand to the base
of the thumb.

47 Cheiralgia Paresthetica 137

Figure 47-2 A positive Tinels sign over the radial nerve at the distal
forearm is usually present in patients with cheiralgia paresthetica. (From
Waldman SD: Physical diagnosis of pain: an atlas of signs and symptoms,
Philadelphia, 2006, Saunders, p 168.)

with median nerve entrapment at the wrist or carpal tunnel syndrome. It should be remembered that radial nerve compression
has many causes and the nerve can be compressed anywhere
along its path (Table 47-1).

Treatment
The first step in the treatment of cheiralgia paresthetica is the
removal of the cause of pressure on the radial nerve. A trial of
nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors represents a reasonable next step.
For patients for whom these treatment modalities fail, injection of the sensory branch of the radial nerve at the wrist with a
local anesthetic and steroid should be considered. For persistent

Figure 47-3 Positive wristwatch sign suggests cheiralgia paresthetica.


(From Waldman SD: Physical diagnosis of pain: an atlas of signs and
symptoms, Philadelphia, 2006, Saunders, p 169.)

symptoms, surgical exploration and decompression of the nerve


are indicated.

Complications and Pitfalls


Radial nerve block at the wrist is a relatively safe block, with the
major complications being inadvertent intravascular injection and
persistent paresthesia secondary to needle trauma to the nerve.
This technique can be performed safely in the presence of anticoagulation by using a 25- or 27-gauge needle, albeit at increased
risk for hematoma, if the clinical situation dictates a favorable
risk-to-benefit ratio. These complications can be decreased if
manual pressure is applied to the area of the block immediately
after injection. Application of cold packs for 20-minute periods
after the block also decreases the amount of postprocedure pain
and bleeding the patient may experience.

138 SECTION 5 Wrist and Hand Pain Syndromes


TABLE 47-1

Causes of Compressive Radial Neuropathies


Site

Cause

High radial nerve

Trauma
Fractures: Diaphyseal, distal third of the humerus
Aneurysms
Tumors
Infection
Inflammation: Local
Anomalous muscles and arteries
Idiopathic: Nerve torsion or localized constrictions
Muscular effort: Lateral triceps
Muscular hypertrophy
Hereditary neuropathies
External compression: Casts, crutches, braces, sleeping positions, tourniquets, walkers

Radial nerve

Radial tunnel: Pain without muscular weakness


Anatomy: (1) Fibrous band, (2) vasculature leash (of Henry), (3) extensor carpi radialis brevis,
(4) arcade of Frohse, (5) distal edge of supinator
Musculature compression: Rowers, tennis players, weightlifters
Metabolic: Pseudogout (joint swelling), rheumatoid arthritis
Tumor: Synovial chondromatosis, ganglion, bicipital bursitis
Infection: Septic arthritis
External compression: Casts

Posterior interosseous nerve (PIN)

Same sites as the radial tunnel


Surgical: Arthroscopy portals
Tumor: Scapholunate ganglion, lipoma, intramuscular myxoma, ganglion
Metabolic: Pseudogout
Arteriovenous malformation, vasculitis
Trauma: Dislocated radial head
External compression: Casts, weight
Idiopathic nerve constriction

Superficial branch

Wrist ganglion
Anatomical: Fascia at brachoradialis/extensor carpi radialis brevis
External compression: Casts, watch bands
Crush injury

Modified from Markiewitz AD, Merryman J: Radial nerve compression in the upper extremity, J Am Soc Surg Hand 5:8799, 2005.

Clinical Pearls
Radial nerve block at the wrist is an effective treatment for
the symptoms of cheiralgia paresthetica. Careful neurological examination to identify preexisting neurological deficits that may later be attributed to the nerve block should
be performed in all patients before beginning radial nerve
block at the wrist when treating cheiralgia paresthetica. If
cheiralgia paresthetica is identified early, removal of the
offending pressure and radial nerve block with a local anesthetic and steroid should lead to marked improvement in
most patients.

SUGGESTED READINGS
Markiewitz AD, Merryman J: Radial nerve compression in the upper extremity,
JAm Soc Surg Hand 5:8799, 2005.
Massey EW, Pleet AB: Handcuffs and cheiralgia paresthetica, Neurology 28:1312
1313, 1978.
Smith MS: Handcuff neuropathy, Ann Emerg Med 10:668, 1981.
Waldman SD: Cheiralgia paresthetica. In Waldman SD, editor: Pain review, Philadelphia, 2009, Saunders, pp 275276.

Chapter 48
SECRETANS SYNDROME

ICD-9 CODE 782.3


ICD-10 CODE F68.1
The Clinical Syndrome
Secretans syndrome, also known as posttraumatic edema syndrome,
is caused by a peritendinous fibrosis that occurs after trauma to
the dorsum of the hand. Often, the trauma is seemingly minor,
such as hitting the back of the hand on the corner of a desk.
Initially, the swelling and tenderness may be attributed to the
trauma, but instead of improvement with time, the dorsum of the
hand becomes more indurated with the edema becoming brawny.
Without treatment, peritendinous fibrosis and an almost myxedematous hardening of the soft tissues of the dorsum of the hand
occur (Figure 48-1). Similar to the pain of Dupuytrens contracture, the pain of Secretans syndrome seems to burn itself out as
the disease progresses.

Signs and Symptoms


Brawny edema with associated loss of extensor function of the
hand after seemingly minor trauma is the sine qua non of Secretans syndrome. In contrast to reflex sympathetic dystrophy, which
can mimic Secretans syndrome, no sudomotor, vasomotor, or
trophic nail changes occur, although the skin changes can appear
identical to the skin changes of reflex sympathetic dystrophy.

Testing
Plain radiographs are indicated in all patients who present with
Secretans syndrome to rule out underlying occult bony pathological processes. Based on the patients clinical presentation, additional tests, including complete blood cell count, uric acid level,
erythrocyte sedimentation rate, and antinuclear antibody testing,
may be indicated. Magnetic resonance imaging (MRI) of the hand
will help confirm the diagnosis and is also indicated if joint instability, infection, or tumor is suspected. Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected.
Injection of the areas of fibrosis provides improvement of the pain
and disability of this disease if implemented early.

Differential Diagnosis
Coexistent arthritis, gout of the metacarpal and interphalangeal
joints, and tendinitis also may coexist with Secretans syndrome and
exacerbate the pain and disability of Secretans syndrome. Reflex
sympathetic dystrophy may manifest in a similar clinical manner,
but can be distinguished from Secretans syndrome because the pain
of reflex sympathetic dystrophy responds to sympathetic neural
blockade and the pain of Secretans syndrome does not.

Treatment

Figure 48-1 Secretans syndrome is caused by a peritendinous fibrosis


that occurs after trauma to the dorsum of the hand.

Initial treatment of the pain and functional disability associated


with Secretans syndrome should include a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2
(COX-2) inhibitors and physical therapy. Local application of
heat and cold also may be beneficial. For patients who do not
respond to these treatment modalities, an injection of a local anesthetic and steroid into the areas of the peritendinous fibrosis may
be a reasonable next step. The use of physical therapy, including
gentle range-of-motion exercises, should be introduced several
days after the patient undergoes this injection technique. Vigorous exercises should be avoided because they would exacerbate the
patients symptoms.
139

140 SECTION 5 Wrist and Hand Pain Syndromes

Complications and Pitfalls


Injection of the peritendinous fibrosis responsible for Secretans
syndrome is a relatively safe technique if the clinician is attentive
to detail. Such tendons may rupture if directly injected, and needle
position should be confirmed outside the tendon before injection
to avoid this complication. Another complication of this injection
technique is infection. This complication should be exceedingly
rare if strict aseptic technique is followed. Approximately 25%
of patients report a transient increase in pain after this injection
technique, and patients should be warned of this possibility.

Clinical Pearls
This injection technique is extremely effective in the treatment of pain and dysfunction secondary to Secretans syndrome. Coexistent arthritis, tendinitis, and gout also may
contribute to the pain and may require additional treatment
with more localized injection of a local anesthetic and depot
steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas
to be injected. Care must be taken to use sterile technique
to avoid infection and universal precautions to avoid risk
to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is placed on
the injection site immediately after injection. The use of
physical modalities, including local heat, massage, and gentle range-of-motion exercises, should be introduced several
days after the patient undergoes this injection technique.
Vigorous exercises should be avoided because they would
exacerbate the patients symptoms. Simple analgesics and
NSAIDs may be used concurrently with this injection technique.

SUGGESTED READINGS
Moretta DN, Cooley RD Jr: Secretans disease: a unique case report and literature
review, Am J Orthop 31:524527, 2002.
Reading G: Secretans syndrome: hard edema of the dorsum of the hand, Plast
Reconstr Surg 65:182187, 1980.
Whitney TM, Jones NF: Magnetic resonance imaging findings in Secretans disease, J Hand Surg 20:464466, 1995.
Winkelmann RK, Barker SM: Factitial traumatic panniculitis, J Am Acad Dermatol
13:988994, 1985.

Chapter 49
FOREIGN BODY SYNOVITIS

ICD-9 CODE 727.00


ICD-10 CODE M65.9
The Clinical Syndrome
Foreign body synovitis is an uncommon cause of joint or soft tissue pain encountered in clinical practice. Although foreign body
synovitis can occur anywhere in the body when a foreign material
is introduced into or near a joint, tendon sheath, or soft tissue
surrounding a joint, the hand is most often affected. When this
occurs, a chronic, inflammatory monarthritis or tenosynovitis
results. Plant thorns, wood splinters, glass, and sea urchin spines
are most commonly implicated.
After the initial injury, a patient with foreign body tenosynovitis may note localized pain in and around the joint. If the patient
realizes a foreign body is present, he or she may try to remove
it. If a portion of the foreign body is left behind, foreign body
synovitis can occur. After the acute injury, a period of quiescence
may occur, lasting weeks to months. After this latent period, the
patient begins to experience pain and loss of function in the area
of the retained foreign body and an inflammatory monarthritis or
tenosynovitis may result.

Signs and Symptoms


The diagnosis of foreign body synovitis is easy if the antecedent trauma is recognized. This is not usually the case, however.
A patient with foreign body synovitis presents with a localized
monarthritis or synovitis without obvious cause (Figure 49-1).
The patient also may report myalgias or flulike symptoms in
some cases. Examination of other joints fails to reveal evidence of
inflammatory arthritis, and the targeted history is negative. A high
index of suspicion in any patient with monoarthritis combined
with appropriate testing leads the clinician to a correct diagnosis.

Testing
Magnetic resonance imaging (MRI) of the affected joint often
reveals the offending foreign body. Vegetable matter such as plant
thorns, wood, and glass are not radiopaque and do not show up
on plain radiographs; failure to obtain MRI results in a missed
diagnosis (Figures 49-2 and 49-3). Sea urchin spines have a high
calcium content and may appear on plain radiographs. Based
on the patients clinical presentation, additional tests, including

complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated.
Electromyography is indicated if coexistent ulnar or carpal tunnel syndrome is suspected. Joint aspiration and synovial biopsy
may be required to make the diagnosis of foreign body synovitis.
Arthroscopy or arthrotomy may be the mechanism by which the
diagnosis is finally made.

Differential Diagnosis
The recognition of the possibility of antecedent trauma with the
introduction of a foreign body makes the diagnosis apparent. Foreign body synovitis must be distinguished from other causes of
monarthritis and synovitis. Table 49-1 lists common causes of
monarthritis. The ultimate differential diagnosis usually requires a
careful targeted history and physical examination combined with
appropriate laboratory and radiographic testing.

Treatment
Initial treatment of the pain and functional disability associated
with foreign body synovitis should include a combination of
nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. For patients who
do not respond to these treatment modalities, an injection into
the affected area with a local anesthetic and steroid may be a
reasonable next step. The use of physical therapy, including gentle range-of-motion exercises, should be introduced several days
after the patient undergoes this injection technique. Vigorous
exercises should be avoided because they would exacerbate the
patients symptoms. Surgical removal of the offending foreign
body often is the only intervention that successfully treats foreign
body synovitis.

Complications and Pitfalls


The main complication associated with foreign body synovitis
is the risk for permanent joint damage resulting from delayed
diagnosis. Injection of the area of synovitis syndrome is a safe
technique if the clinician is attentive to detail. Inflamed tendons
may rupture if directly injected, and needle position should be
confirmed outside the tendon before injection to avoid this complication. Another complication of this injection technique is
infection. This complication should be exceedingly rare if strict
aseptic technique is followed. Approximately 25% of patients
report a transient increase in pain after this injection technique,
and patients should be warned of this possibility.
141

142 SECTION 5 Wrist and Hand Pain Syndromes

Middle phalanx
Joint cavity
Thorn
Inflamed synovial
membrane
Proximal phalanx
Figure 49-1 Foreign body synovitis manifests as a monarthritis without apparent cause.

Figure 49-2 T1-weighted axial postgadolinium image. White arrow


indicates possible foreign body within area of enhancement. (From
Yewlett A, Oakley J, Makwana N, Patel HJ: Retained blackthorn causing
peroneal tendonitis: a case report, Foot Ankle Surg 15:205206, 2009.)

Figure 49-3 T2-weighted sagittal foot MRI depicting foreign body as


a longitudinal structure plantar to first metatarsal similar in appearance
to flexor hallucis brevis tendon. Other findings include synovitis with
erosions and nonspecific marrow edema of the first metatarsal head and
proximal phalanx. (From Bode KS, Haggerty CJ, Krause J: Latent foreign
body synovitis, J Foot Ankle Surg 46:291296, 2007.)

49 Foreign Body Synovitis 143


TABLE 49-1

Common Causes of Monarthritis


Gout
Traumatic arthritis
Gonococcal arthritis
Charcots joint
Other crystal arthropathies

Clinical Pearls
The diagnosis of foreign body synovitis is easy if the clinician thinks of it. By including foreign body synovitis in
the differential diagnosis of patients with monarthritis or
tenosynovitis, the diagnosis is more easily recognized. The
early use of MRI of the affected area also helps increase the
diagnostic accuracy of the clinician.

Sarcoidosis
Amyloidosis

SUGGESTED READINGS

Osteoarthritis

Bode KS, Haggerty CJ, Krause J: Latent foreign body synovitis, J Foot Ankle Surg
46:291296, 2007.
Kandel L, Friedman A, Chaimski G, etal: Foreign-body synovitis mimicking septic arthritis of the knee, Arthroscopy 17:993996, 2001.
Olenginski TP, Bush DC, Harrington TM: Plant thorn synovitis: an uncommon
cause of monoarthritis, Semin Arthritis Rheum 21:4046, 1991.
Yewlett A, Oakley J, Makwana N, Patel HJ: Retained blackthorn causing peroneal
tendonitis: a case report, Foot Ankle Surg 15:205206, 2009.

Osteonecrosis
Villonodular synovitis
Neoplasm
Foreign body synovitis

Chapter 50
GLOMUS TUMOR OF THE HAND

ICD-9 CODE 228.00


ICD-10 CODE D18.00
The Clinical Syndrome
Glomus tumor of the hand is an uncommon cause of distal finger
pain. It is the result of tumor formation of the glomus body, which is
a neuromyoarterial apparatus whose function is to regulate peripheral blood flow in the digits. Most patients with glomus tumor are
women 30 to 50 years of age. The pain is severe in intensity, lancinating, and boring. The tumor frequently involves the nail bed and
may invade the distal phalanx. Patients with glomus tumor of the
hand exhibit the classic triad of excruciating distal finger pain, cold
intolerance, and tenderness to palpation of the affected digit. Multiple glomus tumors are present in approximately 25% of patients
diagnosed with this disease. Glomus tumors also can occur in the
foot and occasionally in other parts of the body.

Signs and Symptoms


The diagnosis of glomus tumor of the hand is based primarily
on three points in the patients clinical history: (1) excruciating
pain localized to a distal digit, (2) the ability to trigger the pain
by palpating the area (Loves test), and (3) marked intolerance to
cold. The pain of glomus tumor can be reproduced by placing the
affected digit in a glass of ice water. If glomus tumor is present,
the characteristic lancinating, boring pain occurs within 30 to 60
seconds. Placing other unaffected fingers of the same hand in ice
water does not trigger the pain in the affected finger. Hildreths
test also is useful in the diagnosis of glomus tumor. It is performed
by placing a tourniquet proximal to the area of suspected tumor.
As the distal area becomes ischemic, the sharp lancinating pain
characteristic of glomus tumor will occur. Nail bed ridging is present in many patients with glomus tumor of the hand, and a small
blue or dark red spot at the base of the nail is visible in 10% to
15% of patients with the disease (Figure 50-1). The patient with
glomus tumor of the hand frequently wears a finger protector on
the affected digit and guards against hitting the digit on anything
to avoid triggering the pain.

Testing
Magnetic resonance imaging (MRI) of the affected digit often
reveals the actual glomus tumor and may reveal erosion or a
144

perforating lesion of the phalanx beneath the tumor (Figure 50-2).


The tumor appears as a very high and homogeneous signal on
T2-weighted images. The bony changes associated with glomus
tumor of the hand also may appear on plain radiographs if a careful comparison of the corresponding contralateral digit is made.
Radionuclide bone scan also may reveal localized bony destruction. The ice water test mentioned earlier helps the clinician
strengthen the diagnosis. Based on the patients clinical presentation, additional tests, including complete blood cell count, uric
acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Electromyography is indicated if
coexistent ulnar or carpal tunnel syndrome is suspected. Surgical
exploration of the affected digit and nail bed often is necessary to
confirm the diagnosis.

Differential Diagnosis
The triad of localized excruciating distal digit pain, tenderness to
palpation, and cold intolerance makes the diagnosis apparent to
an astute clinician. Glomus tumor of the hand must be distinguished from other causes of localized hand pain, including subungual melanoma and osteoid osteoma. If a history of trauma is
present, fracture, osteomyelitis, tenosynovitis, and foreign body
synovitis should be considered. If there is no history of trauma,
gout, other crystal monarthropathies, tumors, and diseases of the
nail and nail bed should be considered. Reflex sympathetic dystrophy should be distinguishable from glomus tumor of the hand
because the pain of reflex sympathetic dystrophy is less localized
and is associated with trophic skin and nail changes and vasomotor and sudomotor abnormalities. Raynauds syndrome usually
involves the entire hand, and the ice water test mentioned typically triggers pain if the unaffected finger is tested.

Figure 50-1 The classic bluish discoloration of the nail plate is seen on
the right proximal corner of the nail. (From McDermott EM, Weiss A-P C:
Glomus tumors, J Hand Surg Am 31:13971400, 2006.)

50 Glomus Tumor of the Hand 145

Treatment
The mainstay of treatment of glomus tumor is surgical removal.
Medication management is uniformly disappointing. Injection of
the affected digit in the point of maximal tenderness may provide
temporary relief of the pain of glomus tumor and blocks the positive ice water test response, further strengthening the diagnosis.

Complications and Pitfalls


A

The main complication associated with glomus tumor of the hand


involves the problems associated with delayed diagnosis, mainly
ongoing destruction of the bone and nail bed. Although usually
localized and well encapsulated, rarely, these tumors can exhibit
aggressive invasive tendencies, making complete excision of the
tumor and careful follow-up mandatory.

Clinical Pearls
The diagnosis of glomus tumor of the hand is usually
straightforward if the clinician identifies the unique nature
of its clinical presentation. Because of the rare potential for
aggressive, invasive behavior, complete excision and careful
follow-up are important.

B
Figure 50-2 Glomus tumor: MRI abnormalities. A, On a sagittal
T1-weighted (TR/TE, 350/25) spin echo magnetic resonance image, a
glomus tumor (arrows) led to subtle erosion of the dorsal surface of
the distal phalanx. Its signal intensity is identical to that of the nail bed
(arrowhead). B, After intravenous gadolinium administration, a sagittal fat-suppressed T1-weighted (TR/TE, 500/25) spin echo image shows
the glomus tumor (arrows) and nail bed (arrowhead) as regions of high
signal intensity. (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3999.)

SUGGESTED READINGS
Abou Jaoude JF, Roula Farah A, Sargi Z, Khairallah S, Fakih C: Glomus tumors:
report on eleven cases and a review of the literature, Chirurg Main 19:243252,
2000.
Constantinesco A, Arbogast S, Foucher G, etal: Detection of glomus tumor of the
finger by dedicated MRI at 0.1 T, Magn Reson Imaging 12:11311134, 1994.
Gandon F, Legaillard Ph, Brueton R, Le Viet D, Foucher G: Forty-eight glomus
tumours of the hand: retrospective study and four-year follow-up, Ann Chir
Main Memb Super 11:401405, 1992.
Gombos Z, Fogt F, Zhang PJ: Intraosseous glomus tumor of the great toe: a case
report with review of the literature, J Foot Ankle Surg 47:299301, 2008.
McDermott EM, Weiss A-P C: Glomus tumors, J Hand Surg Am 31:13971400,
2006.

Chapter 51
BOXERS KNUCKLE

ICD-9 CODE 736.1


ICD-10 CODE M20.019
The Clinical Syndrome
It is not surprising that given the tremendous forces placed on a
boxers clenched fist when throwing a punch that traumatic injury
can occur. Along with fractures of the metacarpals and phalanges, carpal boss and boxers knuckle are the most common hand
injuries seen in clinical practice. Boxers knuckle is characterized
by localized tenderness and sharp pain over metacarpophalangeal
joints with subluxation or dislocation of the longitudinal central
tendon following to rupture of the extensor hood mechanism and
associated longitudinal central tendon dysfunction (Figure 51-1).
Along with the sagittal bands, the longitudinal central tendon
serves as a shock absorber that protects the underlying articular
capsule and surfaces. When these structures are damaged by the
trauma of a punch, the central tendon subluxes or dislocates, leaving the underlying joint unprotected (Figure 51-2).

Signs and Symptoms


On physical examination, the patient with boxers knuckle will
exhibit swelling over the affected joint with a decreased range of
motion. The examiner may detect lag of extension of the affected
digit in contrast to the adjacent untraumatized fingers. The pain
associated with boxers knuckle can be reproduced by applying
pressure to the affected knuckle and by active flexion and extension.
Patients with boxers knuckle often demonstrate ulnar deviation of the central tendon (see Figure 51-1). With acute trauma
to the dorsum of the hand, ecchymosis over the affected joint or
joints may be present.

Testing
Plain radiographs are indicated in all patients with boxers knuckle
to rule out fractures and identify subchondral cysts, which are
often associated with osteochondral fracture (Figure 51-3). Based
on the patients clinical presentation, additional testing may be
warranted to rule out inflammatory arthritis, including a complete blood count, erythrocyte sedimentation rate, uric acid level,
and antinuclear antibody testing. Magnetic resonance imaging
(MRI) of the fingers and wrist is indicated if joint instability,
occult mass, occult fracture, infection, or tumor is suspected.
Radionuclide bone scanning may be useful to identify stress
fractures.

Differential Diagnosis
The tentative diagnosis of boxers knuckle is made on clinical
grounds and confirmed by radiographic testing. Arthritis, tenosynovitis, or gout of the affected digits may accompany boxers
knuckle and exacerbate the patients pain. Occult fractures
occasionally confuse the clinical presentation.

Treatment

Figure 51-1 Subluxation of the central tendon and rupture of the


extensor hood mechanism create the classic deformity associated with
boxers knuckle.

146

Initial treatment of the pain and functional disability associated


with carpal boss consists of nonsteroidal anti-inflammatory drugs
(NSAIDs), simple analgesics, or cyclooxygenase-2 (COX-2)
inhibitors. Physical modalities, including local heat and gentle
range-of-motion exercises, should be introduced to avoid loss of
function. Vigorous exercises should be avoided, because they will
exacerbate the patients symptoms. A nighttime splint to protect
the fingers may be helpful. If sleep disturbance is present, lowdose tricyclic antidepressants are indicated. Ultimately, surgical
repair is required to alleviate the patients pain and functional
disability.

51 Boxers Knuckle 147


Normal anatomy
Interosseous muscles
Central extensor tendon
Joint capsule
Sagittal band

Central
extensor tendon
Metacarpal
Torn capsule
Boxers knuckle

Injured joint
cartilage
Torn sagittal
band

Figure 51-2 Normal anatomy of


the metacarpophalangeal (MP)
joint extensor hood mechanism
and the pathoanatomy of boxers
knuckle. Despite variations in extent
and exact location, the characteristic lesion consistently comprised
rupture of the sagittal band with
subluxation or overt dislocation of
the central extensor tendon. (From
Melone CP JR, Polatsch DB, Beldner
S: Disabling hand injuries in boxing:
boxers knuckle and traumatic carpal
boss, Clin Sports Med 28:609621,
2009.)

Complications and Pitfalls


The clinician should always keep in mind that occult fracture
or tumor may mimic the clinical symptoms of boxers knuckle.
Radiographic imaging is important to avoid misdiagnosis. Given
the amount of trauma sustained with the sport of boxing, coexistent arthritis is usually present.

Clinical Pearls

Figure 51-3 Radiograph demonstrating a subchondral cyst at the head


of the index metacarpal highly suggestive of boxers knuckle associated
with osteochondral fracture (arrow). (From Polatsch DB, Beldner S: Disabling hand injuries in boxing: boxers knuckle and traumatic carpal boss,
Clin Sports Med 28:609621, 2009.)

Pain emanating from the hand is a common problem.


Boxers knuckle must be distinguished from stress fracture, arthritis, and other occult pathological conditions of
the wrist and hand. Although NSAIDs may palliate the
pain of carpal boss, patients often require surgical repair to
obtain long-lasting relief and restore functionality. Coexistent arthritis bursitis and tendinitis may contribute to the
patients pain, necessitating additional treatment with more
localized injection of local anesthetic and steroid.

148 SECTION 5 Wrist and Hand Pain Syndromes


SUGGESTED READINGS
Aronowitz AR, Leddy JP: Closed tendon injuries of the hand and wrist in athletes,
Clin Sports Med 17:449467, 1998.
Melone CP Jr, Polatsch DB, Beldner S: Disabling hand injuries in boxing: boxers
knuckle and traumatic carpal boss, Clin Sports Med 28:609621, 2009.

Posner MA, Ambrose L: Boxers knuckle: dorsal capsular rupture of the metacarpophalangeal joint of a finger, J Hand Surg Am 14:229236, 1989.
Waldman SD: Painful conditions of the wrist and hand. Physical diagnosis of pain:
an atlas of signs and symptoms, ed 2, Philadelphia, 2010, Saunders, pp 153154.

Chapter 52
TRIANGULAR FIBROCARTILAGE TEAR
SYNDROME

ICD-9 CODE 718.03


ICD-10 CODE M24.139
The Clinical Syndrome
Triangular fibrocartilage tear syndrome, also known as triangular fibrocartilage complex (TFCC) lesion, is caused by trauma
or degenerative changes to the wrist. The TFCC is a group or
complex of ligament and cartilage structures that together serve
four major functions related to the function of the human wrist,
as follows: (1) The TFCC helps suspend the distal radius and
ulnar carpus from the distal ulna; (2) the TFCC is the major ligamentous stabilizer of the distal radioulnar joint; (3) the TFCC
provides a continuous gliding surface across the entire distal
face of the radius and ulna to allow smooth flexion/extension
and translational movements of the wrist; and (4) the TFCC
acts a shock absorber for forces transmitted over the ulnocarpal
axis (Figure 52-1 and Table 52-1).
Degeneration of the TFCC begins to occur as part of the
natural aging process in the third decade. This degenerative process predisposes the TFCC to traumatic injury. Common injuries that lead to TFCC tear syndrome are listed in Table 52-2.
These injuries include falls onto a fully pronated and hyperextended wrist; waterskiing and horseback riding injuries, in
which the patient is dragged by the wrist by a tangled ski rope or
reins, causing critical distraction forces to be applied to the volar
forearm and wrist; power drill injuries, in which the drill bit
binds and the drill handle forcibly rotates the wrist rather than
the drill bit; and distal radius fractures (Figure 52-2). Fractures
of the distal radius usually affect the radial side of the TFCC,
and the clinical symptoms, as described subsequently, may be
less clear-cut.
Patients with triangular fibrocartilage tear syndrome usually give a history of trauma to the affected wrist, although older
patients may report ulnar-side wrist pain in the absence of trauma,
often attributing their symptoms to arthritis. Reports of increased
pain when stirring coffee or other activities that require rotation
of the distal radioulnar joint are common with triangular fibrocartilage tear syndrome. Some patients also may report a catching
or clicking sensation with movement of the wrist and a feeling
of weakness. Occasionally, patients note that the bones beneath
their little finger have sunken in. This finding is due to the loss of
support of the carpal bones on the ulnar side of the wrist resulting
from disruption of the TFCC.

Signs and Symptoms


Physical examination of patients with triangular fibrocartilage
tear syndrome reveals pain on rotation of the wrist with a marked
exacerbation of this pain with stress loading of the distal radioulnar joint with the wrist in pronation and supination. A clicking
sensation may be appreciated by the examiner on range of motion
and depression or sag of the carpals on the ulnar side of the unsupported wrist. Instability of the distal radioulnar joint often can be
shown by shucking or pressing ones fingers between the distal
radius and ulna. Similar instability may be shown between the
lunotriquetral interval. A positive piano key sign is often present
and can be elicited by pressing down on the ulnar styloid as if it

Triangular fibrocartilage
complex

FIGURE 52-1 Anatomy of the triangular fibrocartilage complex.

149

150 SECTION 5 Wrist and Hand Pain Syndromes


TABLE 52-1

Function of the Triangular Fibrocartilage Complex


Helps suspend distal radius and ulnar carpus from distal ulna
Acts as major ligamentous stabilizer of distal radioulnar joint
Provides continuous gliding surface across entire distal face of
radius and ulna
Allows for smooth flexion/extension and translational movements
of wrist
Acts as shock absorber for forces transmitted over ulnocarpal axis

TABLE 52-2

Common Causes of Triangular Fibrocartilage Tear


Syndrome
Falls onto fully pronated and hyperextended wrist

is indicated if coexistent ulnar or carpal tunnel syndrome is


suspected. A very gentle injection of the radioulnar joint with
small volumes of local anesthetic and steroid provides immediate improvement of the pain, but ultimately surgical repair is
required.

Differential Diagnosis
Coexistent arthritis, gout of the radioulnar joint, carpometacarpal
and interphalangeal joints, and tendinitis also may coexist with triangular fibrocartilage tear syndrome and exacerbate the patients
pain and disability. Ulnocarpal abutment syndrome, Kienbcks
disease, and extensor carpi ulnaris tendinitis also may mimic the
pain of triangular fibrocartilage tear syndrome.

Treatment

were a piano key. If the ulnar styloid readily depresses, the test is
considered positive (Figure 52-3).

Initial treatment of the pain and functional disability associated


with triangular fibrocartilage tear syndrome should include a
combination of nonsteroidal anti-inflammatory drugs (NSAIDs)
or cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may
be beneficial. For patients who do not respond to these treatment
modalities, an injection of a local anesthetic and steroid into the
radioulnar joint may be a reasonable next step. Vigorous exercises
should be avoided because they would exacerbate the patients
symptoms. Ultimately, surgical repair is the treatment of choice.

Testing

Complications and Pitfalls

Plain radiographs are indicated in all patients who present with


triangular fibrocartilage tear syndrome to rule out underlying
occult bony pathological processes. Based on the patients clinical presentation, additional tests, including complete blood cell
count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance
imaging (MRI) of the wrist is indicated in all patients suspected
to have triangular fibrocartilage tear syndrome or if other causes of
joint instability, infection, or tumor are suspected (Figures 52-4
and 52-5). Magnetic resonance arthography also will help confirm
the diagnosis of fibrocartilage tear syndrome in questionable cases,
as will arthroscopy of the wrist (Figure 52-6). Electromyography

Failure to treat significant triangular fibrocartilage tear syndrome


surgically usually results in continued pain and disability and in
some patients leads to ongoing damage to the wrist. Injection of
the radioulnar joint with local anesthetic and steroid is a safe technique if the clinician is attentive to detail, specifically using small
amounts of local anesthetic and steroid and avoiding high injection pressures, which may disrupt the complex further. Another
complication of this injection technique is infection. This complication should be exceedingly rare if strict aseptic technique is followed. Approximately 25% of patients report a transient increase
in pain after this injection technique, and patients should be
warned of this possibility.

Waterskiing and horseback riding dragging injuries causing critical


distraction forces to be applied to volar forearm and wrist
Power drill injuries in which the drill bit binds and the drill handle
forcibly rotates the wrist rather than the drill bit
Distal radius fractures
Degenerative changes

52 Triangular Fibrocartilage Tear Syndrome 151

Distraction of
radius and ulna
from metacarpals

FIGURE 52-2 Common injuries that lead to triangular fibrocartilage tear syndrome include waterskiing and horseback riding injuries, in which the
patient is dragged by the wrist by a tangled ski rope or reins, causing critical distraction forces to be applied to the volar forearm and wrist.

Protruding ulna

Torn and inflamed


triangular fibrocartilage
complex
Figure 52-3 Piano key sign is elicited by pressing down on the ulnar styloid as if it were a piano key. If the ulnar styloid readily depresses, the test is
considered positive.

152 SECTION 5 Wrist and Hand Pain Syndromes

A
B

C
Figure 52-4 Ulnar-sided triangular fibrocartilage complex (TFCC) tears. A, Coronal two-dimensional T2 gradient echo magnetic resonance imaging.
A large amount of fluid signal is seen replacing the ulnar attachment of the TFCC, extending beyond the ulnar capsule, along the extensor carpi ulnaris tendon sheath more proximally (long arrows). A tear of the scapholunate ligament also is present (short arrow). B, Coronal T2 fast spin echo image
with fat saturation reveals fluid signal and altered morphology indicating disruption of the ulnar attachment (arrow) of the TFCC. C, Coronal short tau
inversion recovery MR image. The ulnar aspect of the TFCC is torn and detached (white arrows). A fracture of the ulnar styloid tip can be seen (black
arrow). (From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2006, Saunders, p 3325.)

52 Triangular Fibrocartilage Tear Syndrome 153

C
Figure 52-5 Radial-sided triangular fibrocartilage complex (TFCC) tears. Coronal two-dimensional T2* gradient echo magnetic resonance imaging
(A) and T2 fast spin echo image with fat saturation (B) show fluid signal intensity in the radial aspect of the TFCC (arrow), which extends to the
radiocarpal and the distal radioulnar joint articular surfaces. Fluid is seen in the distal radioulnar joint. C, Coronal high-resolution T1 fast spin echo
image after intra-articular contrast injection into the radiocarpal joint in another patient illustrates high signal intensity contrast extending through
a TFCC defect into the distal radioulnar joint (arrow). (From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors: Clinical magnetic resonance imaging,
3rd ed, Philadelphia, 2006, Saunders, p 3324.)

154 SECTION 5 Wrist and Hand Pain Syndromes

Figure 52-6 A, Digital subtraction magnetic resonance arthrogram image demonstrating a leak of contrast agent from the radiocarpal joint into the
distal radioulnar joint (DRUJ) (broken black arrow) resulting from a TFC tear. B, The postinjection radiograph also shows contrast agent within the
DRUJ. In addition, contrast agent is seen in the midcarpal joint because of a leak through an asymptomatic central perforation of the scapholunate
ligament. C, The coronal gradient echo magnetic resonance arthrogram image shows the tear of the TFC (white arrow). The articular cartilage of the
wrist is well demonstrated and normal. (From In Waldman SD, Campbell RSD, editors: Imaging of pain, Philadelphia, 2011, Saunders, p 310.)

Clinical Pearls
Triangular fibrocartilage tear syndrome is a straightforward
diagnosis in the presence of obvious antecedent trauma. The
diagnosis is less obvious in the absence of trauma, however,
unless the clinician includes it in the differential diagnosis with all patients with ulnar-sided wrist pain. Coexistent
arthritis, tendinitis, and gout also may contribute to the
pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The
use of physical modalities, including local heat and cold
and immobilization of the wrist, may provide symptomatic
relief. Vigorous exercises should be avoided because they
would exacerbate the patients symptoms and may cause
further damage to the wrist. Simple analgesics and NSAIDs
may be used concurrently with this injection technique.

SUGGESTED READINGS
Buterbaugh GA, Brown TR, Horn PC: Ulnar-sided wrist pain in athletes, Clin
Sports Med 17:567583, 1998.
Coggins CA: Imaging of ulnar-sided wrist pain, Clin Sports Med 25:505526,
2006.
Kovachevich R, Elhassan BT: Arthroscopic and open repair of the TFCC,
Hand Clin 26:485494, 2010.
Sachar K: Ulnar-sided wrist pain: evaluation and treatment of triangular fibrocartilage complex tears, ulnocarpal impaction syndrome, and lunotriquetral
ligament tears, J Hand Surg Br 33:16691679, 2008.

Chapter 53
SCAPHOLUNATE LIGAMENT TEAR
SYNDROME

ICD-9 CODE 718.03


ICD-10 CODE M24.139
The Clinical Syndrome
Scapholunate ligament tear syndrome is caused by trauma or, rarely,
degenerative changes to the wrist. The scapholunate ligament serves
as a stabilizer of the scaphoids palmarward rotational force against
the opposite dorsalward rotational force of the lunate. The ligament also maintains the spacing of the scapulolunate gap, keeping
the proximal pole of the scaphoid in proper position relative to the
lunate (Figure 53-1).
Degeneration of the scapholunate ligament complex begins to
occur as part of the natural aging process in the third decade.
This degenerative process predisposes the scapholunate ligament
complex to traumatic injury. Common injuries that lead to scapholunate ligament tear include falls onto a hyperextended wrist
(Figure 53-2). If the tear is partial, the patient reports dorsoradial
wrist pain. If the tear is complete, instability of the wrist accompanies the pain. Some patients report an audible click with any ulnar
to radial deviation of the wrist.

Signs and Symptoms


Physical examination of patients with scapholunate ligament tear
reveals pain on ulnar deviation of the wrist with the pain worsened
by having the patient tightly clench the fist, which places stress on
the carpal bones. Pain is present on palpation of the anatomical
snuffbox, and a widening of the scapholunate gap may be appreciated. A clicking sensation may be appreciated by the examiner
on range of motion. A positive Watsons test also is present when
the wrist is moved from the ulnar to radial position while the
patient tightly clutches the fist (Figure 53-3). If left untreated,
degeneration of the radioscaphoid, midcarpal, and radiolunate
joints results in a deformity termed scapholunate advanced collapse,
which is also referred to as an SLAC wrist.

Testing
Plain radiographs are indicated in all patients who present with
scapholunate ligament tear syndrome to rule out underlying
occult bony pathological conditions and identify widening of
the scapholunate gap (also known as a positive Terry Thomas or
David Lettermans sign after the space between the teeth of these

celebrities), palmar flexion of the scaphoid, and dorsiflexion of


the lunate, which is termed scapholunate dissociation with dorsal
intercalary segment instability (Figure 53-4). Based on the patients
clinical presentation, additional tests, including complete blood
cell count, uric acid level, erythrocyte sedimentation rate, and
antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the wrist is indicated in all patients
thought to have scapholunate ligament tear or if other causes of
joint instability, infection, or tumor are suspected (Figure 53-5).
Electromyography is indicated if coexistent ulnar or carpal tunnel
syndrome is suspected. A very gentle injection of the radioulnar
joint with small volumes of local anesthetic and steroid provides
immediate improvement of the pain, but ultimately surgical
repair is required.

Differential Diagnosis
Coexistent arthritis and gout of the radioulnar, carpal, metacarpal,
and interphalangeal joints; dorsal wrist ganglion; de Quervains
stenosing tenosynovitis; and tendinitis may coexist with scapholunate ligament tear syndrome and exacerbate the patients pain and
disability. Kienbcks disease, avascular necrosis of the scaphoid,
and scaphoid fractures also may mimic the pain of scapholunate
ligament tear.

Treatment
Initial treatment of the pain and functional disability associated
with scapholunate ligament tear syndrome should include a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) or
cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may
be beneficial. For patients who do not respond to these treatment
modalities, an injection of a local anesthetic and steroid into
the scapholunate joint may be a reasonable next step. Vigorous
exercises should be avoided because they would exacerbate the
patients symptoms. Ultimately, surgical repair is the treatment
of choice.

Complications and Pitfalls


Failure to treat significant scapholunate ligament tear surgically
usually results in continued pain and disability and, in some
patients, leads to ongoing damage to the wrist. Injection of the
scapholunate joint with local anesthetic and steroid is a safe
technique if the clinician is attentive to detail, specifically using
small amounts of local anesthetic and steroid and avoiding high
injection pressures, which may disrupt the ligament further.
155

156 SECTION 5 Wrist and Hand Pain Syndromes

Scapholunate
ligament
complex

Figure 53-1 Anatomy of the scapholunate ligament complex.

Torn and inflamed


scapholunate
ligament complex

Figure 53-2 Common injuries that lead to scapholunate ligament tear include falls onto a hyperextended wrist.

53 Scapholunate Ligament Tear Syndrome 157

Figure 53-3 Watsons test (scaphoid displacement test) for the diagnosis of scapholunate dissociation is performed by pushing upward on
the scaphoid tuberosity while the hand is in ulnar deviation. This action
tends to cause the scaphoid to ride out of the radial fossa over the dorsal rim, at times producing a painful snap. The test might be positive
in loose-jointed individuals and should always be compared with the
contralateral side. (From Manuel J, Moran SL: The diagnosis and treatment
of scapholunate instability, Hand Clin 26:129144, 2010.)

Figure 53-4 Posteroanterior radiograph of the wrist depicting severe


scapholunate dissociation with increased scapholunate gap, also
referred to as a positive Terry Thomas sign. The scaphoid is foreshortened with respect to the longitudinal axis of the forearm. The scaphoid
tuberosity is seen in profile providing the ring sign. The lunate appears
to be trapezoidal in shape because the palmar pole is rotated under
the capitate. (From Manuel J, Moran SL: The diagnosis and treatment of
scapholunate instability, Hand Clin 26:129144, 2010.)

Figure 53-5 Intercarpal ligaments: three-dimensional Fourier transform gradient recalled magnetic resonance imaging. Normal and abnormal scapholunate interosseous ligament. A, Normal scapholunate interosseous ligament. Coronal three-dimensional Fourier transform (TR/TE, 60/11; flip
angle, 10 degrees) MRI shows the low signal intensity and linear morphology that characterize normal scapholunate (arrow) and lunotriquetral
(arrowhead) interosseous ligaments. The triangular fibrocartilage also is normal. B, Communicating defect of the scapholunate interosseous ligament.
Coronal oblique three-dimensional Fourier transform (TR/TE, 60/10; flip angle, 30 degrees) MRI shows altered morphology (arrow) of the scapholunate interosseous ligament. (From Resnick D, editor: Diagnosis of bone and Joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3039.)

158 SECTION 5 Wrist and Hand Pain Syndromes

Another complication of this injection technique is infection.


This complication should be exceedingly rare if strict aseptic
technique is followed. Approximately 25% of patients report
a transient increase in pain after this injection technique, and
patients should be warned of this possibility.

Clinical Pearls
Scapholunate ligament tear and other disorders of the
scaphoid are a straightforward diagnosis in the presence
of obvious antecedent trauma. The diagnosis is less obvious in the absence of trauma, however, unless the clinician
includes it in the differential diagnosis with all patients
reporting radial-sided wrist pain. Coexistent arthritis, tendinitis, and gout also may contribute to the pain and may
require additional treatment with more localized injection
of a local anesthetic and depot steroid. The use of physical
modalities, including local heat and cold and immobilization of the wrist, may provide symptomatic relief. Vigorous
exercises should be avoided because they would exacerbate
the patients symptoms and may cause further damage to
the wrist. Simple analgesics and NSAIDs may be used concurrently with this injection technique.

SUGGESTED READINGS
Goldberg SH, Riansuwan K, Rosenwasser MP: Arthroscopic treatment of
scapholunate ligament tears, In Slutsky DJ, Osterman AL (eds) Fractures and
Injuries of the Distal Radius and Carpus 463474, 2009.
Manuel J, Moran SL: The diagnosis and treatment of scapholunate instability,
Orthop Clin North Am 38:261277, 2007.
Manuel J, Moran SL: The diagnosis and treatment of scapholunate instability,
Hand Clin 26:129144, 2010.
OMeeghan CJ, Stuart W, Mamo V, Stanley JK, Trail IA: The natural history of
an untreated isolated scapholunate interosseus ligament injury, J Hand Surg Br
28:307310, 2003.

Chapter 54
LUNOTRIQUETRAL INSTABILITY
PAIN SYNDROME

ICD-9 CODE 18.03


ICD-10 CODE M24.139

instability pain syndrome or if other causes of joint instability,


infection, or tumor are suspected (Figure 54-3). Electromyography is indicated if coexistent ulnar or carpal tunnel is suspected. A
gentle injection of the lunotriquetral joint with small volumes of
local anesthetic and steroid provides immediate improvement of
the pain, but ultimately surgical repair is required.

The Clinical Syndrome

Differential Diagnosis

Lunotriquetral instability pain syndrome is caused by trauma or,


rarely, degenerative changes to the wrist. The lunotriquetral ligament stabilizes the wrist and helps maintain the proper spacing of
the lunotriquetral gap (Figure 54-1).
Degeneration of the lunotriquetral ligament complex begins
to occur as part of the natural aging process in the third decade.
This degenerative process predisposes the lunotriquetral ligament complex to traumatic injury. Common injuries that lead
to lunotriquetral instability pain syndrome include backward falls
onto a hyperextended wrist (Figure 54-2). If the tear is partial,
the patient reports dorsoulnar wrist pain. If the tear is complete,
instability of the wrist accompanies the pain. Some patients report
an audible click with any ulnar deviation of the wrist.

Coexistent arthritis and gout of the radioulnar, carpometacarpal,


and interphalangeal joints; dorsal wrist ganglion; and tendinitis
may coexist with lunotriquetral instability pain syndrome and
exacerbate the patients pain and disability. Kienbcks disease
and lunate fractures also may mimic the pain of lunotriquetral
instability pain syndrome, as can tear of the triangular fibrocartilage complex and ulnar impaction syndrome.

Signs and Symptoms


Physical examination of patients with lunotriquetral instability
pain syndrome reveals pain on ulnar or radial deviation of the
wrist with the pain worsened by having the patient tightly clench
the fist, which places stress on the carpal bones. Pain is felt on
palpation of the lunate and triquetrum, and a widening of the
lunotriquetral gap may be appreciated. A clicking sensation may
be appreciated by the examiner on range of motion. A positive
lunotriquetrum shear test is often present. This test is performed
by displacing the triquetrum dorsally, while displacing the lunate
palmarly. The test is considered positive if the examiner demonstrates increased excursion of the lunotriquetral joint over the
normal side.

Testing
Plain radiographs are indicated in all patients who present with
lunotriquetral instability pain syndrome to rule out underlying
occult bony pathological processes and identify widening of the
lunotriquetral gap. Based on the patients clinical presentation,
additional tests, including complete blood cell count, uric acid
level, erythrocyte sedimentation rate, and antinuclear antibody
testing, may be indicated. Magnetic resonance imaging (MRI) of
the wrist is indicated in all patients suspected to have lunotriquetral

Lunotriquetral
ligament complex

Figure 54-1 Anatomy of the lunotriquetral ligament complex.

159

160 SECTION 5 Wrist and Hand Pain Syndromes

Treatment
Initial treatment of the pain and functional disability associated
with lunotriquetral instability pain syndrome should include a
combination of nonsteroidal anti-inflammatory drugs (NSAIDs)
or cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may
be beneficial. For patients who do not respond to these treatment
modalities, an injection of a local anesthetic and steroid into the
lunotriquetral joint may be a reasonable next step. Vigorous exercises should be avoided because they would exacerbate the patients
symptoms. Ultimately, surgical repair is the treatment of choice.

Complications and Pitfalls

Torn lunotriquetral
ligament

Figure 54-2 Common injuries that lead to lunotriquetral instability


pain syndrome include backward falls onto a hyperextended wrist.

Failure to treat significant lunotriquetral instability pain syndrome


surgically usually results in continued pain and disability and in some
patients leads to ongoing damage to the wrist. Injection of the joint
with local anesthetic and steroid is a safe technique if the clinician
is attentive to detail, specifically using small amounts of local anesthetic and steroid and avoiding high injection pressures, which may
disrupt the ligament further. Another complication of this injection
technique is infection. This complication should be exceedingly
rare if strict aseptic technique is followed. Approximately 25% of
patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

54 Lunotriquetral Instability Pain Syndrome 161

Right

Figure 54-3 A, Lateral radiograph demonstrating the lunate (white arrows) with its axis (white line) tilted in a volar direction compared with the axis
of capitate and radius (broken white line); this tilting is consistent with a volar intercalated segment instability (VISI) deformity. B, A digital subtraction
arthrogram of the same patient after injection of contrast agent into the radiocarpal joint demonstrates contrast agent passing through the lunotriquetral
(LT) joint (black arrow) and into the metacarpal joint (broken black arrow), indicating a tear of the LT ligament. The coronal T1-weighted (C) and
T2-weighted with fat suppression (D) magnetic resonance arthrogram images show sclerosis, subchondral cyst formation, and marrow edema within
the lunate resulting from secondary osteoarthritis change in the LT joint. E, The gradient echo magnetic resonance image best demonstrates absence
of the LT ligament (white arrow) and loss of articular cartilage. Compare with the normal appearance of the scapholunate ligament (broken white arrow).
(From: Lunotriquetral instability pain syndrome. In Waldman SD, Campbell RSD, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 307308.)

Clinical Pearls
Lunotriquetral instability pain syndrome and other disorders of the lunate and triquetrum are a straightforward
diagnosis in the present of obvious antecedent trauma. The
diagnosis is less obvious in the absence of trauma, however,
unless the clinician included it in the differential diagnosis with all patients with ulnar-sided wrist pain. Coexistent
arthritis, tendinitis, and gout also may contribute to the
pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The
use of physical modalities, including local heat and cold
and immobilization of the wrist, may provide symptomatic
relief. Vigorous exercises should be avoided because they
would exacerbate the patients symptoms and may cause
further damage to the wrist. Simple analgesics and NSAIDs
may be used concurrently with this injection technique.

SUGGESTED READINGS
Butterfield WL, Joshi AB, Lichtman D: Lunotriquetral injuries, J Am Soc Surg
Hand 2:195203, 2002.
Goldberg SH, Strauch RE, Rosenwasser MP: Scapholunate and lunotriquetral
instability in the athlete: diagnosis and management, Oper Tech Sports Med
14:108121, 2006.
Lunotriquetral instability pain syndrome. In Waldman SD, Campbell RSD,
editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 307308.
Sachar K: Ulnar-sided wrist pain: evaluation and treatment of triangular fibrocartilage complex tears, ulnocarpal impaction syndrome, and lunotriquetral
ligament tears, J Hand Surg Am 33:16691679, 2008.

Chapter 55
KIENBCKS DISEASE

ICD-9 CODE 732.3


ICD-10 CODE M92.30
The Clinical Syndrome
Kienbcks disease, or lunatomalacia, is caused by avascular necrosis of the lunate after repeated microfractures or major fractures to
the lunate after trauma to the wrist. Repetitive microtrauma to the
wrist from repetitive compressive loading and unloading such as
use of a jackhammer and recurrent compression of the lunate by
the capitate and distal radius resulting from extreme wrist positions
also has been implicated in the evolution of this painful condition
of the wrist and forearm (Figure 55-1). A patient with Kienbcks
disease reports unilateral dorsal wrist pain over the lunate that radiates into the forearm and decreasing range of motion of the wrist.
Weakened grip strength also may be noticed. Kienbcks disease
usually affects one wrist; incidence of bilateral disease is extremely
low. The disease is most common in the second through fourth
decades of life.

the lunotriquetral joint with small volumes of local anesthetic and


steroid provides immediate improvement of the pain, but ultimately surgical repair is required.

Differential Diagnosis
Coexistent arthritis and gout of the radioulnar, carpometacarpal,
and interphalangeal joints; dorsal wrist ganglion; and tendinitis
may coexist with Kienbcks disease and exacerbate the pain and
disability of the patient. Lunate cysts, contusions, and fractures
also may mimic the pain of Kienbcks disease, as can tear of the
triangular fibrocartilage complex and ulnar impaction syndrome
(Figure 55-3).

Signs and Symptoms


Physical examination of patients with Kienbcks disease reveals
pain on ulnar or radial deviation of the wrist, with the pain
worsened by passively dorsiflexing the middle phalanx on the
affected side. Pain is felt on palpation of the lunate, and a click
or crepitus may be appreciated by the examiner when putting
the wrist through range of motion.

Testing
Plain radiographs are indicated in all patients who present with
Kienbcks disease to rule out underlying occult bony pathological
conditions and identify sclerosis and fragmentation of the lunate.
Based on the patients clinical presentation, additional tests,
including complete blood cell count, uric acid level, erythrocyte
sedimentation rate, and antinuclear antibody testing, may also
be indicated. Magnetic resonance imaging (MRI) of the wrist is
indicated in all patients suspected to have Kienbcks disease or if
other causes of joint instability, infection, or tumor are suspected
(Figure 55-2). Electromyography is indicated if coexistent ulnar
or carpal tunnel syndrome is suspected. A very gentle injection of
162

Figure 55-1 Repetitive microtrauma to the wrist from repetitive compressive loading and unloading and recurrent compression of the lunate by the
capitate and distal radius owing to extreme wrist positions have been implicated in the evolution of this painful condition of the wrist and forearm.

55 Kienbcks Disease 163

Figure 55-2 Kienbcks disease and nonunion of a scaphoid fracture: Magnetic resonance imaging (MRI). A, Conventional tomography shows cystic
changes and sclerosis in the lunate bone and an ununited fracture of the midportion of the scaphoid bone. The fracture lines are smooth with sclerotic
margins. Mild negative ulnar variance is seen. B, Coronal T1-weighted (TR/TE, 800/20) spin echo MRI reveals low signal intensity throughout the
lunate bone and in the fracture gap of the scaphoid bone. C, Coronal T2-weighted (TR/TE, 2500/60) spin echo MRI shows foci of high signal intensity
(arrowhead) in the lunate bone. Fluid of high signal intensity (arrow) is evident in a portion of the fracture gap in the scaphoid bone. (From Resnick D,
editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3044.)

Treatment

Complications and Pitfalls

Initial treatment of the pain and functional disability associated


with Kienbcks disease should include a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2
(COX-2) inhibitors and short-term immobilization of the wrist.
Local application of heat and cold also may be beneficial. For
patients who do not respond to these treatment modalities, an
injection of a local anesthetic and steroid into the lunotriquetral
joint may be a reasonable next step to provide palliation of acute
pain. Vigorous exercises should be avoided because they would
exacerbate the patients symptoms. Ultimately, surgical repair is
the treatment of choice.

Failure to treat significant Kienbcks disease surgically usually


results in continued pain and disability and in some patients leads
to ongoing damage to the wrist. Injection of the joint with local
anesthetic and steroid is a safe technique if the clinician is attentive to detail, specifically using small amounts of local anesthetic
and steroid and avoiding high injection pressures, which may
damage the joint further. Another complication of this injection
technique is infection. This complication should be exceedingly
rare if strict aseptic technique is followed. Approximately 25%
of patients report a transient increase in pain after this injection
technique, and patients should be warned of this possibility.

164 SECTION 5 Wrist and Hand Pain Syndromes

C
Figure 55-3 Kienbcks disease mimics. Posteroanterior x-ray (A) and B T1-weighted coronal MRI of a patient with stage 1 Kienbcks disease (B).
(From Beredjiklian PK: Kienbcks disease, J Hand Surg Am 34:167175, 2009.)

Clinical Pearls
Kienbcks disease and other disorders of the lunate are a
relatively straightforward diagnosis in the present of obvious antecedent trauma. The diagnosis is less obvious in the
absence of trauma, however, unless the clinician included it
in the differential diagnosis with all patients with dorsoulnar wrist pain that radiated into the forearm. Coexistent
arthritis, tendinitis, and gout also may contribute to the
pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The
use of physical modalities, including local heat and cold
and immobilization of the wrist, may provide symptomatic
relief. Vigorous exercises should be avoided because they
would exacerbate the patients symptoms and may cause
further damage to the wrist. Simple analgesics and NSAIDs
may be used concurrently with this injection technique.

SUGGESTED READINGS
Beredjiklian PK: Kienbcks disease, J Hand Surg Am 34:167175, 2009.
Innes L, Strauch RJ: Systematic review of the treatment of Kienbcks disease in its
early and late stages, J Hand Surg Am 35:713717, 2010.
Taniguchi Y, Yoshida M, Iwasaki H, Otakara H, Iwata S: Kienbcks disease in
elderly patients, J Hand Surg Am 28:779783, 2003.
Wagner JP, Chung KC: A historical report on Robert Kienbck (18711953) and
Kienbcks disease, J Hand Surg Am 30:11171121, 2005.
Yazaki N, Nakamura R, Nakao E, etal: Bilateral Kienbcks disease, J Hand Surg
Br 30:133136, 2005.

Chapter 56
AVASCULAR NECROSIS OF THE
SCAPHOID

ICD-9 CODE 733.40


ICD-10 CODE M84.1
The Clinical Syndrome
Avascular necrosis of the scaphoid is a common sequela to scaphoid fracture. Second only to the hip in the incidence of avascular necrosis, the scaphoid is extremely susceptible to this disease
because of the tenuous blood supply of the scaphoid, which enters
the bone through its distal half. The dorsal blood supply and the
volar blood supply are easily disrupted by fracture of the scaphoid,
often leaving the proximal portion of the bone without nutrition,
leading to osteonecrosis.
Common causes of scaphoid fracture include trauma to the
scaphoid from falls on a hyperextended wrist and from steering
wheel injuries during motor vehicle accidents, although an idiopathic form of the disease, known as Preisers disease, can occur
(Figure 56-1). A patient with avascular necrosis of the scaphoid
reports unilateral wrist pain over the anatomical snuffbox that
may radiate into the radial aspect of the forearm and decreasing range of motion of the wrist. Weakened grip strength also
may be noticed. Movement of the thumb usually exacerbates the
patients pain.

sedimentation rate, and antinuclear antibody testing, also may


be indicated. Computerized tomography (CT) and magnetic
resonance imaging (MRI) of the wrist are indicated in all patients
thought to have avascular necrosis of the scaphoid or if other
causes of joint instability, infection, or tumor are suspected.
Administration of gadolinium followed by postcontrast imaging
may help delineate the adequacy of blood supply, with contrast
enhancement of the proximal scaphoid being a good prognostic
sign (Figure 56-4). Ultrasound imaging of the scaphoid also may
aid in the diagnosis (Figure 56-5). Electromyography is indicated
if coexistent ulnar or carpal tunnel syndrome is suspected. A very
gentle injection of the radial aspect of the distal radioulnar joint
with small volumes of local anesthetic and steroid provides immediate improvement of the pain, but ultimately surgical repair is
required.

Differential Diagnosis
Coexistent arthritis and gout of the radioulnar, carpometacarpal,
and interphalangeal joints; dorsal wrist ganglion; and tendinitis
may coexist with avascular necrosis of the scaphoid and exacerbate the patients pain and disability. Distal fractures of the radius,

Signs and Symptoms


Physical examination of patients reports avascular necrosis of the
scaphoid reveals pain on palpation of the anatomical snuffbox
(Figure 56-2). The pain can be worsened by passively moving the
wrist from ulnar to radial position or by moving the thumb of the
affected side. A click or crepitus also may be appreciated by the
examiner when putting the wrist through range of motion. Weakness of dorsiflexion is common, as is weakness of grip strength in
contrast to the nonaffected side.

Testing
Plain radiographs are indicated in all patients who present with
avascular necrosis of the scaphoid to rule out underlying occult
bony pathological conditions and identify sclerosis and fragmentation of the scaphoid, although early in the course of the disease,
plain radiographs can be notoriously unreliable (Figure 56-3).
Based on the patients clinical presentation, additional tests,
including complete blood cell count, uric acid level, erythrocyte

Fractured
scaphoid

Figure 56-1 Common causes of scaphoid fractures include trauma to


the scaphoid from falls on a hyperextended wrist and from steering
wheel injuries during motor vehicle accidents.

165

166 SECTION 5 Wrist and Hand Pain Syndromes

de Quervains stenosis, tenosynovitis, scapholunate ligament tears,


scaphoid cysts, contusions, and fractures also may mimic the pain
of avascular necrosis of the scaphoid, as can tear of the triangular
fibrocartilage complex.

Treatment
Initial treatment of the pain and functional disability associated
with avascular necrosis of the scaphoid should include a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) or
cyclooxygenase-2 (COX-2) inhibitors and short-term immobilization of the wrist. Local application of heat and cold also may
be beneficial. For patients who do not respond to these treatment
modalities, an injection of a local anesthetic and steroid into the
radial aspect of the distal radioulnar joint may be a reasonable next
step to provide palliation of acute pain. Vigorous exercises should
be avoided because they would exacerbate the patients symptoms.
Ultimately, surgical repair is the treatment of choice.

Complications and Pitfalls


Anatomical snuff box

Figure 56-2 Physical examination of patients with avascular necrosis of


the scaphoid reveals pain to palpation of the anatomical snuffbox.

Failure to treat significant avascular necrosis of the scaphoid surgically usually results in continued pain and disability and in some
patients leads to ongoing damage to the wrist. Injection of the joint
with local anesthetic and steroid is a safe technique if the clinician
is attentive to detail, specifically using small amounts of local anesthetic and steroid and avoiding high injection pressures, which may
damage the joint further. Another complication of this injection
technique is infection. This complication should be exceedingly
rare if strict aseptic technique is followed. Approximately 25% of
patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Figure 56-3 A, Radiograph obtained 12 weeks after a scaphoid fracture. There is an apparent cyst in the scaphoid but no fracture line. B, The computed tomography scan, however, confirms fracture nonunion. (From In Waldman SD, Campbell RSD, editors: Imaging of pain, Philadelphia, 2011,
Saunders, pp 313-315.

56 Avascular Necrosis of the Scaphoid 167

Figure 56-4 Osteonecrosis of the scaphoid bone after a fracture. A, Four months after a scaphoid fracture, coronal T1-weighted (TR/TE, 500/14) spin
echo magnetic resonance imaging (MRI) reveals nonunion of the bone and low signal intensity at the fracture line and in the proximal pole of the
scaphoid. B, After intravenous gadolinium administration, fat-suppressed coronal T1-weighted (TR/TE, 550/14) spin echo MRI image shows enhancement in both portions of the scaphoid, a good prognostic sign. (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia,
2002, Saunders, p 3045.)

Figure 56-5 A split-screen side-by-side comparison of the fractured scaphoid (right) and normal scaphoid (left). The arrows identify two cortical fractures in the palmar cortex. (From Senall JA, Failla JM, Bouffard A, Holsbeeck M: Ultrasound for the early diagnosis of clinically suspected scaphoid fracture,
J Hand Surg Am 29:400405, 2004.)

168 SECTION 5 Wrist and Hand Pain Syndromes

Clinical Pearls
Avascular necrosis of the scaphoid is a diagnosis that is
often missed, leading to much unnecessary pain and disability. The clinician should include avascular necrosis of
the scaphoid in the differential diagnosis in all patients with
radial-sided wrist pain after trauma to the wrist. Coexistent
arthritis, tendinitis, and gout also may contribute to the
pain and may require additional treatment with more localized injection of a local anesthetic and depot steroid. The
use of physical modalities, including local heat and cold
and immobilization of the wrist, may provide symptomatic
relief. Vigorous exercises should be avoided because they
would exacerbate the patients symptoms and may cause
further damage to the wrist. Simple analgesics and NSAIDs
may be used concurrently with this injection technique.

SUGGESTED READINGS
Adey L, Souer JS, Lozano-Calderon S: Computed tomography of suspected
scaphoid fractures, J Hand Surg Am 32:6166, 2007.
Kawamura K, Chung KC: Treatment of scaphoid fractures and nonunions,
J Hand Surg Am 33:988997, 2008.
Senall JA, Failla JM, Bouffard A, Holsbeeck M: Ultrasound for the early diagnosis
of clinically suspected scaphoid fracture, J Hand Surg Am 29:400405, 2004.

Chapter 57
EXTENSOR CARPI ULNARIS
TENDINITIS

ICD-9 CODE 727.05


ICD-9 CODE M65.849
The Clinical Syndrome
Extensor carpi ulnaris tendinitis is being seen with increasing frequency in clinical practice as golf and racquet sports have increased
in popularity. The extensor carpi ulnaris tendon is susceptible to
the development of tendinitis at the distal portion. The extensor
carpi ulnaris tendon is subject to repetitive motion that may result
in microtrauma, which heals poorly because of the tendons avascular nature. Exercise is often implicated as the inciting factor of
acute extensor carpi ulnaris tendinitis, with improper grip of golf
clubs and tennis racquets a common inciting cause (Figure 57-1).
Tendinitis of the extensor carpi ulnaris tendon frequently coexists
with bursitis, creating additional pain and functional disability.
Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult.
Continued trauma to the inflamed tendon ultimately may result
in tendon rupture.

on the patients clinical presentation, additional tests, including


complete blood count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. MRI of the wrist is indicated if joint instability is suspected and to confirm the diagnosis
further (Figure 57-2). Ultrasound imaging is also useful in aiding
in diagnosis (Fig. 57-3). Radionuclide bone scanning is useful to
identify stress fractures of the wrist not seen on plain radiographs.

Differential Diagnosis
Extensor carpi ulnaris tendinitis is generally easily identified on clinical grounds; however, coexistent bursitis may confuse the diagnosis.
Fractures of the ulnar styloid and lunate and tears of the triangular
fibrocartilage complex, ulnocarpal abutment syndrome, and Kienbcks disease also may mimic extensor carpi ulnaris tendinitis.

Treatment
Initial treatment of the pain and functional disability associated
with extensor carpi ulnaris tendinitis should include a combination

Signs and Symptoms


The onset of extensor carpi ulnaris tendinitis is usually acute,
occurring after overuse or misuse of the wrist joint. Inciting factors include playing tennis, playing golf, and prolonged use of
a heavy hammer. Injuries ranging from partial to complete tears
of the tendon can occur when the distal tendon sustains direct
trauma while the wrist is in full radial deviation under load or
when the wrist is forced into full radial deviation while under
load. The pain of extensor carpi ulnaris tendinitis is constant,
severe, and localized in the dorsoulnar aspect of the wrist. Significant sleep disturbance is often reported. Patients with extensor
carpi ulnaris tendinitis exhibit pain with resisted radial deviation
of the wrist. A creaking or grating sensation may be palpated when
the wrist is passively deviated radially. As mentioned, the chronically inflamed extensor carpi ulnaris tendon may rupture suddenly
with stress or during vigorous injection procedures inadvertently
injected into the substance of the tendon.

Inflamed extensor
carpi ulnaris tendon

Testing
Plain radiographs and magnetic resonance imaging (MRI) are indicated for all patients who present with ulnar-sided wrist pain. Based

Figure 57-1 Improper grip of golf clubs and tennis racquets is often
implicated as the inciting cause of acute extensor carpi ulnaris tendinitis.

169

170 SECTION 5 Wrist and Hand Pain Syndromes

Figure 57-2 Tenosynovitis of the extensor carpi ulnaris tendon sheath: magnetic resonance imaging. Transaxial T1-weighted (TR/TE, 600/20) spin
echo MRI of the wrist at the level of the radiocarpal joint shows fluid of intermediate signal intensity (arrows) around the extensor carpi ulnaris tendon in the sixth extensor compartment. (From Resnick D, editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3048.)

*
Triquetrum

Ulna

Figure 57-3 Longitudinal ultrasound image of a patient with simple tenosynovitis of the extensor carpi ulnaris (ECU) tendon. There is anechoic fluid
(white arrows) within the ECU tendon sheath. The ECU tendon (asterisks) is not thickened. (From Waldman SD: Extensor carpi ulnaris tendinitis. In Waldman SD, Campbell RSD, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 329330.)

of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of
heat and cold also may be beneficial. Repetitive activities responsible
for the evolution of the tendinitis should be avoided. For patients
who do not respond to these treatment modalities, injection with
local anesthetic and steroid may be a reasonable next step.

Complications and Pitfalls


Trauma to the extensor carpi ulnaris tendon from the injection itself is an ever-present possibility. Tendons that are highly
inflamed or previously damaged are subject to rupture if they are
directly injected. This complication can be greatly decreased if the
clinician uses gentle technique and stops injecting immediately if
significant resistance to injection is encountered. Approximately
25% of patients report a transient increase in pain after this injection technique, and patients should be warned of this possibility.

Clinical Pearls
The extensor carpi ulnaris is a very strong tendon, but it
is also very susceptible to rupture. Coexistent bursitis and
arthritis also may contribute to wrist pain and may require
additional treatment with a more localized injection of local
anesthetic and methylprednisolone acetate.
Injection of the extensor carpi ulnaris tendon is a safe
procedure if careful attention is paid to the clinically relevant anatomy in the areas to be injected. The use of physical
modalities, including local heat and gentle range-of-motion
exercises, should be introduced several days after the patient
undergoes this injection technique for elbow pain. Vigorous
exercises should be avoided because they would exacerbate
the patients symptoms. Simple analgesics and NSAIDs
may be used concurrently with this injection technique.

57 Extensor Carpi Ulnaris Tendinitis 171


SUGGESTED READINGS
Allende C, Le Viet D: Extensor carpi ulnaris problems at the wrist: classification,
surgical treatment and results, J Hand Surg Br 30:265272, 2005.
Jeantroux J, Becce F, Guerini H, et al: Athletic injuries of the extensor carpi
ulnaris subsheath: MRI findings and utility of gadolinium-enhanced fatsaturated T1-weighted sequences with wrist pronation and supination, Eur
Radiol 21:160166, 2011.

Waldman SD: Extensor carpi ulnaris tendinitis. In Waldman SD, Campbell RSD,
editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 329330.
Watanabe A, Souza F, Vezeridis PS, Blazar P, Yoshioka H: Ulnar-sided wrist pain.
II. Clinical imaging and treatment, Skeletal Radiol 39:837857, 2010.

Chapter 58
FLEXOR CARPI RADIALIS TENDINITIS

ICD-9 CODE 727.05


ICD-10 CODE M65.849
The Clinical Syndrome
Flexor carpi radialis tendinitis is being seen with increasing frequency in clinical practice as golf and racquet sports have increased
in popularity. The flexor carpi radialis tendon is susceptible to the
development of tendinitis at its distal portion. The flexor carpi
radialis tendon is subject to repetitive motion that may result in
microtrauma, which heals poorly because of the tendons avascular
nature. Exercise and repetitive trauma are often implicated as inciting factors of acute flexor carpi radialis tendinitis, with improper
grip of golf clubs or tennis racquets and the prolonged use of a
heavy hammer as the common inciting causes (Figure 58-1).
Tendinitis of the flexor carpi radialis tendon frequently coexists
with bursitis, creating additional pain and functional disability.
Calcium deposition around the tendon may occur if the inflammation continues, making subsequent treatment more difficult.
Continued trauma to the inflamed tendon ultimately may result
in tendon rupture.

pain. Based on the patients clinical presentation, additional


tests, including complete blood count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated.
MRI and ultrasound imaging of the wrist are indicated if joint
instability or occult mass is suspected and to further confirm the
diagnosis (Figures 58-2 and 58-3). Radionuclide bone scanning
is useful to identify stress fractures of the wrist not seen on plain
radiographs.

Differential Diagnosis
Flexor carpi radialis tendinitis is generally easily identified on
clinical grounds; however, coexistent bursitis may confuse the
diagnosis. Fractures of the distal radius and scaphoid and tears of
the triangular fibrocartilage complex and avascular necrosis of the
scaphoid also may mimic flexor carpi radialis tendinitis.

Signs and Symptoms


The onset of flexor carpi radialis tendinitis is usually acute, occurring after overuse or misuse of the wrist joint. Inciting factors
include playing tennis, playing golf, and prolonged use of a heavy
hammer. Injuries ranging from partial to complete tears of the tendon can occur when the distal tendon sustains direct trauma while
the wrist is in full ulnar deviation under load or when the wrist is
forced into full ulnar deviation while under load. The pain of flexor
carpi radialis tendinitis is constant and severe and is localized in the
dorsoradial aspect of the wrist. Significant sleep disturbance is often
reported. Patients with flexor carpi radialis tendinitis exhibit pain
with resisted ulnar deviation of the wrist. A creaking or grating sensation may be palpated when the wrist is passively deviated radially.
As mentioned, the chronically inflamed flexor carpi radialis tendon
may rupture suddenly with stress or during vigorous injection procedures inadvertently injected into the substance of the tendon.

Testing
Plain radiographs and magnetic resonance imaging (MRI) are
indicated for all patients who present with ulnar-sided wrist
172

Inflamed
flexor carpi
radialis tendon

Figure 58-1 Exercise and repetitive trauma, such as prolonged use of


a heavy hammer, are often implicated as inciting factors of acute flexor
carpi radialis tendinitis.

58 Flexor Carpi Radialis Tendinitis 173

Figure 58-2 Tenosynovitis of the flexor carpi radialis tendon sheath: magnetic resonance imaging (MRI). Coronal T1-weighted (TR/TE, 600/14) spin
echo MRI of the volar aspect of the wrist shows the enlarged tendon sheath (arrow) containing fluid or inflammatory tissue. (From Resnick D, editor:
Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3049.)

MN

Scaphoid

FCR
tra

Scaphoid

Figure 58-3 Flexor carpi radialis (FCR) tendinopathy in a 65-year-old woman with a painful palpable lump over the ventral radial aspect of the right wrist.
The patient was referred for ultrasound examination for a suspected ventral ganglion cyst. A, Anteroposterior radiograph reveals scapholunate diastasis and advanced triscaphe arthritis (arrows). B, Transverse ultrasound image over the lump demonstrates a swollen and heterogeneous FCR tendon
(arrows) stabilized over the scaphoid tubercle by a thickened retinaculum (white arrowhead). C, Longitudinal ultrasound image shows bony spurs (hollow
arrowhead) from the ventral aspect of the scaphoid and the trapezium (tra) impinging on the undersurface of the abnormal tendon. The retinaculum
is thickened (solid arrowheads). (From Allen PL, Baxter GM, Weston MJ: Clinical ultrasound, 3rd ed, vol 2, New York, 2011, Churchill Livingstone p 1060.)

174 SECTION 5 Wrist and Hand Pain Syndromes

Treatment
Initial treatment of the pain and functional disability associated
with flexor carpi radialis tendinitis should include a combination
of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and physical therapy. Local application of heat and cold also may be beneficial. Repetitive activities
responsible for the evolution of the tendinitis should be avoided.
For patients who do not respond to these treatment modalities,
injection with local anesthetic and steroid may be a reasonable
next step.

Complications and Pitfalls


Trauma to the flexor carpi radialis tendon from the injection
itself is an ever-present possibility. Tendons that are highly
inflamed or previously damaged are subject to rupture if they are
directly injected. This complication can be greatly decreased if
the clinician uses gentle technique and stops injecting immediately if significant resistance to injection is encountered. Approximately 25% of patients report a transient increase in pain after
this injection technique, and patients should be warned of this
possibility.

Clinical Pearls
The flexor carpi radialis is a very strong tendon, yet it is also
very susceptible to rupture. Coexistent bursitis and arthritis
may contribute to wrist pain and may require additional
treatment with a more localized injection of local anesthetic
and methylprednisolone acetate.
Injection of the flexor carpi radialis tendon is a safe procedure if careful attention is paid to the clinically relevant
anatomy in the areas to be injected. The use of physical
modalities, including local heat and gentle range-of-motion
exercises, should be introduced several days after the patient
undergoes this injection technique for elbow pain. Vigorous
exercises should be avoided because they would exacerbate
the patients symptoms. Simple analgesics and NSAIDs
may be used concurrently with this injection technique.

SUGGESTED READINGS
Bishop AT, Gabel G, Carmichael SW: Flexor carpi radialis tendinitis. I. Operative
anatomy, J Bone Joint Surg Am 76:10091014, 1994.
Cowey AJ, Carmont MR, Tins B, Ford DJ: Flexor carpi radialis rupture reined in!,
Injury Extra 38:9093, 2007.
Fitton JM, Shea FW, Goldie W: Lesions of flexor carpi radialis tendon and sheath
causing pain at the wrist, J Bone Joint Surg Br 50:359363, 1968.
Gabel G, Bishop AT, Wood MB: Flexor carpi radialis tendinitis. II. Results of
operative treatment, J Bone Joint Surg Am 76:10151018, 1994.
Kosiyatrakul A, Luenam S, Prachaporn S: Symptomatic flexor carpi radialis brevis:
case report, J Hand Surg 35:633635, 2010.

Chapter 59
TRIGGER WRIST

ICD-9 CODE 727.05


ICD-10 CODE M65.849
The Clinical Syndrome
Trigger wrist is a rare cause of wrist pain and functional disability
caused by inflammation and swelling of the wrist flexor tendon
apparatus or by tumors or masses affecting the wrist flexor tendons. Commonly, the tendinopathy associated with trigger wrist
is due to compression by the carpal bones, especially the hook of
the hamate bone. Sesamoid bones in this region may also compress and cause trauma to the tendons. Trauma is usually the
result of repetitive motion or pressure on the tendon as it passes
over these bony prominences. If the inflammation and swelling
become chronic, the tendon sheath may thicken, resulting in constriction. Frequently, nodules develop on the tendon, and they
can often be palpated when the patient flexes and extends the
wrists. Such nodules may catch in the tendon sheath as they pass
under the transverse palmar ligament in a manner analogous to
the more common trigger finger phenomenon, producing a triggering that causes the wrist to catch or lock. Trigger wrist occurs
in patients engaged in repetitive activities such as playing the
drums (Figure 59-1).

Testing
Plain radiographs are indicated in all patients who present with
trigger wrist to rule out occult bony pathological processes. Based
on the patients clinical presentation, additional testing, including
a complete blood count, uric acid level, erythrocyte sedimentation
rate, and antinuclear antibody testing, may be indicated. Magnetic
resonance imaging (MRI) of the hand is indicated if joint instability, mass, tumor, or some other abnormality is suspected. The
injection technique described later serves as both a diagnostic and
therapeutic maneuver. Occasionally, surgical exploration is required
to accurately ascertain the cause of trigger wrist (Figure 59-3).

Differential Diagnosis
The diagnosis of trigger wrist is usually made on clinical grounds.
Arthritis or gout of the carpal or radioulnar joint may accompany
trigger wrist and exacerbate the patients pain. Occult fractures
occasionally confuse the clinical presentation. Trigger finger and
carpal tunnel syndrome frequently coexist with the much less
commonly occurring trigger wrist.

Treatment
Initial treatment of the pain and functional disability associated
with trigger wrist includes a combination of nonsteroidal antiinflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2)

Signs and Symptoms


The pain of trigger wrist is localized to the distal wrist and
proximal palm, and tender nodules often can be palpated. The
pain is constant and is made worse with active flexion/extension of the wrist. Patients note significant stiffness when flexing
the wrists. Sleep disturbance is common, and patients often
awaken to find that the wrist has become locked in a flexed
position.
On physical examination, tenderness and swelling are noted
over the tendon, with maximal point tenderness over the carpal
bones. Many patients with trigger wrist experience a creaking or
snapping sensation with flexion and extension of the wrists. Range
of motion of the wrists may be decreased because of pain, and a
triggering phenomenon may be noted. A catching tendon sign
may be elicited by having the patient flex the affected wrist for
30 seconds and then relax but not unflex the wrist. The examiner then passively extends the affected wrist, and if a locking,
popping, or catching of the tendon is appreciated as the wrist is
straightened, the sign is positive (Figure 59-2).

Figure 59-1 Trigger wrist is the result of repetitive microtrauma from


repeated flexion and extension of the wrist.

175

176 SECTION 5 Wrist and Hand Pain Syndromes

B
Figure 59-2 The catching tendon sign for trigger wrist. A, The patient is
asked to actively flex the affected wrist for 30 seconds. B, The examiner
passively extends the affected wrist while palpating the flexor tendons.

inhibitors and physical therapy. A nighttime splint to protect the


wrists also may help relieve the symptoms. If these treatments fail,
the following injection technique is a reasonable next step.
Injection of trigger wrist is carried out by placing the patient in
the supine position with the arm fully adducted at the patients side
and the dorsal surface of the hand resting on a folded towel. A total
of 2 mL local anesthetic and 40 mg methylprednisolone is drawn
up in a 5-mL sterile syringe. After sterile preparation of the skin
overlying the affected tendon, the carpal bone beneath the tendon
is identified. Using strict aseptic technique, at a point just proximal
to the joint, a 1-inch, 25-gauge needle is inserted at a 45-degree
angle parallel to the affected tendon through the skin and into the
subcutaneous tissue overlying the tendon, with care taken to avoid
the median nerve as it passes under the transverse carpal ligament
and radial nerve and artery. If bone is encountered, the needle
is withdrawn into the subcutaneous tissue. The contents of the
syringe is then gently injected. The tendon sheath should distend as
the injection proceeds. Little resistance to injection should be felt; if
resistance is encountered, the needle is probably in the tendon and
should be withdrawn until the injection can be accomplished without significant resistance. The needle is then removed, and a sterile
pressure dressing and ice pack are applied to the injection site.
Physical modalities, including local heat and gentle rangeof-motion exercises, should be introduced several days after the
patient undergoes injection. Vigorous exercises should be avoided,
because they will exacerbate the patients symptoms.
Surgical treatment should be considered for patients who fail
to respond to the aforementioned treatment modalities.

Complications and Pitfalls


Failure to adequately treat trigger wrist early in its course can result
in permanent pain and functional disability because of continued
trauma to the tendon and tendon sheath. The major complications
associated with injection are related to trauma to the inflamed
and previously damaged tendon. The tendon may rupture if it is
injected directly, so a needle position outside the tendon should
be confirmed before proceeding with the injection. Further more,
the radial artery and superficial branch of the radial nerve are susceptible to damage if the needle is placed too far medially. Another
complication of injection is infection, although it should be
exceedingly rare if strict aseptic technique is used, along with universal precautions to minimize any risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if
pressure is applied to the injection site immediately after injection.
Approximately 25% of patients report a transient increase in pain
after injection, and they should be warned of this possibility.

Clinical Pearls
Figure 59-3 Giant cell tumour of the flexor sheath compressing the
median nerve. (From Chalmers RL, Mandalia M, Contreras R, Schreuder F:
Acute trigger wrist and carpal tunnel syndrome due to giant-cell tumour of
the flexor sheath, J Plast Reconstr Aesthet Surg 61:1557, 2008.)

The injection technique described is extremely effective in


the treatment of pain secondary to trigger wrist. Coexistent
arthritis or gout may contribute to the patients pain, necessitating additional treatment with more localized injection
of local anesthetic and methylprednisolone. A hand splint
to protect the wrists also may help relieve the symptoms of
trigger wrist. Simple analgesics and NSAIDs can be used
concurrently with the injection technique, although surgical treatment may ultimately be required to provide permanent relief.

59 Trigger Wrist 177


SUGGESTED READINGS
Chalmers RL, Mandalia M, Contreras R, Schreuder F: Acute trigger wrist and
carpal tunnel syndrome due to giant-cell tumour of the flexor sheath, J Plast
Reconstr Aesthet Surg 61:1557, 2008.
Giannikas D, Karabasi A, Dimakopoulos P: Trigger wrist, J Hand Surg Br 32:
214216, 2007.
Pople IK: Trigger wrist due to idiopathic synovial hypertrophy, J Hand Surg Br
11:453454, 1986.
Ragheb D, Stanley A, Gentili A, Hughes T, Chung CB: MR imaging of the wrist
tendons: normal anatomy and commonly encountered pathology, Eur J Radiol
56:296306, 2005.

Sonoda H, Takasita M, Taira H, Higashi T, Tsumura H: Carpal tunnel syndrome


and trigger wrist caused by a lipoma arising from flexor tenosynovium: a case
report, J Hand Surg Am 27:10561058, 2002.
Waldman SD: Trigger finger. Atlas of pain management injection techniques, 2nd
ed, Philadelphia, 2007, Saunders, pp 244247.
Waldman SD: Painful conditions of the wrist and hand. Physical diagnosis of pain:
an atlas of signs and symptoms, 2nd ed, Philadelphia, 2010, Saunders, pp 153159.

SECTION 6 Thoracic Pain Syndromes

Chapter 60
DEVILS GRIP
ICD-9 CODE 074.1
ICD-10 CODE R07.81

in the differential diagnosis. Based on the patients clinical


presentation, additional tests, including complete blood cell
count, sedimentation rate, and throat cultures for Streptococcus
spp, may be indicated. Computed tomography (CT) scan of
the thoracic contents is indicated if occult mass or empyema is
suspected.

The Clinical Syndrome


Devils grip is an uncommon cause of chest pain. Also known as
Bornholm disease, the grip of the phantom, dry pleurisy, and Sylvests
disease, devils grip is caused by acute infection with coxsackievirus. This virus is transmitted via the fecaloral route and is highly
contagious, owing to a long period of viral shedding of 6 weeks. In
some patients, their immune system limits the infection to a mild
fever or flulike illness called summer fever. In others, a full-fledged
infection with resultant pleurodynia and cough develops.
Devils grip has a seasonal variation in occurrence, with 90%
of cases occurring in the summer and fall, with August being
the peak month. No gender predilection is seen, but the disease
occurs more commonly in young adults and occasionally in children. The pain is severe and described as sharp or pleuritic. The
pain occurs in paroxysms that can last 30 minutes.

Signs and Symptoms


Physical examination of a patient with devils grip reveals a patient
who appears acutely ill (Figure 60-1). Pallor and fever are invariably present, as is tachycardia. Patients may report of malaise, sore
throat, and arthralgia, which may confuse the clinical picture.
Examination of the chest wall reveals minimal physical findings,
although a friction rub is sometimes present. During the paroxysms of pain, the patient suffering from devils grip attempts to
splint or protect the affected area. Deep inspiration or movement
of the chest wall markedly increases the pain of devils grip.

Testing
Plain radiographs are indicated in all patients with pain thought
to be the result of infection with coxsackievirus to rule out
occult chest wall pathology, pulmonary tumors, pneumonia, or
empyema (Figure 60-2). Ventilation/perfusion studies of the
lungs are indicated if pulmonary embolism is being considered
178

Figure 60-1 Deep inspiration markedly increases the pain of devils grip.

60 Devils Grip 179

*
*

A
D

*
B

Figure 60-2 A, This patient presented with a right upper lobe pneumonia (*) and a pleural effusion (arrow) seen in. B, Chest computed tomography
shows the effusion (*) that appears to be free-flowing as it is dependent. C, An ultrasound shows multiple septations in the pleural fluid (arrows). D,
Radiograph after image-guided insertion of a small-bore chest tube and fibrinolytic therapy. The empyema is nearly resolved, with persistent pneumonia (*) causing persistent fevers. E, Minimal residual pleural thickening (arrow) seen after removal of the chest tube and completion of antibiotics.
(From Hogan MJ, Coley BD: Interventional radiology treatment of empyema and lung abscesses, Paediatr Respir Rev 9:7784, 2008.)

Differential Diagnosis
As is the case with costochondritis, costosternal joint pain, Tietzes syndrome, and rib fractures, many patients with devils
grip first seek medical attention because they believe they are
having a heart attack. If the area innervated by the subcostal
nerve is involved, patients may believe they have gallbladder
disease. Statistically, children with devils grip have abdominal
pain more often than do adults, and such pain may be attributed to appendicitis, leading to unnecessary surgery. In contradistinction to most other causes of pain involving the chest
wall, which are musculoskeletal or neuropathic, the pain of
devils grip is infectious. The constitutional symptoms associated with devils grip may lead the clinician to consider pneumonia, empyema, and occasionally pulmonary embolus as the
most likely diagnosis.

As mentioned earlier, the pain of devils grip is often mistaken for pain of cardiac or gallbladder origin and can lead to
visits to the emergency department and unnecessary cardiac
and gastrointestinal workups. If trauma has occurred, devils
grip may coexist with fractured ribs or fractures of the sternum
itself, which can be missed on plain radiographs and may require
radionucleotide bone scanning for proper identification. Tietzes
syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infection, can be confused
with devils grip.
Neuropathic pain involving the chest wall also may be confused
or coexist with costosternal syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes
zoster involving the thoracic nerves. The possibility of diseases of
the structures of the mediastinum is ever present, and such disease
sometimes can be difficult to diagnose. Pathological processes that

180 SECTION 6 Thoracic Pain Syndromes

Intercostal a.

Intercostal n.

Intercostal v.

Rib
Figure 60-3 Injection technique to relieve the pain of devils grip. a, Artery; n, nerve; v, vertebra.

inflame the pleura, such as pulmonary embolus, infection, and


tumor, also need to be considered.

Treatment
Initial treatment of devils grip should include a combination
of simple analgesics and nonsteroidal anti-inflammatory drugs
(NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these medications do not control the patients symptoms adequately, opioid
analgesics may be added during the period of acute pain. Local
application of heat and cold also may be beneficial to provide
symptomatic relief of the pain of devils grip. The use of an elastic
rib belt may help provide symptomatic relief in some patients.
For patients who do not respond to the aforementioned treatment modalities, the following injection technique using a local
anesthetic and steroid may be a reasonable next step. The patient
is placed in the prone position with the patients arms hanging
loosely off the side of the cart. Alternatively, this block can be
done with the patient in the sitting or lateral position. The rib

to be blocked is identified by palpating its path at the posterior


axillary line. The index and middle fingers are placed on the rib,
bracketing the site of needle insertion. The skin is prepared with
antiseptic solution. A 22-gauge, 112-inch needle is attached to a
12-mL syringe and is advanced perpendicular to the skin, aiming for the middle of the rib between the index and middle fingers. The needle should impinge on bone after being advanced
approximately 34 inch. After bony contact is made, the needle is
withdrawn into the subcutaneous tissues and the skin and subcutaneous tissues are retracted with the palpating fingers inferiorly;
this allows the needle to be walked off the inferior margin of the
rib. As soon as bony contact is lost, the needle is slowly advanced
approximately 2 mm deeper; this places the needle in proximity
to the costal groove, which contains the intercostal nerve and the
intercostal artery and vein (Figure 60-3). After careful aspiration
reveals no blood or air, 3 to 5 mL of 1% preservative-free lidocaine
is injected. If the pain has an inflammatory component, the local
anesthetic is combined with 80 mg of methylprednisolone and
is injected in incremental doses. Subsequent daily nerve blocks

60 Devils Grip 181

are done in a similar manner, substituting 40 mg of methylprednisolone for the initial 80-mg dose. Because of the overlapping
innervation of the chest and upper abdominal wall, the intercostal nerves above and below the nerve suspected of subserving the
painful condition need to be blocked.

Complications and Pitfalls


The major problem in the care of patients believed to have devils
grip is the failure to identify potentially serious pathological conditions of the thorax or upper abdomen. Given the proximity of
the pleural space, pneumothorax after intercostal nerve block is
a distinct possibility. The incidence of the complication is less
than 1%, but it occurs with greater frequency in patients with
chronic obstructive pulmonary disease. Because of the proximity
to the intercostal nerve and artery, the clinician should calculate
carefully the total milligram dosage of local anesthetic administered, because vascular uptake by these vessels is high. Although
uncommon, infection is an ever-present possibility, especially in
an immunocompromised patient with cancer. Early detection of
infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls
Devils grip is an uncommon cause of chest pain that is
frequently misdiagnosed. Correct diagnosis is necessary to
treat this painful condition properly and to avoid overlooking serious intrathoracic or intra-abdominal pathology.
Intercostal nerve block is a simple technique that can produce dramatic relief for patients with devils grip. As mentioned, the proximity of the intercostal nerve to the pleural
space makes careful attention to technique mandatory.

SUGGESTED READINGS
Connolly JH, ONeill HJ: Bornholm disease associated with coxsackie A9 virus
infection, Lancet 298:1035, 1971.
Cotterill JA: The devils grip, Lancet 301:13081309, 1973.
Ikeda RM, Kondracki SF, Drabkin PD, Birkhead GS, Morse DL: Pleurodynia
among football players at a high school: an outbreak associated with coxsackievirus B1, JAMA 270:22052206, 1993.
Stalkup JR, Chilukuri S: Enterovirus infections: a review of clinical presentation,
diagnosis, and treatment, Dermatol Clin 20:217223, 2002.

Chapter 61
STERNOCLAVICULAR SYNDROME

ICD-9 CODE 786.59

Pain emanating from the sternoclavicular joint often mimics the


pain of cardiac origin.

ICD-10 CODE R07.89

Signs and Symptoms

The Clinical Syndrome


With the advent of seat belts that cross the chest, sternoclavicular
syndrome is being seen with greater frequency by clinicians. The
joint is often traumatized during acceleration/deceleration injuries
and blunt trauma to the chest. With severe trauma, the joint may
sublux or dislocate in association with fractures of adjacent structures. Overuse or misuse also can result in acute inflammation of
the sternoclavicular joint, which can be debilitating. Because the
sternoclavicular joint is a true joint, it is susceptible to the development of arthritis, including osteoarthritis, rheumatoid arthritis,
ankylosing spondylitis, Reiters syndrome, infection, and psoriatic
arthritis. The joint also is subject to invasion by tumor from either
primary malignancies, including thymoma, or metastatic disease.

On physical examination, obvious physical deformity may be


present and the patient vigorously attempts to splint the joint by
keeping the shoulders stiffly in neutral position (Figures 61-1 and
61-2). Pain is reproduced with active protraction or retraction
of the shoulder and full elevation of the arm. Shrugging of the
shoulder also may reproduce the pain. The sternoclavicular joint
may be tender to palpation and feel hot and swollen if acutely
inflamed. The patient also may report a clicking sensation with
movement of the joint.

Testing
Plain radiographs are indicated in all patients who have pain
thought to emanate from the sternoclavicular joint to rule out
occult bony pathological processes, including tumor. Based on

Sternoclavicular
joint

Figure 61-1 Acute protraction or retraction of the shoulder reproduces the pain of sternoclavicular syndrome.

182

61 Sternoclavicular Syndrome 183

B
Figure 61-2 A, Anterior view shows anterior dislocation of the right
sternoclavicular joint. B, Superior view shows posterior dislocation of
the acromioclavicular joint. (From Schemitsch LA, Schemitsch EH, McKee
MD: Bipolar clavicle injury: posterior dislocation of the acromioclavicular
joint with anterior dislocation of the sternoclavicular jointa report of two
cases, J Shoulder Elbow Surg 20:e18e22, 2011.)

the patients clinical presentation, additional tests, including complete blood cell count, prostate-specific antigen level, erythrocyte
sedimentation rate, and antinuclear antibody testing, may be
indicated. Computed tomography (CT) or magnetic resonance
imaging (MRI) of the joint is indicated if joint instability, tumor,
or infection is suspected (Figure 61-3). Injection of the sternoclavicular joint with a local anesthetic, steroid, or both serves as a
diagnostic and therapeutic maneuver.

Differential Diagnosis
As mentioned earlier, the pain of sternoclavicular syndrome is
often mistaken for pain of cardiac origin and can lead to visits
to the emergency department and unnecessary cardiac workups.
If trauma has occurred, sternoclavicular syndrome may coexist
with fractured ribs or fractures of the sternum itself, which can
be missed on plain radiographs and may require radionucleotide bone scanning for proper identification. Tietzes syndrome,
which is painful enlargement of the upper costochondral cartilage

B
Figure 61-3 Three-dimensional computed tomography images show
bipolar dislocation of the clavicle. (From Schemitsch LA, Schemitsch EH,
McKee MD: Bipolar clavicle injury: posterior dislocation of the acromioclavicular joint with anterior dislocation of the sternoclavicular jointa report
of two cases, J Shoulder Elbow Surg 20:e18e22, 2011.)

associated with viral infections, can be confused with sternoclavicular syndrome.


Neuropathic pain involving the chest wall also may be confused
or coexist with sternoclavicular syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes
zoster involving the thoracic nerves. The possibility of diseases of
the structures of the mediastinum is ever present and these diseases
sometimes can be difficult to diagnose. Pathological processes that
inflame the pleura, such as pulmonary embolus, infection, and
Bornholms disease, also should be considered.

Treatment
Initial treatment of the pain and functional disability associated with
sternoclavicular syndrome should include a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2
(COX-2) inhibitors. Local application of heat and cold also may
be beneficial. The use of an elastic clavicle splint may help provide symptomatic relief and help protect the sternoclavicular joints
from additional trauma. For patients who do not respond to these
treatment modalities, injection of the sternoclavicular joint using a
local anesthetic and steroid may be a reasonable next step.

184 SECTION 6 Thoracic Pain Syndromes

Complications and Pitfalls


Because of the many pathological processes that may mimic the
pain of sternoclavicular syndrome, the clinician must be careful
to rule out underlying cardiac disease and diseases of the lung and
structures of the mediastinum. Failure to do so could lead to disastrous results. The major complication of this injection technique
is pneumothorax if the needle is placed too laterally or deeply and
invades the pleural space. Infection, although rare, can occur if
strict aseptic technique is not followed. The possibility of trauma
to the contents of the mediastinum remains an ever-present possibility. This complication can be greatly decreased if the clinician
pays close attention to accurate needle placement.

Clinical Pearls
Patients with pain emanating from the sternoclavicular
joint often attribute their pain symptoms to a heart attack.
Reassurance is required, although it should be remembered that this musculoskeletal pain syndrome and coronary artery disease can coexist. Tietzes syndrome, which
is painful enlargement of the upper costochondral cartilage
associated with viral infections, can be confused with sternoclavicular syndrome, although both respond to the injection technique described. The use of physical modalities,
including local heat and gentle range-of-motion exercises,
should be introduced several days after the patient undergoes this injection technique for sternoclavicular joint pain.
Vigorous exercises should be avoided because they would
exacerbate the patients symptoms. Simple analgesics and
NSAIDs may be used concurrently with this injection technique. Laboratory evaluation for collagen-vascular disease
is indicated in patients with sternoclavicular joint pain in
whom other joints are involved.

SUGGESTED READINGS
Bicos J, Nicholson GP: Treatment and results of sternoclavicular joint injuries,
Clin Sports Med 22:359370, 2003.
Crisostomo RA, Laskowski ER, Bond JR, Agerter DC: Septic sternoclavicular
joint: a case report, Arch Physical Med Rehabil 89:884886, 2008.
Puri V, Meyers BF, Kreisel D, etal: Sternoclavicular joint infection: a comparison
of two surgical approaches, Ann Thorac Surg 91:257261, 2011.
Schemitsch LA, Schemitsch EH, McKee MD: Bipolar clavicle injury: posterior
dislocation of the acromioclavicular joint with anterior dislocation of the sternoclavicular jointa report of two cases, J Shoulder Elbow Surg 20:e18e22, 2011.

Chapter 62
POSTMASTECTOMY PAIN

ICD-9 CODE 611.71


ICD-10 CODE N64.4
The Clinical Syndrome
Postmastectomy pain syndrome is a constellation of symptoms that
include pain in the anterior chest, breast, axilla, and medial upper
extremity after surgical procedures on the breast. Postmastectomy
pain is a misnomer because the clinical syndrome includes the pain
mentioned here even if the patient has only a lumpectomy or if
another, less extensive surgical procedure is performed on the breast.
The pain is often described as constricting, with a continuing dull
ache. In addition to these symptoms, many patients with postmastectomy pain syndrome also report sudden paresthesia radiating into
the breast, axilla, or both. In some patients, a burning, allodynic pain
reminiscent of reflex sympathetic dystrophy may be the principal
manifestation. The intensity of postmastectomy pain is moderate
to severe. The onset of postmastectomy pain may be immediately
after surgery and initially be confused with expected postsurgical
pain, or the onset may be more insidious, occurring gradually 2 to 6
weeks after the inciting surgical procedure. If complete mastectomy
is performed, phantom breast pain may confound the diagnosis further, as may associated lymphedema. Sleep disturbance is a common
finding in patients with postmastectomy pain.

The clinician should always be alert to the possibility of


metastatic disease or direct extension of tumor into the chest
wall, which may mimic the pain of postmastectomy syndrome.
The findings of the targeted history and physical examination
assist the clinician in making an assessment of the sympathetic,
neuropathic, and musculoskeletal components of the pain and
designing a rational treatment plan.

Testing
Plain radiographs are indicated in all patients who present with
pain thought to be due to postmastectomy syndrome to rule out
occult bony pathology, including tumor. Electromyography helps
rule out damage to the intercostobrachial nerve or plexopathy
that may be contributing to the patients pain. Radionucleotide

Signs and Symptoms


Evaluation of a patient with postmastectomy syndrome requires
that the clinician take a careful history designed to delineate the
various components that make up the patients pain to help guide
the physical examination. The clinician should question the
patient specifically about the presence of phantom breast pain,
which may be quite distressing to the patient when superimposed
on the pain of postmastectomy syndrome.
Typical physical findings in patients with postmastectomy syndrome include areas of decreased sensation, hyperpathia, and dysesthesia in the distribution of the intercostobrachial nerve, which is
a branch of the second intercostal nerve (Figure 62-1). This nerve is
frequently damaged during breast surgery (Figure 62-2). Allodynia
outside the distribution of the intercostobrachial nerve also is often
present. Movement of the arm and axilla often exacerbates the
pain, which leads to splinting and disuse of the affected shoulder
and upper extremity. This disuse often worsens any lymphedema
that is present. If disuse of the upper extremity continues, frozen
shoulder may develop, further complicating the clinical picture.

Figure 62-1 The pain of postmastectomy syndrome is due to damage


of the intercostobrachial nerve.

185

186 SECTION 6 Thoracic Pain Syndromes

Breast

ICBN
LTV

AV

Fibrofatty
tissue

Figure 62-2 Relationship of the intercostobrachial nerve (ICNB), the


lateral thoracic vein (LTV), the axillary vein, and the breast within the
left axilla during mastectomy. (From Ivanovic N, Granic M, Randjelovic
T, Todorovic S: Fragmentation of axillary fibrofatty tissue during dissection
facilitates preservation of the intercostobrachial nerve and the lateral thoracic vein, Breast 17:293295, 2008.)

bone scanning may be useful to rule out occult pathological fractures of the ribs, sternum, or both. Based on the patients clinical presentation, additional tests, including complete blood cell
count, prostate-specific antigen level, erythrocyte sedimentation
rate, and antinuclear antibody testing, may be indicated. Computed tomography (CT) scan of the thoracic contents is indicated
if occult mass is suspected. Magnetic resonance imaging (MRI)
of the brachial plexus also should be considered if plexopathy
secondary to tumor involvement is a consideration.

Differential Diagnosis
As mentioned earlier, the pain of postmastectomy syndrome is
often mistaken for postoperative pain. If the breast surgery was
performed for malignancy, a careful search for metastatic disease
or tumor invasion of the chest wall is mandatory. Postmastectomy
syndrome may coexist with pathological rib fractures or pathological fractures of the sternum itself, which can be missed on
plain radiographs and may require radionucleotide bone scanning
for proper identification.
Neuropathic pain involving the chest wall also may be confused
or coexist with postmastectomy syndrome. Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes
zoster involving the thoracic nerves. The possibility of diseases of
the structures of the mediastinum is ever present, and these diseases
sometimes can be difficult to diagnose. Pathological processes that
inflame the pleura, such as pulmonary embolus, infection, and
Bornholms disease, also may mimic the pain of postmastectomy
syndrome.

Treatment
Initial treatment of postmastectomy syndrome should include a combination of simple analgesics and nonsteroidal anti-inflammatory

drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these


medications do not control the patients symptoms adequately, a
tricyclic antidepressant or gabapentin should be added.
Traditionally, tricyclic antidepressants have been a mainstay in the palliation of pain secondary to postmastectomy
syndrome. Controlled studies have shown the efficacy of amitriptyline for this indication. Other tricyclic antidepressants,
including nortriptyline and desipramine, also have been shown
to be clinically useful. This class of drugs is associated with
significant anticholinergic side effects, however, including dry
mouth, constipation, sedation, and urinary retention. These
drugs should be used with caution in patients with glaucoma,
cardiac arrhythmia, and prostatism. To minimize side effects
and encourage compliance, the primary care physician should
start amitriptyline or nortriptyline at a 10-mg dose at bedtime.
The dose can be titrated to 25 mg at bedtime as side effects
allow. Upward titration of dosage in 25-mg increments can
be done each week as side effects allow. Even at lower doses,
patients generally report a rapid improvement in sleep disturbance and begin to experience pain relief in 10 to 14 days. If
the patient does not experience any improvement in pain as the
dose is being titrated upward, the addition of gabapentin alone
or in combination with nerve blocks of the intercostal nerves
with local anesthetics, steroid, or both is recommended. Selective serotonin reuptake inhibitors, such as fluoxetine, also have
been used to treat the pain of diabetic neuropathy, and although
better tolerated than tricyclic antidepressants, they seem to be
less efficacious.
If the antidepressant compounds are ineffective or contraindicated, gabapentin represents a reasonable alternative. Gabapentin
should be started with a 300-mg dose at bedtime for 2 nights. The
patient should be cautioned about potential side effects, including
dizziness, sedation, confusion, and rash. The drug is increased in
300-mg increments, given in equally divided doses over 2 days,
as side effects allow, until pain relief is obtained or a total dose of
2400 mg daily is reached. At this point, if the patient has experienced partial pain relief, blood values are measured and the drug
is carefully titrated upward using 100-mg tablets. Rarely is more
than 3600 mg daily required.
Local application of heat and cold, as well as topical capsaicin,
also may be beneficial to provide symptomatic relief of the pain
of postmastectomy syndrome. The use of an elastic rib belt may
help provide symptomatic relief. For patients who do not respond
to these treatment modalities, injection of the affected intercostal
nerves or thoracic epidural nerve block using local anesthetic and
steroid may be a reasonable next step.

Complications and Pitfalls


The major problem in the care of patients thought to have postmastectomy syndrome is the failure to identify potentially serious
pathological conditions of the thorax or upper abdomen secondary to metastatic disease or invasion of the chest wall and thorax
by tumor. Given the proximity of the pleural space, pneumothorax after intercostal nerve block is a distinct possibility. The
incidence of the complication is less than 1%, but it occurs with
greater frequency in patients with chronic obstructive pulmonary
disease. Although uncommon, infection is an ever-present possibility, especially in an immunocompromised patient with cancer.
Early detection of infection is crucial to avoid potentially lifethreatening sequelae.

62 Postmastectomy Pain 187

Clinical Pearls
Postmastectomy syndrome is a cause of chest wall and thoracic pain that should not be overlooked in patients after
breast surgery. Correct diagnosis is necessary to treat this
painful condition properly and to avoid overlooking serious intrathoracic or intra-abdominal pathological processes.
The use of the pharmacological agents mentioned, including gabapentin, allows the clinician to control the pain of
postmastectomy syndrome adequately. Intercostal nerve
block is a simple technique that can produce dramatic relief
for patients with postmastectomy syndrome. As mentioned,
the proximity of the intercostal nerve to the pleural space
makes careful attention to technique mandatory.

SUGGESTED READINGS
Bjrkman B, Arnr S, Hydn L-C: Phantom breast and other syndromes after
mastectomy: eight breast cancer patients describe their experiences over time a
2-year follow-up study, J Pain 9:10181025, 2008.
Chang SH, Mehta V, Langford RM: Acute and chronic pain following breast
surgery, Acute Pain 11:114, 2009.
Katz J, Poleshuck EL, Andrus CH, etal: Risk factors for acute pain and its persistence following breast cancer surgery, Pain 119:1625, 2005.
Watson CP, Evans RJ, Watt VR: The post-mastectomy pain syndrome and the
effect of topical capsaicin, Pain 38:177186, 1989.

Chapter 63
STERNALIS SYNDROME

ICD-9 CODE 786.52


ICD-10 CODE R07.1
The Clinical Syndrome
Chest wall pain syndromes are commonly encountered in clinical practice. Some occur with relatively greater frequency and are
more readily identified by the clinician, such as costochondritis
and Tietzes syndrome. Others occur so infrequently that they are
often misdiagnosed, resulting in suboptimal outcome. Sternalis
syndrome is one such infrequent cause of anterior chest wall pain.
Sternalis is a constellation of symptoms affecting the midline
anterior chest wall that can radiate to the retrosternal area and the
medial aspect of the arm. Sternalis syndrome can mimic the pain
of myocardial infarction and is frequently misdiagnosed as such.
Sternalis syndrome is a myofascial pain syndrome and is characterized by trigger points in the midsternal area. In contrast to
costosternal syndrome, which also manifests as midsternal pain,
the pain of sternalis syndrome is not exacerbated by movement
of the chest wall and shoulder. The intensity of the pain associated with sternalis syndrome is mild to moderate and described as
having a deep, aching character. The pain of sternalis syndrome
is intermittent.

and antinuclear antibody testing, may be indicated. Computed


tomography (CT) and magnetic resonance imaging (MRI) of
the chest are indicated if a retrosternal mass, such as thymoma,
is suspected, as well as to help confirm the presence of a sternalis
muscle or other anterior chest wall mass (Figures 63-2 and 63-3).
Electromyography is indicated in patients with sternalis syndrome
to help rule out cervical radiculopathy or plexopathy that may
be considered because of the referred arm pain. Injection of the
sternalis muscle with a local anesthetic and steroid serves as a
diagnostic and therapeutic maneuver.

Differential Diagnosis
As mentioned earlier, the pain of sternalis syndrome is often
mistaken for pain of cardiac origin and can lead to visits to the
emergency department and unnecessary cardiac workups. If

Myofascial
trigger points

Signs and Symptoms


On physical examination, a patient with sternalis syndrome
exhibits myofascial trigger points at the midline over the sternum (Figure 63-1). Occasionally, a coexistent trigger point
is located in the pectoralis muscle or sternal head of the sternocleidomastoid muscle. Pain is reproduced with palpation of
these trigger points, rather than movement of the chest wall and
shoulders. A positive jump sign is present when these trigger
points are stimulated. Trigger points at the lateral border of the
scapula also may be present and amenable to injection therapy.
As mentioned, movement of the shoulders and chest wall does
not exacerbate the pain.

Testing
Plain radiographs are indicated in all patients thought to have
sternalis syndrome to rule out occult bony pathological processes,
including metastatic lesions. Based on the patients clinical presentation, additional tests, including complete blood cell count,
prostate-specific antigen level, erythrocyte sedimentation rate,
188

Figure 63-1 Patients with sternalis syndrome exhibit myofascial trigger


points at the midline over the sternum.

63 Sternalis Syndrome 189

Figure 63-2 Left sternalis muscle is incidentally discovered anterior to


the left pectoralis major muscle in this young adult man. (From Alpert JB,
Naidich DP: Imaging of incidental findings on thoracic computed tomography, Radiol Clin North Am 49:267289, 2011.)

trauma has occurred, sternalis syndrome may coexist with fractured ribs or fractures of the sternum itself, which can be missed
on plain radiographs and may require radionucleotide bone scanning for proper identification. Tietzes syndrome, which is painful
enlargement of the upper costochondral cartilage associated with
viral infections, can be confused with sternalis syndrome, as can
costosternal syndrome.
Neuropathic pain involving the chest wall also may be confused or coexist with costosternal syndrome. Examples of such
neuropathic pain include diabetic polyneuropathies and acute
herpes zoster involving the thoracic nerves. The possibility of
diseases of the structures of the mediastinum is ever present, and
these diseases sometimes can be difficult to diagnose. Pathological
processes that inflame the pleura, such as pulmonary embolus,
infection, and tumor, also should be considered.

Treatment
Initial treatment of sternalis syndrome should include a combination of simple analgesics and nonsteroidal anti-inflammatory
drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. Local
application of heat and cold also may be beneficial to provide
symptomatic relief of the pain of sternalis syndrome. The use of
an elastic rib belt may help provide symptomatic relief in some
patients. For patients who do not respond to these treatment
modalities, injection of the trigger areas located in the sternalis
muscle using a local anesthetic and steroid may be a reasonable
next step.

Figure 63-3 Well-encapsulated, fat density lesion associated with the


left serratus anterior muscle is consistent with chest wall lipoma, the
most common benign chest wall neoplasm. (From Alpert JB, Naidich DP:
Imaging of incidental findings on thoracic computed tomography, Radiol
Clin North Am 49:267289, 2011.)

Clinical Pearls
Patients with sternalis syndrome often present to the emergency department fearing they are having a heart attack. The
syndrome also is misdiagnosed frequently as cervical radiculopathy secondary to the referred arm pain. Electromyography
helps delineate the cause and extent of neural compromise.
The injection technique is extremely effective in the treatment of sternalis syndrome. Coexistent costosternal or manubriosternal arthritis also may contribute to anterior chest wall
pain and may require additional treatment with a more localized injection of a local anesthetic and depot steroid. This
technique is a safe procedure if careful attention is paid to the
clinically relevant anatomy in the areas to be injected. Pneumothorax can be avoided if shorter needles are used, and the
needle is not advanced too deeply. Care must be taken to
use sterile technique to avoid infection and universal precautions to avoid risk to the operator. The incidence of ecchymosis and hematoma formation can be decreased if pressure is
placed on the injection site immediately after injection. The
use of physical modalities, including local heat and gentle
range-of-motion exercises, should be introduced several days
after the patient undergoes this injection technique for shoulder pain. Vigorous exercises should be avoided because they
would exacerbate symptoms. Simple analgesics and NSAIDs
may be used concurrently with this injection technique.

Complications and Pitfalls


The major problem in the care of patients thought to have sternalis syndrome is the failure to identify potentially serious pathological conditions of the thorax, mediastinum, or both. Given the
proximity of the pleural space, pneumothorax after injection of
the sternalis muscle is a possibility, as is injury to the mediastinal and intrathoracic structures. Approximately 25% of patients
report a transient increase in pain after this injection technique
and should be warned about this.

SUGGESTED READINGS
Alpert JB, Naidich DP: Imaging of incidental findings on thoracic computed
tomography, Radiol Clin North Am 49:267289, 2011.
Baldry P: The chest wall. In Baldry P, editor: Myofascial pain fibromyalgia
syndromes, London, 2001, Churchill Livingstone, pp 303327.
Bennett R: Myofascial pain syndromes and their evaluation, Best Pract Res Clin
Rheumatol 21:427445, 2007.
Baldry PE, Thompson JW, editors: Acupuncture, trigger points and musculoskeletal
pain, ed 3, London, 2005, Churchill Livingstone, pp 165185.

Chapter 64
MANUBRIOSTERNAL JOINT PAIN

ICD-9 CODE 786.52


ICD-10 CODE R07.1
The Clinical Syndrome
The manubriosternal joint can serve as a source of pain that
often may mimic pain of cardiac origin. The manubriosternal
joint is susceptible to the development of arthritis, including
osteoarthritis, rheumatoid arthritis, ankylosing spondylitis, Reiters syndrome, and psoriatic arthritis (Figure 64-1). The joint is
often traumatized during acceleration/deceleration injuries and
blunt trauma to the chest. With severe trauma, the joint may
sublux or dislocate (Figure 64-2). Overuse or misuse can result
in acute inflammation of the manubriosternal joint, which can
be quite debilitating. The joint is subject to invasion by tumor

from primary malignancies, including thymoma, metastatic disease, and infection.

Signs and Symptoms


On physical examination, the physical deformity of joint subluxation after traumatic injury may be obvious on inspection
(Figure 64-3). The patient vigorously attempts to splint the
joint by keeping the shoulders stiffly in neutral position. Pain is
reproduced by active protraction or retraction of the shoulder,
deep inspiration, and full elevation of the arm. Shrugging of the
shoulder also may reproduce the pain. The manubriosternal joint
may be tender to palpation and feel hot and swollen if acutely
inflamed. The patient may report a clicking sensation with
movement of the joint.

Testing
Plain radiographs are indicated for all patients who present with
pain thought to be emanating from the manubriosternal joint
to rule out occult bony pathological processes, including tumor.
Based on the patients clinical presentation, additional testing may
be indicated, including complete blood count, prostate-specific
antigen level, erythrocyte sedimentation rate, and antinuclear
antibody testing. Computed tomography (CT) or magnetic resonance imaging (MRI) of the joint is indicated if infection, tumor,
or joint instability is suspected (Figure 64-4). Injection of the
manubriosternal joint with local anesthetic and steroid serves as a
diagnostic maneuver and a therapeutic maneuver.

Differential Diagnosis

Figure 64-1 Abnormalities of the manubriosternal joint. Radiographic


abnormalities of the manubriosternal joint are illustrated in this coronal section of the sternum. They include osseous erosions and sclerosis.
Note the irregularity of the costosternal joints (arrow). (From Resnick D,
editor: Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002,
Saunders, p 924.)

190

As mentioned earlier, manubriosternal joint pain is often mistaken for cardiac pain. A careful search for metastatic disease or
tumor invasion of the chest wall is mandatory in all patients with
manubriosternal joint pain because this pain may coexist with
pathological rib fractures or pathological fractures of the sternum
itself, which can be missed on plain radiographs and may require
radionucleotide bone scanning for proper identification.
Neuropathic pain involving the chest wall and sternum also
may be confused or coexist with manubriosternal joint pain.
Examples of such neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the thoracic nerves. The
possibility of diseases of the structures of the mediastinum is ever
present, and these diseases sometimes can be difficult to diagnose.
Pathological processes that inflame the pleura, such as pulmonary
embolus, infection, and Bornholms disease, also may mimic the
pain emanating from the manubriosternal joint.

64 Manubriosternal Joint Pain 191

Dislocated
manubriosternal
joint

Figure 64-2 With severe trauma, the manubriosternal joint may sublux or dislocate.

Figure 64-3 Obvious step in manubriosternal joint. (From Lyons I, Saha


S, Arulampalam T: Manubriosternal joint dislocation: an unusual risk of
trampolining, J Emerg Med 39:596598, 2010.)

Figure 64-4 Lateral chest CT scan showing posterior dislocation of the


sternum. (From Lyons I, Saha S, Arulampalam T: Manubriosternal joint dislocation: an unusual risk of trampolining, J Emerg Med 39:596598, 2010.)

Treatment
Initial treatment of manubriosternal joint pain should include a
combination of simple analgesics and nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these medications do not control the patients symptoms
adequately, or if considerable sleep disturbance exists, a tricyclic
antidepressant should be added.
Traditionally, tricyclic antidepressants have been a mainstay
in the palliation of sleep disturbance associated with painful

conditions. Controlled studies have shown the efficacy of amitriptyline for this indication. Other tricyclic antidepressants,
including nortriptyline and desipramine, also have been shown
to be clinically useful. This class of drugs is associated with
considerable anticholinergic side effects, including dry mouth,
constipation, sedation, and urinary retention. These drugs
should be used with caution in patients with glaucoma, cardiac arrhythmia, and prostatism. To minimize side effects and

192 SECTION 6 Thoracic Pain Syndromes

encourage compliance, the primary care physician should start


amitriptyline or nortriptyline at a 10-mg dose at bedtime. The
dose can be titrated upward to 25 mg at bedtime as side effects
allow. Upward titration of dosage in 25-mg increments can
be done each week as side effects allow. Even at lower doses,
patients generally report a rapid improvement in sleep disturbance and begin to experience pain relief in 10 to 14 days.
Selective serotonin reuptake inhibitors, such as fluoxetine, also
have been used to treat the pain of diabetic neuropathy, and
although better tolerated than tricyclic antidepressants, they
seem to be less efficacious than the tricyclic antidepressants.
Local application of heat and cold may be beneficial to provide symptomatic relief of the pain of manubriosternal joint pain.
The use of an elastic rib belt may help provide symptomatic relief.
For patients who do not respond to these treatment modalities,
injection of the manubriosternal joint using local anesthetic and
steroid may be a reasonable next step.

Complications and Pitfalls


The major problem in the care of patients thought to have manubriosternal pain is the failure to identify potentially serious pathology of the thorax or upper abdomen secondary to metastatic
disease or invasion of the chest wall and thorax by tumor. Given
the proximity of the pleural space, pneumothorax after injection
of the manubriosternal joint is a possibility. The incidence of the
complication is less than 1%, but it occurs with greater frequency
in patients with chronic obstructive pulmonary disease. Although
uncommon, infection is an ever-present possibility, especially in
an immunocompromised patient with cancer. Early detection of
infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls
Patients with pain emanating from the manubriosternal
joint often attribute their pain symptoms to a heart attack.
Reassurance is required, although it should be remembered
that this musculoskeletal pain syndrome and coronary
artery disease can coexist. Care must be taken to use sterile
technique to avoid infection and universal precautions to
avoid risk to the operator. The incidence of ecchymosis and
hematoma formation can be decreased if pressure is placed
on the injection site immediately after injection. The use of
physical modalities, including local heat and gentle rangeof-motion exercises, should be introduced several days after
the patient undergoes this injection technique for manubriosternal joint pain. Vigorous exercise should be avoided
because it exacerbates the symptoms. Simple analgesics and
NSAIDs may be used concurrently with this injection technique. Laboratory evaluation for collagen-vascular disease is
indicated for patients who have manubriosternal joint pain
with other joints involved.
SUGGESTED READINGS
Al-Dahiri A, Pallister I: Arthrodesis for osteoarthritis of the manubriosternal joint,
Eur J Cardiothorac Surg 29:119121, 2006.
Ellis H: The superior mediastinum, Anaesth Intens Care Med 10:360361, 2009.
Lyons I, Saha S, Arulampalam T: Manubriosternal joint dislocation: an unusual
risk of trampolining, J Emerg Med 39:596598, 2010.
Stochkendahl MJ, Christensen HW: Chest pain in focal musculoskeletal disorders,
Med Clin North Am 94:259273, 2010.
Waldman SD: Manubriosternal joint syndrome. In Waldman SD, editor: Pain
review, Philadelphia, 2009, Saunders, pp 247248.

Chapter 65
XIPHODYNIA

ICD-9 CODE 733.90


ICD-10 CODE M94.9

(MRI) of the joint is indicated if joint instability or an occult mass


is suspected (Figure 65-3). The following injection technique
serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis
The Clinical Syndrome
An uncommon cause of anterior chest wall pain, xiphodynia is
often misdiagnosed as pain of cardiac or upper abdominal origin.
Xiphodynia syndrome is a constellation of symptoms consisting
of severe intermittent anterior chest wall pain in the region of
the xiphoid process that worsens with overeating, stooping, and
bending. The patient may report a nauseated feeling associated
with the pain of xiphodynia syndrome. This xiphisternal joint
seems to serve as the nidus of pain for xiphodynia syndrome.
The xiphisternal joint is often traumatized during acceleration/
deceleration injuries and blunt trauma to the chest. With severe
trauma, the joint may sublux or dislocate. The xiphisternal joint
also is susceptible to the development of arthritis, including osteoarthritis, rheumatoid arthritis, ankylosing spondylitis, Reiters
syndrome, and psoriatic arthritis. The joint is subject to invasion
by tumor from either primary malignancies, including thymoma,
or metastatic disease.

As with costochondritis, costosternal joint pain, devils grip, Tietzes syndrome, and rib fractures, many patients with xiphodynia
first seek medical attention because they believe they are having a
heart attack. Patients also may believe they have ulcer or gallbladder disease. In contrast to most other causes of pain involving
the chest wall that are musculoskeletal or neuropathic in origin,
the pain of devils grip results from infection. The constitutional
symptoms associated with devils grip may lead the clinician to

Xiphisternal joint

Signs and Symptoms


Physical examination reveals that the pain of xiphodynia syndrome
is reproduced with palpation or traction on the xiphoid. The xiphisternal joint may feel swollen (Figure 65-1). Stooping or bending
may reproduce the pain. Coughing may be difficult, and this may
lead to inadequate pulmonary toilet in patients who have sustained trauma to the anterior chest wall. The xiphisternal joint and
adjacent intercostal muscles also may be tender to palpation. The
patient may report a clicking sensation with movement of the joint.
Furthermore, patients with a prominent xiphoid process on visual
inspection indicating an xiphisternal angle less than 160 degrees
are more prone to the development of xiphodynia (Figure 65-2).

Testing
Plain radiographs are indicated in all patients with pain thought to
be emanating from the xiphisternal joint to rule out occult bony
pathological conditions, including tumor. Based on the patients
clinical presentation, additional tests, including complete blood
cell count, prostate-specific antigen level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated.
Computed tomography (CT) or magnetic resonance imaging

Figure 65-1 The xiphisternal joint is swollen in patients with xiphodynia.

193

194 SECTION 6 Thoracic Pain Syndromes

medications do not control the patients symptoms adequately,


opioid analgesics may be added during the period of acute pain.
Local application of heat and cold may be beneficial to provide
symptomatic relief of the pain of xiphodynia. The use of an elastic
rib belt may help provide symptomatic relief in some patients.
For patients who do not respond to these treatment modalities,
the injection of the xiphisternal joint using a local anesthetic and
steroid may be a reasonable next step.

Complications and Pitfalls

Figure 65-2 Visible prominence of the xyphoid process. (From Maigne


J-Y, Vareli M, Rousset P, Cornelis P: Xiphodynia and prominence of the
xyphoid process: value of xiphosternal angle measurementthree case
reports, Joint Bone Spine 77:474476, 2010.)

The major problem in the care of patients thought to have xiphodynia is the failure to identify potentially serious pathology of the
thorax or upper abdomen. The major complication of injection
of the xiphisternal joint is pneumothorax if the needle is placed
too laterally or deeply and invades the pleural space. Infection,
although rare, can occur if strict aseptic technique is not followed.
Trauma to the contents of the mediastinum is an ever-present
possibility. This complication can be greatly decreased if the
clinician pays close attention to accurate needle placement.

Clinical Pearls

Figure 65-3 The xiphosternal angle was 105 degrees. The curved shape
of the xyphoid process hindered the measurement of the angle. (From
Maigne J-Y, Vareli M, Rousset P, Cornelis P: Xiphodynia and prominence of
the xyphoid process: value of xiphosternal angle measurementthree case
reports, Joint Bone Spine 77:474476, 2010.)

consider pneumonia, empyema, and occasionally pulmonary


embolus as the most likely diagnosis.

Treatment
Initial treatment of xiphodynia should include a combination
of simple analgesics and nonsteroidal anti-inflammatory drugs
(NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these

Patients with pain emanating from the xiphisternal joint


often attribute their pain symptoms to a heart attack or
ulcer disease. Reassurance is required, although it should
be remembered that this musculoskeletal pain syndrome,
ulcer disease, and coronary artery disease can coexist. The
xiphoid process articulates with the sternum via the xiphisternal joint. The xiphoid process is a plate of cartilaginous
bone that becomes calcified in early adulthood. The xiphisternal joint is strengthened by ligaments, but it can be subluxed or dislocated by blunt trauma to the anterior chest.
The xiphisternal joint is innervated by the T4-7 intercostal
nerves and the phrenic nerve. It is thought that this innervation by the phrenic nerve is responsible for the referred pain
associated with xiphodynia syndrome. Tietzes syndrome,
which is painful enlargement of the upper costochondral
cartilage associated with viral infections, can be confused
with xiphisternal syndrome, although both respond to the
injection technique described.
The use of physical modalities, including local heat and
gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique
for xiphisternal joint pain. Vigorous exercises should be
avoided because they would exacerbate the patients symptoms. Simple analgesics and NSAIDs may be used concurrently with this injection technique. Laboratory evaluation
for collagen-vascular disease is indicated in patients with
xiphisternal joint pain in whom other joints are involved.

SUGGESTED READINGS
Howell J: Xiphodynia: an uncommon cause of exertional chest pain, Am J Emerg
Med 8:176, 1990.
Howell JM: Xiphodynia: a report of three cases, J Emerg Med 10:435438, 1992.
Jelenko C III, Cowan GSM Jr: Perichondritis (Tietzes syndrome) at the xiphisternal
joint: a mimic of severe disease, J Am Coll Emerg Physicians 6:536542, 1977.
Koren W, Shahar A: Xiphodynia masking acute myocardial infarction: a diagnostic
cul-de-sac, Am J Emerg Med 16:177178, 1998.
Stochkendahl MJ, Christensen HW: Chest pain in focal musculoskeletal disorders,
Med Clin North Am 94:259273, 2010.

Chapter 66
SERRATUS ANTERIOR MUSCLE
SYNDROME

ICD-9 CODE 729.1


ICD-10 CODE M79.7
The Clinical Syndrome
Chest wall pain syndromes are commonly encountered in clinical practice. Some occur with relatively greater frequency and are
more readily identified by the clinician, such as costochondritis
and Tietzes syndrome. Others occur so infrequently that they are
often misdiagnosed, resulting in less-than-optimal outcome. Serratus anterior muscle syndrome is one such infrequent cause of
anterior chest wall pain. The syndrome is a constellation of symptoms consisting of pain overlying the fifth to the seventh ribs in
the midaxillary line, with referred pain that may radiate down the
ispilateral upper extremity into the palmar aspect of the ring and
little finger. Serratus anterior muscle syndrome can mimic the pain
of myocardial infarction and is frequently misdiagnosed as such. It
is a myofascial pain syndrome. The intensity of the pain associated
with serratus anterior muscle syndrome is mild to moderate and is
described as having a deep, aching character. The pain of serratus
anterior muscle syndrome is intermittent.

Signs and Symptoms


On physical examination, the patient with serratus anterior muscle
syndrome will exhibit myofascial trigger points overlying the 5th
to 7th ribs in the midaxillary line, with referred pain that may radiate down the ispilateral upper extremity into the palmar aspect of
the ring and little fingers (Figure 66-1). Pain is reproduced with
palpation of these trigger points rather than with movement of
the chest wall and shoulders. A positive jump sign will be present when these trigger points are stimulated. Trigger points at the
lateral border of the scapula may be present and amenable to injection therapy. As mentioned, movement of the shoulders and chest
wall will not exacerbate the pain.

Testing
Plain radiographs are indicated in all patients with suspected
serratus anterior muscle syndrome to rule out occult bony
pathological processes, including metastatic lesions. Based on
the patients clinical presentation, additional testing may be

indicated, including complete blood cell count, prostate-specific


antigen level, sedimentation rate, and antinuclear antibody testing. Magnetic resonance imaging (MRI) of the chest is indicated
if a retrosternal mass such as thymoma is suspected or if trauma
to the serratus anterior muscle itself has occurred (Figure 66-2).
Electromyography is indicated in patients with serratus anterior muscle syndrome to help rule out cervical radiculopathy
or plexopathy that may be considered because of the referred
arm pain. Injection of the serratus anterior muscle with a local
anesthetic and steroid serves as both a diagnostic and therapeutic
maneuver.

Differential Diagnosis
As mentioned, the pain of serratus anterior muscle syndrome is
often mistaken for pain of cardiac origin and can lead to visits
to the emergency department and unnecessary cardiac workups.
If trauma has occurred, serratus anterior muscle syndrome may
coexist with fractured ribs or fractures of the sternum itself, which
can be missed on plain radiographs and may require radionucleotide bone scanning for proper identification. Tietzes syndrome,
which is painful enlargement of the upper costochondral cartilage
associated with viral infections, can be confused with sternalis
syndrome, as can costosternal syndrome.
Neuropathic pain involving the chest wall may be confused or
coexist with costosternal syndrome. Examples of such neuropathic
pain include diabetic polyneuropathies and acute herpes zoster
involving the thoracic nerves. The possibility of diseases of the
structures of the mediastinum remains ever present and at times
can be difficult to diagnose. Pathological processes that inflame
the pleura, such as pulmonary embolus, infection, and tumor, also
should be considered.

Treatment
Initial treatment of serratus anterior muscle syndrome should
include a combination of simple analgesics and the nonsteroidal
anti-inflammatory agents or the cyclooxygenase-2 (COX-2)
inhibitors. The local application of heat and cold may be beneficial to provide symptomatic relief of the pain of serratus anterior
muscle syndrome. The use of an elastic rib belt may help provide
symptomatic relief in some patients. For patients who do not
respond to these treatment modalities, injection of the trigger
areas located in the sternalis muscle using a local anesthetic and
steroid may be a reasonable next step.
195

196 SECTION 6 Thoracic Pain Syndromes


Side view

Serratus
anterior m.

Trigger point
Referred pain

Figure 66-1 Serratus anterior muscle syndrome is a constellation of symptoms consisting of anterior chest wall pain that can radiate to the retrosternal
area and the medial aspect of the arm.

Complications and Pitfalls

*
A

Figure 66-2 T2-weighted magnetic resonance image showing the


hematoma (*) in the right chest wall after traumatic avulsion of the
serratus anterior muscle. A, Axial view. B, Coronal view. (From Otoshi
K, Itoh Y, Tsujino A, Hasegawa M, Kikuchi S: Avulsion injury of the serratus
anterior muscle in a high-school underhand pitcher: a case report, J Shoulder Elbow Surg 16:e45e47, 2007.)

The major problem in the care of patients thought to have serratus


anterior muscle syndrome is the failure to identify potentially serious pathological conditions of the thorax or mediastinum. Given
the proximity of the pleural space, pneumothorax after injection
of the serratus anterior muscle is a distinct possibility, as is injury
to the mediastinal and intrathoracic structures. Approximately
25% of patients will report a transient increase in pain after this
injection technique and should be warned of this.

66 Serratus Anterior Muscle Syndrome 197

Clinical Pearls
Patients with serratus anterior muscle syndrome will often
go the emergency department, fearing they are having a
heart attack. The syndrome is also frequently misdiagnosed
as a cervical radiculopathy because of the referred arm pain.
Electromyography will help delineate the cause and extent
of neural compromise.
This injection technique is extremely effective in the
treatment of serratus anterior muscle syndrome. Coexistent
costosternal or manubriosternal arthritis may contribute to
anterior chest wall pain and may require additional treatment with a more localized injection of an anesthetic and
depot steroid. This technique is a safe procedure if careful attention is paid to the clinically relevant anatomy in
the areas to be injected. Pneumothorax can be avoided if
shorter needles are used and the needle is not advanced
too deeply. Care must be taken to use sterile technique to
avoid infection, and universal precautions must be used to
avoid risk to the operator. The incidence of ecchymosis and
hematoma formation can be decreased if pressure is placed
on the injection site immediately after injection. The use of
physical modalities, including local heat and gentle rangeof-motion exercises, should be introduced several days after
the patient undergoes this injection technique for shoulder
pain. Vigorous exercises should be avoided because they will
exacerbate the symptoms. Simple analgesics and nonsteroidal anti-inflammatory agents may be used concurrently
with this injection technique.

SUGGESTED READINGS
Bradley LA: Pathophysiology of fibromyalgia, Am J Med 122(Suppl 1):S22S30,
2009.
Ge H-Y, Nie H, Madeleine P, etal: Contribution of the local and referred pain from
active myofascial trigger points in fibromyalgia syndrome, Pain 147:233240,
2009.
Son MBF, Sundel RP: Musculoskeletal causes of pediatric chest pain, Pediatr Clin
North Am 57:13851395, 2010.
Yurtsever I, Topal U, Yalin R, Adm B, Bayram S: Desmoid tumor of the chest
wall, Eur J Radiol Extra 46:119121, 2003.

Chapter 67
SLIPPING RIB SYNDROME

ICD-9 CODE 786.59


ICD-10 CODE R07.82
The Clinical Syndrome
Encountered more frequently in clinical practice since the
increased use of across-the-chest seat belts and airbags, slipping
rib syndrome is often misdiagnosed, leading to prolonged suffering and excessive testing for intra-abdominal and intrathoracic
pathological conditions. Slipping rib syndrome is a constellation
of symptoms consisting of severe knifelike pain emanating from
the lower costal cartilages associated with hypermobility of the
anterior end of the lower costal cartilages. The tenth rib is most
commonly involved, but the eighth and ninth ribs also can be
affected. This syndrome is also known as the rib-tip syndrome.
Slipping rib syndrome is almost always associated with trauma
to the costal cartilage of the lower ribs. These cartilages are often
traumatized during acceleration/deceleration injuries and blunt
trauma to the chest. With severe trauma, the cartilage may sublux
or dislocate from the ribs. Patients with slipping rib syndrome
may report a clicking sensation with movement of the affected
ribs and associated cartilage.

imaging (MRI) of the affected ribs and cartilage is indicated if


joint instability or occult mass is suspected. The injection technique discussed in this chapter serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis
As mentioned earlier, the pain of slipping rib syndrome is often
mistaken for pain of cardiac or gallbladder origin and can lead
to visits to the emergency department and unnecessary cardiac
and gastrointestinal workups. If trauma has occurred, slipping rib
syndrome may coexist with rib fractures or fractures of the sternum, which can be missed on plain radiographs and may require
radionucleotide bone scanning for proper identification. Tietzes
syndrome, which is painful enlargement of the upper costochondral cartilage associated with viral infections, can be confused with

8th rib
9th rib
10th rib

Signs and Symptoms


On physical examination, the patient vigorously attempts to splint
the affected costal cartilage joints by keeping the thoracolumbar
spine slightly flexed (Figure 67-1). Pain is reproduced with pressure on the affected costal cartilage. Patients with slipping rib
syndrome exhibit a positive hooking maneuver test. The hooking
maneuver test is performed by having the patient lie in the supine
position with the abdominal muscles relaxed while the clinician
hooks his or her fingers under the lower rib cage and pulls gently
outward. Pain and a clicking or snapping sensation of the affected
ribs and cartilage indicate a positive test.

Testing
Plain radiographs are indicated in all patients who present with
pain thought to be emanating from the lower costal cartilage and
ribs to rule out occult bony pathological processes, including rib
fracture and tumor. Based on the patients clinical presentation,
additional tests, including complete blood cell count, prostatespecific antigen level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance
198

Figure 67-1 Patients with slipping rib syndrome exhibit pain on hooking of the affected costochondral cartilage.

67 Slipping Rib Syndrome 199

slipping rib syndrome, as can devils grip, which is a pleura-based


pain syndrome of infectious origin.
Neuropathic pain involving the chest wall also may be confused or coexist with slipping rib syndrome. Examples of such
neuropathic pain include diabetic polyneuropathies and acute
herpes zoster involving the thoracic nerves. The possibility of diseases of the structures of the mediastinum is ever present, and
these diseases sometimes can be difficult to diagnose. Pathological processes that inflame the pleura, such as pulmonary embolus,
infection, and tumor, also should be considered.

Treatment
Initial treatment of the pain and functional disability associated
with slipping rib syndrome should include a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2
(COX-2) inhibitors and physical therapy. The local application of
heat and cold may be beneficial. The repetitive movements that
incite the syndrome should be avoided. For patients who do not
respond to these treatment modalities, injection of the affected
costochondral cartilages with a local anesthetic and steroid may be
a reasonable next step.
To inject the slipping ribs, the patient is placed in the supine
position, and proper preparation with antiseptic solution of the skin
overlying the affected costal cartilage and rib is done. A sterile syringe
containing 1 mL of 0.25% preservative-free bupivacaine for each
joint to be injected and 40 mg of methylprednisolone is attached to
a 25-gauge, 1-inch needle using strict aseptic technique.
With strict aseptic technique, the distal rib and costal cartilage
are identified. The lower margin of each affected distal rib is identified and marked with a sterile marker. The needle is carefully
advanced at the point marked through the skin and subcutaneous
tissues until the needle tip impinges on the periosteum of the
underlying rib. The needle is withdrawn back into the subcutaneous tissues and walked inferiorly off the inferior rib margin. The
needle should be advanced just beyond the inferior rib margin,
but no farther, or pneumothorax or damage to the abdominal
viscera could result. After careful aspiration to ensure that the
needle tip is not in an intercostal vein or artery, 1 mL of solution
is gently injected. There should be limited resistance to injection. If significant resistance is encountered, the needle should be
withdrawn slightly until the injection proceeds with only limited
resistance. This procedure is repeated for each affected rib and
associated cartilage. The needle is removed, and a sterile pressure
dressing and ice pack are placed at the injection site.

Complications and Pitfalls


The major problem in the care of patients thought to have slipping rib syndrome is the failure to identify potentially serious

pathological conditions of the thorax or upper abdomen. Given


the proximity of the pleural space, pneumothorax after the injection technique described is a possibility. The incidence of the
complication is less than 1%, but it occurs with greater frequency
in patients with chronic obstructive pulmonary disease. Because
of the proximity to the intercostal nerve and artery, the clinician
should calculate carefully the total milligram dosage of local anesthetic administered, in consideration of the high vascular uptake
via these vessels. Although uncommon, infection is an ever-present possibility, especially in an immunocompromised patient with
cancer. Early detection of infection is crucial to avoid potentially
life-threatening sequelae.

Clinical Pearls
Patients with pain from slipping rib syndrome often attribute their pain symptoms to a gallbladder attack or ulcer
disease. Reassurance is required, although it should be
remembered that this musculoskeletal pain syndrome and
intra-abdominal pathological conditions can coexist. Care
must be taken to use sterile technique to avoid infection
and universal precautions to avoid risk to the operator. The
incidence of ecchymosis and hematoma formation can be
decreased if pressure is placed on the injection site immediately after injection. The use of physical modalities, including local heat and gentle range-of-motion exercises, should
be introduced several days after the patient undergoes this
injection technique for slipping rib syndrome. Vigorous
exercises should be avoided because they would exacerbate
the symptoms. Simple analgesics and NSAIDs may be used
concurrently with this injection technique. Laboratory evaluation for collagen-vascular disease is indicated in patients
with costal cartilage pain in whom other joints are involved.

SUGGESTED READINGS
Brunse MH, Stochkendahl MJ, Vach W, etal: Examination of musculoskeletal
chest pain: an inter-observer reliability study, Manual Ther 15:167172, 2010.
Cranfield KAW, Buist RJ, Nandi PR, Baranowski AP: The twelfth rib syndrome,
J Pain Symptom Manage 13:172175, 1997.
Fam AG, Smythe HA: Musculoskeletal chest wall pain, CMAJ 133:379389,
1985.
Stochkendahl MJ, Christensen HW: Chest pain in focal musculoskeletal disorders,
Med Clin North Am 94:259273, 2010.
Verdon F, Herzig L, Burnand B, etal: Chest pain in daily practice: occurrence,
causes and management, Swiss Med Wkly 138:340347, 2008.
Wright JT: Slipping-rib syndrome, Lancet 316:632634, 1980.

Chapter 68
WINGED SCAPULA SYNDROME

ICD-9 CODE 736.89


ICD-10 CODE M21.80
The Clinical Syndrome
Winged scapula syndrome is an uncommon cause of musculoskeletal pain of the shoulder and posterior chest wall. Caused
by paralysis of the serratus anterior muscle, winged scapula
syndrome begins as a painless weakness of the muscle with the
resultant pathognomonic finding of winged scapula. As a result
of dysfunction secondary to paralysis of the muscle, musculoskeletal pain often results. Winged scapula syndrome is often initially
misdiagnosed as strain of the shoulder groups and muscles of
the posterior chest wall because the onset of the syndrome often
occurs after heavy exertion, most commonly after carrying heavy
backpacks. The syndrome may coexist with entrapment of the
suprascapular nerve.
Trauma to the long thoracic nerve of Bell is most often responsible for the development of winged scapula syndrome. Arising
from the fifth, sixth, and seventh cervical nerves, the nerve is susceptible to stretch injuries and direct trauma. The nerve is often
injured during first rib resection for thoracic outlet syndrome.
Injuries to the brachial plexus or cervical roots also may cause
scapular winging, but usually in conjunction with other neurological findings.
The pain of winged scapula syndrome is aching and is localized
to the muscle mass of the posterior chest wall and scapula. The pain
may radiate into the shoulder and upper arm. The intensity of the
pain of winged scapula syndrome is mild to moderate, but it may
produce significant functional disability, which, if untreated, continues to exacerbate the musculoskeletal component of the pain.

Signs and Symptoms


Regardless of the mechanism of injury to the long thoracic
nerve of Bell, the common clinical feature of winged scapula
syndrome is paralysis of the scapula resulting from weakness of
the serratus anterior muscle. The pain of winged scapula syndrome generally develops after the onset of acute muscle weakness, but it is often erroneously attributed to overuse during
vigorous exercise. On physical examination, the last 30 degrees
of overhead arm extension is lost and the scapular rhythm is
disrupted. By having the patient press the outstretched arms
against a wall, the scapular winging is easily viewed by the clinician observing the patient from behind. The remainder of the
200

patients neurological examination should be within normal


limits (Figure 68-1).

Testing
Owing to the ambiguity and confusion surrounding this clinical
syndrome, testing is important to help confirm the diagnosis of
winged scapula syndrome. Electromyography helps distinguish
isolated damage to the long thoracic nerve of Bell associated
with winged scapula syndrome from brachial plexopathy. Plain
radiographs are indicated in all patients who present with winged
scapula syndrome to rule out occult bony pathological processes.
Based on the patients clinical presentation, additional tests,
including complete blood cell count, uric acid level, erythrocyte
sedimentation rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the brachial plexus,
cervical spine, or both is indicated if the patient exhibits other
neurological deficits.

Differential Diagnosis
Lesions of the cervical spinal cord, brachial plexus, and cervical
nerve roots can produce clinical symptoms that include winging
of the scapula. Such lesions also should produce additional neurological findings that allow the clinician to distinguish these pathological conditions from the isolated neurological findings seen in
winged scapula syndrome. Pathology of the scapula or shoulder
group also may confuse the clinical diagnosis.

Treatment
No specific treatment for winged scapula syndrome exists other than
removal of the cause of nerve entrapment (e.g., heavy backpacks
or tumor compressing a nerve) and use of an orthotic device to
help stabilize the scapula to allow normal shoulder function. Initial
symptomatic relief of the pain and functional disability associated
with winged scapula should include a combination of nonsteroidal
anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2)
inhibitors and physical therapy. Local application of heat and cold
also may be beneficial. Repetitive movements or movements that
incite the syndrome should be avoided.

Complications and Pitfalls


The major complications associated with winged scapula syndrome fall into two categories: (1) damage to the shoulder resulting
from the functional disability associated with the syndrome and (2)
failure to recognize that the cause of winging of the scapula is the

68 Winged Scapula Syndrome 201

Scapula
Long thoracic
nerve (C5-C7)
Serratus anterior
muscle

Figure 68-1 Scapular winging is best viewed by having the patient push his or her hands against the wall.

result not of an isolated lesion of the long thoracic nerve of Bell but
rather a part of a larger neurological problem.

Clinical Pearls
Winged scapula syndrome is a distinct clinical entity that
is difficult to treat. Early removal of the offending cause
of nerve entrapment should allow rapid recovery of nerve
function with resultant improvement in pain and shoulder
dysfunction. A careful search for other causes of winging of
the scapula should occur before attributing this neurological finding to winged scapula syndrome.

SUGGESTED READINGS
Akgun K, Aktas I, Terzi Y: Winged scapula caused by a dorsal scapular nerve
lesion: a case report, Arch Phys Med Rehabil 89:20172020, 2008.
Belville RG, Seupaul RA: Winged scapula in the emergency department: a case
report and review, J Emerg Med 29:279282, 2005.
Nakatsuchi Y, Saitoh S, Hosaka M, Uchiyama S: Long thoracic nerve paralysis
associated with thoracic outlet syndrome, J Shoulder Elbow Surg 3:2833, 1994.
Sherman SC, OConnor M: An unusual cause of shoulder pain: winged scapula,
J Emerg Med 28:329331, 2005.

SECTION 7 Abdominal and Groin Pain Syndromes

Chapter 69
ANTERIOR CUTANEOUS NERVE
ENTRAPMENT

ICD-9 CODE 355.9


ICD-10 CODE G58.9
The Clinical Syndrome
Anterior cutaneous nerve entrapment is an uncommon cause of
anterior abdominal wall pain that is a frequently overlooked clinical
diagnosis. Anterior cutaneous nerve entrapment syndrome is a constellation of symptoms consisting of severe knifelike pain emanating
from the anterior abdominal wall associated with the physical finding of point tenderness over the affected anterior cutaneous nerve.
The pain radiates medially to the linea alba but in almost all cases
does not cross the midline. Anterior cutaneous nerve entrapment
syndrome occurs most commonly in young women. The patient
can often localize the source of pain accurately by pointing to the
spot at which the anterior cutaneous branch of the affected intercostal nerve pierces the fascia of the abdominal wall at the lateral
border of the abdominus rectus muscle (Figure 69-1). At this point,
the anterior cutaneous branch of the intercostal nerve turns sharply
in an anterior direction to provide innervation to the anterior wall.
The nerve passes through a firm fibrous ring as it pierces the fascia,
and at this point the nerve becomes subject to entrapment. The
nerve is accompanied through the fascia by an epigastric artery and
vein. The potential exists for small amounts of abdominal fat to
herniate through this fascial ring and become incarcerated, which
results in further entrapment of the nerve. The pain of anterior
cutaneous nerve entrapment is moderate to severe in intensity.

Signs and Symptoms


As mentioned earlier, the patient often can point to the exact
spot that the anterior cutaneous nerve is entrapped. Palpation
of this point often elicits sudden sharp, lancinating pain in the
distribution of the affected anterior cutaneous nerve. Voluntary
contraction of the abdominal muscles puts additional pressure on
the nerve and may elicit the pain. The patient attempts to splint
202

the affected nerve by keeping the thoracolumbar spine slightly


flexed to avoid increasing tension on the abdominal musculature
(Figure 69-2). Having the patient do a sit-up often reproduces
the pain, as does a Valsalva maneuver. Patients with anterior cutaneous nerve entrapment will also exhibit a positive Carnetts test
when the patient is asked to tense his or her abdominal musculature, which is indicative of abdominal wall pain rather than pain
with an intra-abdominal nidus (Figure 69-3).

Testing
Plain radiographs are indicated in all patients with pain thought
to be emanating from the lower costal cartilage and ribs to rule
out occult bony pathological conditions, including rib fracture
and tumor. Radiographic evaluation of the gallbladder is indicated if cholelithiasis is suspected. Based on the patients clinical presentation, additional tests, including complete blood cell
count, rectal examination with stool guaiac, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. Ultrasonography and computed tomography (CT) scan
of the abdomen are indicated if intra-abdominal pathological
process or occult mass is suspected. Injection of the anterior
cutaneous nerve with or without ultrasound guidance at the
point at which it pierces the fascia serves as a diagnostic and
therapeutic maneuver (Figure 69-4).

Differential Diagnosis
The differential diagnosis of anterior cutaneous nerve entrapment
syndrome should consider ventral hernia, peptic ulcer disease,
cholecystitis, intermittent bowel obstruction, renal calculi,
angina, mesenteric vascular insufficiency, diabetic polyneuropathy, and pneumonia (Table 69-1). Rarely, the collagen-vascular
diseases, including systemic lupus erythematosus and polyarteritis nodosa, may cause intermittent abdominal pain; porphyria
also may cause intermittent abdominal pain. Because the pain of
acute herpes zoster may precede the rash by 24 to 72 hours, the
pain may be attributed erroneously to anterior cutaneous nerve
entrapment.

69 Anterior Cutaneous Nerve Entrapment 203

Linea alba
Rectus
abdominis

Entrapped anterior
cutaneous nerve

Transverse
abdominis

Figure 69-1 The course of the anterior cutaneous nerve within the abdominal wall.

A
Rectus
sheath
Anterior
cutaneous
nerve
Figure 69-2 Patients with anterior cutaneous nerve entrapment often
attempt to splint the affected nerve by keeping the thoracolumbar
spine slightly flexed to avoid increasing tension on the abdominal
musculature.

Treatment
Initial treatment of the pain and functional disability associated
with anterior cutaneous entrapment syndrome should include a
combination of nonsteroidal anti-inflammatory drugs (NSAIDs)
or the cyclooxygenase-2 (COX-2) inhibitors and physical
therapy. Local application of heat and cold may be beneficial.
The repetitive movements that incite the syndrome should be
avoided. For patients who do not respond to these treatment
modalities, injection of the anterior cutaneous nerve at the point
at which the nerve pierces the fascia with a local anesthetic and
steroid may be a reasonable next step. If the symptoms of anterior
cutaneous entrapment syndrome persist, surgical exploration and
decompression of the anterior cutaneous nerve are indicated.

B
Figure 69-3 A, The patient is asked to completely relax the abdominal
muscles and point with one finger to the most painful area. B, The
patient is then asked to maximally tense the abdominal muscles. The
Carnetts test is positive if the localized pain increases at the previously
identified painful area.

Complications and Pitfalls


The major complications associated with anterior cutaneous
entrapment syndrome fall into two categories: (1) iatrogenically
induced complications secondary to incorrect diagnosis and (2)

204 SECTION 7 Abdominal and Groin Pain Syndromes

Figure 69-4 Transverse ultrasound image demonstrating the linea alba, rectus muscles, and the skin and subcutaneous tissues.
TABLE 69-1

The Differential Diagnosis of Anterior Cutaneous Nerve Entrapment Syndrome


Differential Diagnosis

Investigations and Characteristics

Anterior cutaneous nerve entrapment syndrome

Carnetts test, injection of local anesthetics

Thoracic lateral cutaneous nerve entrapment

History of previous surgery, clinical examination

Ilioinguinal or iliohypogastric nerve entrapment

History of previous groin surgery, clinical examination, injection of local anesthetics

Endometriosis

History of cyclic abdominal pain, laparoscopy

Myofascial pain syndrome

Clinical examination, myofascial strain

Slipping rib syndrome

Hypermobile, luxating eighth to tenth ribs, clinical examination

Diabetic radiculopathy

Paraspinal EMG, patient with diabetes mellitus

Abdominal wall tear

History of acute pain related to lifting or stretching, athletes

Abdominal wall or rectus sheath hematoma

Abdominal ultrasound or CT scan, after laparoscopy, after coughing in anticoagulated


patient

Herpes zoster

History and clinical examination, dermatomal

Abdominal wall tumor (lipoma, desmoid, metastasis)

History and clinical examination, abdominal CT scan

Spinal nerve irritation

Referred pain by thoracic spine pathological condition

Hernia

Abdominal ultrasound, clinical examination

Traction symphysitis or pubalgia

Athletes, positive findings on MRI or scintigraphy

CT, Computed tomographic; EMG, electromyography; MRI, magnetic resonance imaging.

failure of the clinician to recognize that a hernia coexists with the


nerve entrapment until bowel ischemia occurs.

Clinical Pearls
Patients with pain from anterior cutaneous nerve entrapment syndrome often attribute their pain symptoms to a
gallbladder attack or ulcer disease. Reassurance is required,
although it should be remembered that this musculoskeletal
pain syndrome and intra-abdominal pathological conditions
can coexist. The use of physical modalities, including local
heat and gentle range-of-motion exercises, should be introduced several days after the patient undergoes this injection technique for anterior cutaneous nerve entrapment
syndrome. Vigorous exercises should be avoided because
they would exacerbate the symptoms. Simple analgesics and
NSAIDs may be used concurrently with the aforementioned
injection technique. Radiographic evaluation for intraabdominal pathological conditions is indicated in patients
with anterior abdominal pain of unclear origin.

SUGGESTED READINGS
Hall MW, Sowden DS, Gravestock H, et al: Abdominal wall tenderness test,
Lancet 7:16061607, 1991.
Kanakarajan S, High K, Nagaraja R: Chronic abdominal wall pain and ultrasoundguided abdominal cutaneous nerve infiltration: a case series, Pain Med 12:
382386, 2011.
Kuan L-C, Li Y-T, Chen F-M, etal: Efficacy of treating abdominal wall pain by
local injection, Taiwan J Obstet Gynecol 45:239243, 2006.
Srinivasan R, Greenbaum DS: Chronic abdominal wall pain: a frequently overlooked
problem: practical approach to diagnosis and management, Am J Gastroenterol
97:824830, 2002.

Chapter 70
ACUTE INTERMITTENT PORPHYRIA

ICD-9 CODE 277.1


ICD-10 CODE E802.9
The Clinical Syndrome
Acute intermittent porphyria is an uncommon cause of abdominal
pain that frequently confounds the diagnostic efforts of even the
most astute clinician. The porphyrias are disorders of heme synthesis that can produce a wide range of clinical symptoms. Many
different types of porphyrias occur, each of which manifests in a
distinct clinical manner that reflects the specific enzyme deficiency
of the heme biosynthetic pathway. The porphyrias can be inherited or acquired. The main clinical manifestations of the porphyrias are neurological dysfunction and the unique clinical finding of
cutaneous sensitivity to sunlight.
Acute intermittent porphyria is an autosomal dominant trait
with variable clinical expression. The incidence of the gene
responsible for acute intermittent porphyria is thought to be 1 in
100,000 individuals. The disease rarely manifests before puberty.
Acute abdominal pain is usually the first clinical expression of
the disease. As the name implies, the pain of acute intermittent
porphyria is intermittent and colicky. The pain may be localized
to the abdomen or may radiate to the flanks. The patient also
may exhibit neurological symptoms, suggesting dysfunction of the
central and peripheral nervous systems. Port wine urine, which is
characteristic of hepatic porphyrias, including acute intermittent
porphyria, is often seen during acute attacks.

as may dehydration, calorie restriction, hormonal alterations, and


infection.

Testing
Given the usual delay in the diagnosis of acute intermittent porphyria, a considerable amount of testing is done. Most standard
laboratory testing does not point the clinician toward a diagnosis
of acute intermittent porphyria, however. Specifically, liver function tests are normal. A mild normocytic, normochromic anemia
is sometimes present. Freshly passed urine is colorless, but it turns
a port wine color if exposed to light. Given the low incidence of
porphyria, qualitative urine screening tests, such as the WatsonSchwartz test, is a reasonable first step in diagnosing porphyria. If
the qualitative tests are positive, quantitative testing, such as gas
chromatographic measurements for aminolevulinic acid, should
be performed.

Signs and Symptoms


Although the reports of abdominal pain are often quite impressive
in patients with acute intermittent porphyria, the abdominal examination is often nondescript (Figure 70-1). Vomiting occasionally
occurs. Tachycardia and autonomic dysfunction, including sweating, are common, as is labile hypertension. Occasionally, urinary
retention may occur and may confuse the clinical diagnosis, especially if port wine urine is present. Neurological findings, including
attenuated deep tendon reflexes and decreased distal sensation suggestive of peripheral neuropathy, are often present. Cranial nerve
involvement is less common, but can be severe. Mental disturbance ranging from agitation to frank psychosis can occur in one
third of patients with acute intermittent porphyria. The presence
of nervousness and anxiety often makes the care of these patients
difficult. Alcohol, smoking, pregnancy, barbiturates, and oral contraceptives may precipitate attacks of acute intermittent porphyria,

Figure 70-1 Patients with acute intermittent porphyria often report


very severe abdominal pain.

205

206 SECTION 7 Abdominal and Groin Pain Syndromes

Differential Diagnosis
Essentially all causes of acute intermittent abdominal pain must
be included in the differential diagnosis. The clinician needs to
take a detailed history and perform a careful physical examination to rule out life-threatening causes of acute, intermittent
abdominal pain, such as ischemic bowel, volvulus, and acute
appendicitis. The key distinguishing factor in acute intermittent
porphyria is that the patients report of severe abdominal pain
and the benign abdominal examination do not correlate. Given
the high incidence of psychiatric abnormalities in patients with
acute intermittent porphyria, psychogenic causes of abdominal
pain must be included in the differential diagnosis.

Treatment
Attacks of acute intermittent porphyria can be aborted by the
intravenous administration of large quantities of carbohydrates,
such as glucose. Hematin can be given intravenously and seems to
be well tolerated. Cimetidine, a histamine-2 inhibitor, also may
be useful in ameliorating acute attacks. Avoidance of barbiturates,
anticonvulsants, and alcohol is imperative to avoid exacerbating
the symptoms of acute intermittent porphyria attacks. Careful attention to fluid and electrolyte balance also is important.
Despite careful treatment, fatalities during attacks do occur.

Complications and Pitfalls


The major complications surrounding acute intermittent porphyria relate to misdiagnosis and failure to correct metabolic
and electrolyte abnormalities during acute attacks. Barbiturates

and anticonvulsants are often erroneously given to control


seizures associated with acute intermittent porphyria, which
worsen the porphyria, creating a vicious negative feedback cycle
that ultimately may kill the patient.

Clinical Pearls
The cause of abdominal pain in acute intermittent porphyria is thought to be the result of intermittent autonomic
dysfunction causing abnormal gut motility with alternating
spasm and obstruction. The incidence of psychiatric abnormalities in patients with acute intermittent porphyria often
confounds the clinician and complicates treatment. It has
been said that to make a diagnosis, the clinician must think
of it first. Nowhere is this statement more true than in the
case of acute intermittent porphyria.

SUGGESTED READINGS
Crimlisk HL: The little imitator: porphyriaa neuropsychiatric disorder, J Neurol
Neurosurg Psychiatry 62:319328, 1997.
Herrick AL, McColl KEL: Acute intermittent porphyria, Best Pract Res Clin
Gastroenterol 19:235249, 2005.
Kuo H-C, Lee M-J, Chuang W-L, Huang C-C: Acute intermittent porphyria with
peripheral neuropathy: a follow-up study after hematin treatment, J Neurol Sci
260:231235, 2007.
Peters TJ, Deacon AC: International air travel: a risk factor for attacks in acute
intermittent porphyria, Clin Chim Acta 335:5963, 2003.
Shen FC, Hsieh CH, Huang CR: Acute intermittent porphyria presenting as acute
pancreatitis and posterior reversible encephalopathy syndrome, Acta Neurol
Taiwan 17:177183, 2008.

Chapter 71
RADIATION ENTERITIS

ICD-9 CODE 558.1


ICD-10 CODE K52.0
The Clinical Syndrome
As cancer patients live longer, clinicians are being called on
with greater frequency to manage the side effects and complications of cancer therapy. One such complication is radiation
enteritis. This complication of radiation therapy can occur after
radiation to the abdomen or pelvis. Early symptoms of radiation
enteritis are due to mucosal edema and ulceration and include
abdominal pain, nausea, vomiting, and a sensation of needing
to move the bowels, tenesmus, or both. Late symptoms that
are more related to radiation-induced scarring and narrowing
of the bowel include small-caliber stools, rectal burning, and
mucoid stools. The intensity of pain is mild to moderate and
cramping. The onset of the early symptoms of radiation enteritis can begin within 1 week to 10 days after the completion
of radiation therapy, and the late symptoms can occur months
to years later. A variety of factors can predispose the patient
to the development of radiation enteritis, including preexisting
systemic disease such as diabetes and treatment-related factors
(Table 71-1).

Differential Diagnosis
A history of previous radiation therapy is necessary to consider the
diagnosis of radiation enteritis. The very problem that necessitated
radiation therapy in the first placemalignancy can recur, however, and produce clinical symptoms indistinguishable from those
of radiation enteritis. Given the immunocompromised state of
most patients who have received radiation therapy, the possibility
of infectious enteritis or intra-abdominal abscess always must be
included in the differential diagnosis. Other causes of abdominal
pain, including diverticulitis, bowel obstruction, and appendicitis,
also may occur in conjunction with radiation enteritis.

Treatment
Symptom management is the primary thrust of the treatment of
radiation enteritis. Careful attention to the patients fluid and
metabolic status during the acute phases of the disease is crucial to
avoid complications. Psyllium helps the patient with diarrhea and
with mucoid stools and may decrease the sensations of needing to
move the bowels frequently. Anticholinergics such as dicyclomine
and antiperistaltics such as loperamide can help decrease diarrhea.
Zinc oxide ointment and sitz baths with aluminum acetate soaks
help with the symptoms of tenesmus and rectal pain. Steroid and
sucralfate enemas also have been reported to provide symptomatic
relief in difficult cases of radiation enteritis.

Signs and Symptoms

Complications and Pitfalls

Physical examination of a patient with radiation enteritis reveals


diffuse abdominal tenderness and hyperactive bowel sounds. Mild
abdominal distention may be present. Signs of acute peritoneal
irritation suggestive of perforated viscus, such as rebound tenderness, are absent. The patient may exhibit frequent mucoid stools,
diarrhea, and vomiting. The patient appears systemically ill, but
not septic (Figure 71-1).

The potential for complications after radiation therapy is high.


Spontaneous bowel perforation, stenosis, fistula formation, bleeding,
and malabsorption occur with sufficient frequency to complicate the
management of this painful condition. As mentioned, the potential
for recurrence of tumor and infectious complications is ever present.
TABLE 71-1

Testing

Risk Factors Associated with Chronic Radiation Enteritis

Colonoscopy provides definitive evidence of radiation enteritis,


while helping to exclude other causes of abdominal pain that may
mimic this clinical syndrome. Based on the patients clinical presentation, additional tests, including complete blood cell count,
erythrocyte sedimentation rate, and stool and blood cultures for
infectious enteritis, may be indicated. Computed tomography
(CT) of the abdomen with oral and intravenous contrast material is indicated if occult mass or abscess is suspected. Magnetic
resonance imaging (MRI) of the abdomen also helps confirm the
diagnosis of radiation enteritis (Figure 71-2).

Patient Factors

Treatment Factors

Reduced body mass index

Volume of small bowel in radiotherapy field

Comorbidities (e.g., diabetes


mellitus, hypertension,
inflammatory bowel disease)

Radiotherapy dose and


fractionation

Smoking

Radiotherapy technique

Previous intestinal surgery

Concomitant chemotherapy use

Modified from Theis VS, Sripadam R, Ramani V, etal: Chronic radiation enteritis,
Clin Oncol 2:7083, 2010.

207

208 SECTION 7 Abdominal and Groin Pain Syndromes

Clinical Pearls
Treatment of the symptoms associated with radiation enteritis should be part of the overall management of a patient
with cancer. The recognition and treatment of symptoms
other than pain are often delayed while the clinician focuses
on pain control, further compounding the patients suffering. Vigilance for life-threatening complications of radiation enteritis, including bowel perforation, is mandatory to
avoid disaster.

SUGGESTED READINGS
Andreyev HJ: Gastrointestinal problems after pelvic radiotherapy: the past, the
present and the future, Clin Oncol 1979019799, 2007.
Chon BH, Loeffler JS: The effect of nonmalignant systemic disease on tolerance to
radiation therapy, Oncologist 7:136143, 2002.
Theis VS, Sripadam R, Ramani V, Lal S: Chronic radiation enteritis, Clin Oncol
22:7083, 2010.
Waddell BE, Rodriguez-Bigas MA, Lee RJ, Weber TK, Petrelli NJ: Prevention of
chronic radiation enteritis, J Am Coll Surg 189:611624, 1999.

Figure 71-1 Patients with radiation enteritis typically exhibit diffuse


abdominal tenderness, hyperactive bowel sounds, and mild abdominal
distention, with frequent mucoid stools, diarrhea, and vomiting. They
appear systemically ill, but are not septic.

Figure 71-2 Radiation enteritis. Gadolinium-enhanced spoiled gradient


echo image after pelvic radiation shows segmental mural thickening
and enhancement (arrows) representing evidence of radiation enteritis.
(From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2006, Saunders, p 2701.)

Chapter 72
LIVER PAIN

ICD-9 CODE 573.8


ICD-10 CODE K76.8

of peritoneal irritation over the right upper quadrant. A friction


rub is often present with auscultation over the liver. The liver may
be extremely tender to palpation. Primary tumor or metastatic
disease or both may be present.

Testing
The Clinical Syndrome
Liver pain is a common clinical occurrence, but it is often poorly
diagnosed and treated. The liver can serve as a source of pain in and
of itself through the sympathetic nervous system and via referred
pain secondary to peritoneal irritation through the intercostal and
subcostal nerves. Pain that emanates from the liver itself tends to
be ill defined and may be referred primarily to the epigastrium. It
is dull and aching and is mild to moderate in severity. The pain
can be related to swelling of the liver and concomitant stretching
of the liver capsule or distention of the veins, as is seen with portal
obstruction. This pain is carried via sympathetic fibers from the
celiac ganglion that enter the liver along with the hepatic artery and
vein. This type of liver pain responds poorly to adjuvant analgesics.
Occasionally, hepatic enlargement causes diaphragmatic irritation,
which produces pain that is referred to the ipsilateral supraclavicular
and shoulder region. This referred pain is known as Kehrs sign and
is transmitted via the phrenic nerve and is often misdiagnosed.
Referred liver pain is caused by mechanical irritation and
inflammation of the inferior pleura and peritoneum. This pain is
somatic and carried primarily by the lower intercostal and subcostal
nerves. This somatic pain is sharp and pleuritic and is moderate to
severe in intensity. It responds more favorably to nonsteroidal antiinflammatory drugs (NSAIDs) and opioid analgesics in contrast to
sympathetically mediated liver pain.

Testing for patients with liver pain should be aimed at identifying


the primary source of liver disease responsible for the pain and ruling
out other pathological processes that may be responsible for the pain.
Plain radiographs of the chest and abdomen, including an upright
abdominal film, are indicated in all patients with pain thought to be
emanating from the liver. Radiographs of the ribs are indicated to

Clavicle

Signs and Symptoms


The clinical presentation of liver pain is directly related to whether
the pain is mediated via the sympathetic or somatic nervous system or both. In patients with sympathetically mediated pain, the
abdominal examination may reveal hepatomegaly with tenderness
to palpation of the liver. Primary tumor or metastatic disease also
may be identified. The remainder of the abdominal examination
is nondescript. Auscultation over the liver fails to reveal a friction
rub in most cases. As mentioned, the patient may report ill-defined
pain in the supraclavicular region (Figure 72-1).
Patients with somatically mediated liver pain present in an
entirely different manner. The patient often splints the right lower
chest wall and abdomen and takes small, short breaths to avoid
exacerbating the pain. The patient may avoid coughing because of
the pain and accumulated upper airway secretions, and atelectasis
may be a problem. The abdominal examination may reveal signs

Liver

Figure 72-1 Patients with liver pain may report ill-defined pain in the
supraclavicular region.

209

210 SECTION 7 Abdominal and Groin Pain Syndromes

rule out occult bony pathological conditions, including tumor. Based


on the patients clinical presentation, additional tests, including
complete blood cell count, automated chemistries, liver function test,
erythrocyte sedimentation rate, and antinuclear antibody testing, may
be indicated. Computed tomography (CT) and magnetic resonance
imaging (MRI) of the lower thoracic contents and abdomen are
indicated in most patients with liver pain to rule out occult pulmonary and intra-abdominal pathological processes, including cancer of
the gallbladder and pancreas (Figures 72-2 and 72-3). Differential
neural blockade on an anatomical basis can serve as a diagnostic and
therapeutic maneuver (see discussion of treatment).

Differential Diagnosis
Pain of hepatic origin must be taken seriously. It is often the result
of an underlying serious disease, such as biliary malignancy, portal

hypertension, or hepatic metastatic disease. Pain emanating from


the liver is often mistaken for pain of cardiac or gallbladder origin
and can lead to visits to the emergency department and unnecessary cardiac and gastrointestinal workups. If trauma has occurred,
liver pain may coexist with rib fractures or fractures of the sternum
itself that can be missed on plain radiographs and may require
radionucleotide bone scanning for proper identification.
Neuropathic pain involving the chest wall may be confused or
coexist with liver pain. Examples of neuropathic pain include diabetic polyneuropathies and acute herpes zoster involving the lower
thoracic and upper lumbar nerves. The possibility of diseases of the
structures of the inferior mediastinum and retroperitoneum is ever
present, and these diseases sometimes can be difficult to diagnose.
Pathological processes that inflame the pleura, such as pulmonary
embolus, infection, and Bornholms disease, may mimic or coexist
with pain of hepatic origin.

Treatment
Initial treatment of liver pain should include a combination of simple analgesics and nonsteroidal anti-inflammatory drugs (NSAIDs)
or cyclooxygenase-2 (COX-2) inhibitors. If these medications do
not control the patients symptoms adequately, an opioid analgesic
may be added. Local application of heat and cold may be beneficial
to provide symptomatic relief of liver pain. The use of an elastic rib
belt over the liver may help provide symptomatic relief.
For patients who do not respond to these treatment modalities,
an intercostal nerve block using a local anesthetic and steroid may
be a reasonable next step. If the pain is thought to be sympathetically mediated, a celiac plexus block is a reasonable next step. This
technique provides diagnostic and therapeutic benefit. If the pain
is thought to be somatic, intercostal nerve blocks should be the
next step. Pain of hepatic origin may be somatic and sympathetic
and require celiac plexus and intercostal nerve block for complete
control.
Figure 72-2 Gallbladder carcinoma (small arrows) manifesting as thickening of the gallbladder wall with a gallstone (large arrow) and metastasis to
lymph nodes (n). (From Haaga JR, Lanzieri CF, Sartoris UJ, etal: Computed
tomography and magnetic resonance imaging of the whole body, 3rd ed,
St Louis, 1994, Mosby, p 1359.)

Complications and Pitfalls


The major problem in the care of patients thought to have liver
pain is the failure to identify potentially serious pathological processes of the thorax or upper abdomen. Given the proximity of
the pleural space, pneumothorax after intercostal nerve block is a
possibility. The incidence of the complication is less than 1%, but
it occurs with greater frequency in patients with chronic obstructive pulmonary disease. Although uncommon, infection, including liver abscess, remains an ever-present possibility, especially in
an immunocompromised patient with cancer. Early detection of
infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls

Figure 72-3 Axial precontrast T1-weighted magnetic resonance imaging


shows numerous bright metastases within the liver and vertebral body.
(From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors: Clinical magnetic
resonance imaging, 3rd ed, Philadelphia, 2006, Saunders, p 2572.)

Liver pain is often poorly diagnosed and treated. Correct


diagnosis of the cause of liver pain and the nerves subserving
the pain is necessary to treat this painful condition properly and to avoid overlooking serious intrathoracic or intraabdominal pathological processes. Intercostal nerve block
is a simple technique that can produce dramatic relief for
patients with liver pain thought to be somatically mediated.
Celiac plexus block is technically more demanding and
should be performed only by clinicians well versed in the
technique and potential complications.

72 Liver Pain 211


SUGGESTED READINGS
Goodman CC: Screening for medical problems in patients with upper extremity
signs and symptoms, J Hand Ther 23:105126, 2010.
Hansen L, Sasaki A, Zucker B: End-stage liver disease: challenges and practice
implications, Nurs Clin North Am 45:411426, 2010.
Khoury GF, Stein C, Ramming KP: Neck and shoulder pain associated with
hepatic arterial chemotherapy using an implantable infusion pump, Pain
32:275277, 1988.

Tsunekawa K, Matsuda R, Ohgushi N, Ogasawara A: Ohnishi: Basic problems of


the pain from the gallbladder and liver, Pain 30(Suppl 1):S24, 1987.
Waldman SW, Feldstein GS, Donohoe CD, Waldman KA: The relief of body
wall pain secondary to malignant hepatic metastases by intercostal nerve block
with bupivacaine and methylprednisolone, J Pain Symptom Manage 3:3943,
1988.

Chapter 73
ABDOMINAL ANGINA

ICD-9 CODE 557.1


ICD-10 CODE K55.1

vasculitis, a collagen-vascular workup is indicated in all patients


with abdominal angina. Computed tomography (CT) of the abdomen with oral and intravenous contrast material is indicated if an

The Clinical Syndrome


Abdominal angina is an uncommon cause of intermittent
abdominal pain. Patients with abdominal angina report severe
cramping abdominal pain that begins 15 to 30 minutes after
eating (Figure 73-1). This postprandial pain persists for 2 to 3
hours. Additional ingestion of food aggravates the patients pain,
forcing the patient to stop eating. Weight loss is common. As
the disease progresses, malabsorption and diarrhea occur as a
result of mucosal and mural injury, which further exacerbates the
patients weight loss.
The cause of abdominal angina is arterial vascular insufficiency.
The term angina is used because the pain occurs only after eating,
when the insufficient fixed arterial supply is unable to meet the
increased demands needed to support digestion. The most common cause of abdominal angina is stenosis of the celiac artery with
inadequate collateralization. Aneurysms of the superior mesenteric
artery, the vasculitides, fibromuscular hyperplasia, and tumor
encroachment on the celiac artery also have been implicated as
causes of abdominal angina.

Signs and Symptoms


Physical examination of a patient with abdominal angina reveals
diffuse abdominal tenderness. Mild abdominal distention may be
present. Signs of acute peritoneal irritation suggestive of perforated viscus, such as rebound tenderness, are absent. The patient
may exhibit frequent defecation of mucoid stools, diarrhea, and
vomiting. The patient appears systemically ill, but not septic.

15 to 30 minutes
after eating

Testing
The diagnosis of abdominal angina is based on clinical history.
Angiography of the celiac artery provides proof of vascular insufficiency and often identifies the cause of the problem. Barium
enema shows the classic finding of thumbprinting that is strongly
suggestive of mucosal ischemia (Figure 73-2). Colonoscopy reveals
localized hemorrhage and ulceration of the affected mucosa. Based
on the patients clinical presentation, additional tests, including
complete blood cell count, erythrocyte sedimentation rate, and
stool and blood cultures for infectious enteritis, may be indicated.
Given the possibility that the patients abdominal angina is due to
212

Figure 73-1 Abdominal angina is an uncommon cause of intermittent


abdominal pain. Patients with abdominal angina report severe cramping
abdominal pain that begins 15 to 30 minutes after eating.

73 Abdominal Angina 213

A
Figure 73-2 Thumbprinting in acute ischemic colitis at the splenic flexure. (From Grainger RG, Allison D: Grainger and Allisons diagnostic radiology: a textbook of medical imaging, 3rd ed, New York, 1997, Churchill
Livingstone, p 1036.)

B
Figure 73-4 Ischaemic colitis. A, Longitudinal view shows thickened
descending colon with absence of blood flow. The mural stratification is
maintained. B, Transverse view shows diffuse, poorly reflective thickening (arrow), loss of the mural stratification and absence of blood flow.
Focal area of pneumatosis is seen (arrow) with edema of the paracolic
fat and ascites (arrowhead). (From Allen PL, Baxter GM, Weston MJ: Clinical ultrasound, vol 1, ed 3, New York, 2011, Churchill Livingstone, p 402.)

Figure 73-3 Severe chronic mesenteric ischemia secondary to superior


mesenteric artery occlusion and a high-grade celiac stenosis (arrow)
depicted by contrast-enhanced magnetic resonance angiography (left).
Anterior projection shows a large inferior mesenteric artery (arrow) that
supplies the entire abdomen via large mesenteric and retroperitoneal
collaterals (right). (From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2006,
Saunders, p 805.)

included in the differential diagnosis. Other causes of abdominal


pain, including diverticulitis, bowel obstruction, and appendicitis,
may occur in conjunction with abdominal angina.

Treatment

occult mass or abscess is suspected. Magnetic resonance angiography (MRA) of the celiac and mesenteric vessels also can help clarify
the diagnosis and aid in planning a treatment strategy, as can ultrasonogrpahic and Doppler flow studies (Figures 73-3 and 73-4).

The only definitive treatment of abdominal angina is correction


of the arterial insufficiency via either angioplasty or surgical revascularization. Careful attention to the patients fluid and metabolic
status is crucial to avoid complications. Anticholinergics such
as dicyclomine and antiperistaltics such as loperamide can help
decrease diarrhea. Small, frequent feedings also may help palliate
the postprandial pain.

Differential Diagnosis

Complications and Pitfalls

Any disease process that can produce ischemic bowel can mimic
the pain of abdominal angina. The vasculitides, including polyarteritis nodosum and Henoch-Schnlein purpura, also can cause
the symptoms of abdominal angina. Embolic disease that may
cause occlusion of the vascular supply to the gut also should be
considered. The possibility of infectious enteritis always must be

The potential for complications in patients with abdominal


angina is high. Spontaneous bowel perforation, stenosis, fistula
formation, bleeding, and malabsorption occur with sufficient
frequency to complicate the management of this painful condition. Untreated, abdominal angina frequently progresses to bowel
infarction.

214 SECTION 7 Abdominal and Groin Pain Syndromes

Clinical Pearls
Treatment of the symptoms associated with abdominal
angina is difficult, and, ultimately, correction of the vascular insufficiency is required. Vigilance for life-threatening
complications of abdominal angina, including bowel infarction, is mandatory to avoid disaster.

SUGGESTED READINGS
Cho JS, Carr JA, Jacobsen G, etal: Long-term outcome after mesenteric artery
reconstruction: a 37-year experience, J Vasc Surg 35:453460, 2002.
Cognet F, Ben Salem D, Dranssart M, etal: Chronic mesenteric ischemia: imaging
and percutaneous treatment, Radiographics 22:863879, 2002.
Hamed RMA, Ghandour K: Abdominal angina and intestinal gangrene: a catastrophic presentation of arterial fibromuscular dysplasiacase report and
review of the literature, J Pediatr Surg 32:13791380, 1997.
Rha SE, Ha HK, Lee SH, etal: CT and MR imaging findings of bowel ischemia
from various primary causes, RadioGraphics 20:2942, 2000.

SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes

Chapter 74
EPIDURAL ABSCESS
ICD-9 CODE 324.1
ICD-10 CODE G06.1
The Clinical Syndrome
Epidural abscess is an uncommon cause of spine pain that, if undiagnosed, can result in paralysis and life-threatening complications.
Epidural abscess can occur anywhere in the spine and intracranially.
It can occur spontaneously via hematogeneous seeding, most frequently as a result of urinary tract infections that spread to the spinal
epidural space via Batsons plexus. More commonly, epidural abscess
occurs after instrumentation of the spine, including surgery and epidural nerve blocks. The literature suggests that the administration of
steroids into the epidural space results in immunosuppression, with
a resultant increase in the incidence of epidural abscess. Although
theoretically plausible, the statistical evidencegiven the thousands
of epidural steroid injections performed around the United States on
a daily basiscalls this belief into question.
A patient with epidural abscess initially presents with ill-defined
pain in the segment of the spine affected (e.g., cervical, thoracic,
or lumbar) (Figure 74-1). This pain becomes more intense and
localized as the abscess increases in size and compresses neural
structures. Low-grade fever and vague constitutional symptoms,
including malaise and anorexia, progress to frank sepsis with
a high-grade fever, rigors, and chills. At this point, the patient
begins to experience sensory and motor deficits and bowel and
bladder symptoms as the result of neural compromise. As the
abscess continues to expand, compromise of the vascular supply to
the affected spinal cord and nerve occurs with resultant ischemia
and, if untreated, infarction and permanent neurological deficits.

Signs and Symptoms


A patient with epidural abscess initially has ill-defined pain in the
general area of the infection. At this point, mild pain may occur on
range of motion of the affected segments. The neurological examination is within normal limits. A low-grade fever, night sweats,
or both may be present. Theoretically, if the patient has received
steroids, these constitutional symptoms may be attenuated or their

onset may be delayed. As the abscess increases in size, the patient


appears acutely ill, with fever, rigors, and chills. The clinician
may be able to identify neurological findings suggestive of spinal
nerve root compression, spinal cord compression, or both. Subtle
findings that point toward the development of myelopathy (e.g.,
Babinskis sign, clonus, and decreased perineal sensation) may be
overlooked if not carefully sought. As compression of the involved
neural structures continues, the patients neurological status may
deteriorate rapidly. If the diagnosis is not made, irreversible motor
and sensory deficit occurs.

Testing
Myelography is still considered the best test to ascertain compromise of the spinal cord and exiting nerve roots by an extrinsic
mass such as an epidural abscess. In this era of readily available
magnetic resonance imaging (MRI) and high-speed computed
tomography (CT), it may be more prudent to perform this noninvasive testing first, rather than wait for a radiologist or spine
surgeon to perform a myelogram (Figure 74-2). MRI and CT are
highly accurate in the diagnosis of epidural abscess and are probably more accurate than myelography in the diagnosis of intrinsic
disease of the spinal cord and spinal tumor. All patients suspected
to have epidural abscess should undergo laboratory testing consisting of complete blood cell count, erythrocyte sedimentation
rate, and automated blood chemistries. Blood and urine cultures
should be performed immediately in all patients thought to have
epidural abscess to allow immediate implementation of antibiotic
therapy while the workup is in progress. Gram stains and cultures
of the abscess material also should be performed, but antibiotic
treatment should not be delayed waiting for this information.

Differential Diagnosis
The diagnosis of epidural abscess should be strongly considered
in any patient with spine pain and fever, especially if the patient
has undergone spinal instrumentation or epidural nerve blocks for
either surgical anesthesia or pain control. Other pathological processes that must be considered in the differential diagnosis include
intrinsic disease of the spinal cord, such as demyelinating disease
and syringomyelia, and other processes that can result in compression of the spinal cord and exiting nerve roots, such as metastatic
215

216 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes


Lumbar
vertebrae

Spinal cord
Cauda equina
Dura mater

Epidural
space
Abscess

Figure 74-1 Patients with epidural abscess initially present with ill-defined pain in the affected segment of the spine.

tumor, Pagets disease, and neurofibromatosis. As a general rule,


unless the patient has concomitant infection, these diseases are
routinely associated with only back pain and not with fever.

Treatment
The rapid initiation of treatment of epidural abscess is mandatory if the patient is to avoid the sequelae of permanent neurological deficit or death. The treatment of epidural abscess has
two goals: (1) treatment of the infection with antibiotics and (2)
drainage of the abscess to relieve compression on neural structures. Because most epidural abscesses are caused by Staphylococcus
aureus, antibiotics such as vancomycin that treat staphylococcal
infection should be started immediately after blood and urine culture samples are taken. Antibiotic therapy can be tailored to the
culture and sensitivity reports as they become available. As mentioned, antibiotic therapy should not be delayed while waiting for
definitive diagnosis if epidural abscess is being considered as part
of the differential diagnosis.
Antibiotics alone rarely treat an epidural abscess successfully
unless the diagnosis is made very early in the course of the disease;
drainage of the abscess is required to effect full recovery. Drainage
of the epidural abscess is usually accomplished via decompression
laminectomy and evacuation of the abscess. More recently, interventional radiologists have been successful in draining epidural
abscesses percutaneously using drainage catheters placed with the

use of CT or MRI guidance. Serial CT or MRI scans are useful


in following the resolution of epidural abscess; scans should be
repeated immediately at the first sign of negative change in the
patients neurological status.

Complications and Pitfalls


Failure to diagnose and treat epidural abscess rapidly and accurately can result in disaster for the clinician and patient alike. The
insidious onset of neurological deficit associated with epidural
abscess can lull the clinician into a sense of false security that can
result in permanent neurological damage to the patient. If epidural abscess or other causes of spinal cord compression is suspected,
the algorithm shown in Table 74-1 should be followed.

Clinical Pearls
Delay in diagnosis puts the patient and clinician at tremendous risk for a poor outcome. The clinician should assume
that all patients who present with fever and back pain have
an epidural abscess until proved otherwise and should treat
accordingly. Overreliance on a single negative or equivocal imaging test is a mistake. Serial CT or MRI scans are
indicated should there be any deterioration in the patients
neurological status.

74 Epidural Abscess 217

Figure 74-2 Sagittal (A and B) and axial (C and D) T2-weighted magnetic resonance imaging (MRI) of discitis at the LF-S1 disk level showing highsignal-intensity fluid within the disk. High-signal-intensity fluid collections (white arrows) are seen in the epidural space, consistent with abscesses. The
sagittal postcontrast T1-weighted MRI with fat saturation (E and F) show the abscesses as low-signal-intensity areas with only peripheral enhancement
(broken white arrows). (In Waldman SD, Campbell RSD, editors: Imaging of pain, Philadelphia, 2011, Saunders, p 152.)

TABLE 74-1

Algorithm for Evaluation of Spinal Cord Compression Caused by Epidural Abscess


Immediately obtain blood and urine samples for cultures.
Immediately start high-dose antibiotics that cover Staphylococcus aureus.
Immediately order the most readily available spinal imaging technique (computed tomography, magnetic resonance imaging, myelography)
that can confirm the presence of spinal cord compression (e.g., abscess, tumor).
Simultaneously obtain emergency consultation from a spine surgeon.
Continuously and carefully monitor patients neurological status.
If any of the measures listed here are unavailable, arrange emergency transfer of patient to tertiary care center by the most rapidly available
transportation.
Repeat imaging, and obtain repeat surgical consultation if any deterioration occurs in the patients neurological status.

218 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes


SUGGESTED READINGS
Bandikatla VB, Rizwan B, Skalimis A, Patel H: Spinal epidural abscess and meningitis following an epidural catheterisation, Acute Pain 9:3538, 2007.
Esteves Pereira C, Lynch JC: Spinal epidural abscess: an analysis of 24 cases, Surg
Neurol 63(Suppl 1):S26S29, 2005.

Recinos PF, Pradilla G, Crompton P, Thai Q-A, Rigamonti D: Spinal epidural


abscess: diagnosis and treatment, Oper Techn Neurosurg 7:188192, 2004.
Rigamonti D, Liem L, Sampath P, et al: Spinal epidural abscess: contemporary
trends in etiology, evaluation, and management, Surg Neurol 52:189197, 1999.

Chapter 75
MULTIPLE MYELOMA

ICD-9 CODE 203.0


ICD-10 CODE C90.00
The Clinical Syndrome
Multiple myeloma is an uncommon cause of back pain that is
frequently initially misdiagnosed. It is a unique disease in that it
may cause pain via several mechanisms that can act alone or in
concert. These mechanisms include invasion or compression of
pain-sensitive structures (1) by the tumor itself, (2) by the products that the tumor produces, and (3) by the host response to the
tumor and its products.
Although the exact cause of multiple myeloma is unknown, the
following facts have been elucidated. There seems to be a genetic
predisposition to the development of myeloma. It also is known
that exposure to radiation increases the incidence of the disease, as
witnessed in survivors of the nuclear bombs used in World War II.
RNA viruses also have been implicated in the evolution of multiple
myeloma. The disease is rare in individuals younger than 40 years,
with a median age of diagnosis of 60 years. A male gender predilection is seen, and blacks have twice the incidence of multiple myeloma
than whites. Worldwide, the incidence of multiple myeloma is 3 per
100,000 population.
The most common clinical presentation of multiple myeloma
is back and rib pain. It occurs in more than 70% of patients ultimately diagnosed with the disease. These bone lesions are osteolytic and are best diagnosed with plain radiography rather than
with radionucleotide bone scanning. Pain with movement is common, and hypercalcemia occurs with sufficient frequency to be the
presenting symptom in many patients with multiple myeloma.
Life-threatening infection, anemia, bleeding, and renal failure are
often present in conjunction with the symptoms of pain. Hyperviscosity of the serum that is the result of the products of tumor
production may lead to cerebrovascular accidents.

secondary to hypercalcemia also may be elicited. Anasarca resulting from renal failure, if present, is an ominous prognostic sign.

Testing
The presence of Bence Jones protein in the urine, anemia, and
increased M protein on serum protein electrophoresis point

Rib pain

Spine pain

Signs and Symptoms


Pain is the most common clinical symptom that ultimately leads
the clinician to the diagnosis of multiple myeloma (Figure 75-1).
Seemingly minor trauma may cause pathological vertebral compression or rib fractures. Pain on movement of the affected bones
is a common finding on physical examination, as is the finding of
tumor mass on palpation of the skull and other affected bones.
Neurological findings, either based on neural compression secondary to tumor or fracture or as a result of cerebrovascular accident, are often present. Positive Trousseaus and Chvosteks signs

Figure 75-1 Pain is the most common clinical symptom that ultimately
leads to the diagnosis of multiple myeloma.

219

220 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes

Figure 75-2 Elderly patient with low back pain. Anteroposterior (A) and lateral (B) radiographs show a presumed insufficiency fracture of
L2 with minor end-plate collapse of L3. The sagittal T1-weighted (C), T2-weighted (D), and short tau inversion recovery (STIR) (E) magnetic
resonance images acquired a few months later show multilevel vertebral fractures. Diffuse abnormalities of the bone marrow are seen, with
a generally patchy appearance and some rounded areas of high signal intensity on the T2-weighted and STIR images. The appearances are
strongly suspicious for disorders such as plasma cell dyscrasias and other reticuloendothelial disorders. Immunoglobin testing yielded results
positive for myeloma, and a subsequent skeletal survey showed lytic lesions in the skull (F) typical of multiple myeloma. (In Waldman SD,
Campbell RSD, editors: Imaging of pain, Philadelphia, 2011, Saunders, p 192.)

strongly to the diagnosis of multiple myeloma. Classic punched-out


bone lesions in the skull and spine on plain radiographs are pathognomonic for the disease (Figures 75-2 and 75-3). Because little
osteoclastic activity is present in patients with multiple myeloma,
radionucleotide bone scanning can be negative in the face of diffuse

bony destruction. Magnetic resonance imaging (MRI) is indicated


in any patient thought to have multiple myeloma who exhibits
signs of spinal cord compression. Serum creatine testing and automated blood chemistries that include serum calcium determinations are indicated in all patients with multiple myeloma.

75 Multiple Myeloma 221

Differential Diagnosis

Complications and Pitfalls

A variety of other abnormalities of the bone marrow, including the


heavy chain diseases and Waldenstrms macroglobulinemia, can
mimic the clinical presentation of multiple myeloma. Amyloidosis
also shares many common clinical signs and symptoms. Metastatic
disease from prostate and breast cancer can produce pathological
fractures of the spine and ribs and calvarial metastases that may be
mistaken for multiple myeloma. A small group of patients have
benign monoclonal gammopathy, which in most patients requires
no therapy but can mimic the laboratory findings of multiple
myeloma.

Approximately 15% of patients with multiple myeloma die within


the first 3 months after diagnosis despite aggressive treatment. An
additional 15% of patients die each successive year. The most
common causes of death are renal failure, overwhelming sepsis,
hypercalcemia, bleeding, the development of acute leukemia,
and stroke. Nonfatal complications such as pathological fractures
can make life for patients with multiple myeloma quite difficult.
Failure to recognize and treat these complications of multiple
myeloma early can increase patient suffering and hasten death.

Treatment

Clinical Pearls

Management of multiple myeloma is aimed at the treatment of


progressive bone lesions and reduction in serum myeloma proteins. These goals are accomplished with radiation therapy and
chemotherapy, alone or in combination. High-dose pulsed steroids
have been shown to provide symptomatic relief and to extend life
expectancy in patients with multiple myeloma.
Initial treatment of the pain associated with multiple myeloma
should include either nonsteroidal anti-inflammatory drugs
(NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. It may be
necessary to add opioid analgesics to control the more severe pain
of pathological fractures. Orthotic devices such as the Cash brace
and rib belts may help stabilize the spine and ribs and should be
considered in the presence of pathological fractures. Local application of heat and cold may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients
who do not respond to these treatment modalities, injection of
the affected areas with a local anesthetic and steroid using either
intercostal or epidural nerve blocks is a reasonable next step. Spinal administration of opioids may be beneficial in selected cases.
Ultimately, radiation therapy is often required if significant bony
involvement occurs, to provide adequate pain control. Stem cell
transplant and gene therapy are emerging as exciting new options
in treatment of this disease.

Careful evaluation of patients with the triad of proteinuria,


spine or rib pain, and abnormal serum protein electrophoresis is mandatory to help avoid the inevitable complications of a delayed diagnosis of multiple myeloma. The
clinician and patient must understand that despite early
treatment, most patients with multiple myeloma die within
2 to 5 years of the time the disease is diagnosed. Epidural
and intercostal injection of local anesthetics and steroids
can provide excellent palliation of the pain associated with
multiple myeloma.

SUGGESTED READINGS
Kaufman J, Lonial S: Multiple myeloma: the role of transplant and novel treatment
strategies, Semin Oncol 31(Suppl 4):99105, 2004.
Mahindra A, Hideshima T, Anderson KC: Multiple myeloma: biology of the
disease, Blood Rev 24(Suppl 1):S5S11, 2010.
Mitsiades CS, Hayden PJ, Anderson KC, Richardson PG: From the bench to the
bedside: emerging new treatments in multiple myeloma, Best Pract Res Clin
Haematol 20:797816, 2007.
Reece DE: Management of multiple myeloma: the changing landscape, Blood Rev
21:301314, 2007.
San Miguel JF, Gutirrez NC, Mateo G, Orfao A: Conventional diagnostics in
multiple myeloma, Eur J Cancer 42:15101519, 2006.

Chapter 76
PAGETS DISEASE

ICD-9 CODE 731.1


ICD-10 CODE M90.60
The Clinical Syndrome
Pagets disease is an uncommon cause of back pain that is frequently
diagnosed on plain radiographs obtained for other purposes or
when the patient notices swelling of a long bone. Pagets disease is
also known as osteitis deformans, and its cause is unknown. The
incidence of Pagets disease is approximately 2%, with the disease
occurring much less commonly in India, Japan, the Middle East,
and Scandinavia.
In the early phase of the disease, resorption of bone occurs, and
the affected areas become vascular. The resorption phase is followed
by the formation of new pagetic bone that is laid down in a dense,
haphazard fashion. This process of bone resorption and formation
can be quite active, with the bone turnover rate increased 20 times
above normal. This process results in a characteristic pattern on
plain radiographs that includes areas of bone resorption termed
osteoporosis circumscripta. Areas of new bone formation show an
irregularly widened cortex, with a dense, striated pattern and focal
variations in density that reflect the chaotic nature in which the
pagetic bone is laid down.
Although many patients with Pagets disease are asymptomatic, and their disease is a fortuitous finding when radiographs are
obtained for other reasons, back pain also may occur. It is thought
that the cause of the back pain associated with Pagets disease is
multifactorial. The pain may be caused by the bone resorption
process itself or distortion of the facet joints as new pagetic bone is
formed. Both of these processes may alter the functional stability
of the spine and exacerbate preexisting facet arthropathy.
Patients with Pagets disease may experience thickening and
widening of the long bones or enlargement of the skull resulting
from new bone formation. Rarely, exuberant bone growth at the
base of the skull may cause compression of the brainstem, with
disastrous results. Hearing loss secondary to compression of the
eighth cranial nerve by new bone formation or by direct involvement of the ossicles themselves may occur. Occasionally, excessive bone formation in the dorsal spine may result in spinal cord
compression, which, if untreated, may result in paraplegia. Pathological fractures resulting from excessive resorption of the vertebra
may occur, with resultant acute back pain. Hip pain secondary to
calcific periarthritis also may be present. An increased incidence of
222

renal calculi and gout may occur, especially in men with Pagets
disease. In less than 1% of patients, a pagetic bone lesion may
transform into a malignant osteosarcoma.

Signs and Symptoms


Although the disease is often asymptomatic, pain is the most common clinical symptom that ultimately leads the clinician to the
diagnosis of Pagets disease (Figure 76-1). Seemingly minor trauma
may cause pathological vertebral compression fractures. Pain on
movement of the affected bones is a common finding on physical
examination, as is excess bone growth on palpation of the skull and
other affected bones. Neurological findings based on neural compression secondary to either excessive bone growth or pathological
fracture may be present. Pain on range of motion of the peripheral
joints, especially the hip, owing to calcific periarthritis is a common
physical finding in patients with Pagets disease. Hearing loss may
be noted on physical examination. In rare patients with Pagets
disease, high-output cardiac failure resulting from increased blood
flow to new bone may be present.

Testing
As mentioned previously, Pagets disease is often fortuitously
diagnosed when the patient is undergoing radiographic testing
for an unrelated problem, such as intravenous pyelography for
renal calculi. The classic radiographic appearance of areas of bone
resorption with surrounding areas of dense, chaotic bone points
strongly to the diagnosis of Pagets disease. In patients with Pagets
disease, radionucleotide bone scanning can be used to assess the
extent of the disease because many bone lesions are clinically silent
(Figure 76-2). Magnetic resonance imaging (MRI) is indicated in
any patient thought to have Pagets disease who exhibits signs of
spinal cord compression. Serum creatine testing and automated
blood chemistries, including serum calcium determinations, are
indicated in all patients with Pagets disease. Alkaline phosphatase
levels are elevated, especially during the resorption phase of the
disease. Given the increased incidence of hearing loss in patients
with Pagets disease, audiometric testing is indicated.

Differential Diagnosis
Numerous other diseases of the bone, including osteoporosis, multiple myeloma, osteopetrosis, and primary and metastatic bone
tumors, can mimic the clinical presentation of Pagets disease.
Acromegaly also shares many common clinical signs and symptoms.
Metastatic disease from prostate and breast cancer can produce

76 Pagets Disease 223

Spine pain

Thickening of
long bones

Figure 76-2 Whole-body bone scan patterns in a patient with elevated


serum alkaline phosphatase levels and Pagets disease in multiple bones.
(From Grainger RG, Allison D: Grainger and Allisons diagnostic radiology: a textbook of medical imaging, ed 3, New York, 1997, Churchill
Livingstone, p 1927.)

pathological fractures of the spine and ribs and calvarial metastases


that may be mistaken for Pagets disease.

respond to these treatment modalities, the injection of the affected


areas with a local anesthetic and steroid using either intercostal or
epidural nerve blocks is a reasonable next step. Spinal administration of opioids may be beneficial in selected cases.
In patients who fail to respond to these treatments, calcitonin and etidronate have been used with some degree of success.
Cytotoxic drugs, including dactinomycin, may rarely be required
if excessive bone destruction occurs. High-dose pulsed steroids
have been shown to provide symptomatic relief in patients with
Pagets disease.

Treatment

Complications and Pitfalls

Most patients with asymptomatic Pagets disease require only


reassurance. Initial treatment of the pain associated with Pagets
disease should include aspirin, nonsteroidal anti-inflammatory
drugs (NSAIDs), or cyclooxygenase-2 (COX-2) inhibitors. Opioid analgesics may be needed to control the more severe pain of
pathological fractures. Orthotic devices such as the Cash brace
and rib belts may help stabilize the spine and ribs and should be
considered if pathological fractures occur. Local application of
heat and cold may be beneficial. The repetitive movements that
incite the syndrome should be avoided. For patients who do not

The primary complications associated with Pagets disease are


related to the resorptive and formation phases of the disease. Excessive resorption of bone may result in vertebral compression fractures, rib fractures, and occasionally fractures of the long bones.
Excessive bone formation may result in compression of neural
structures, which may result in hearing loss, brainstem compression, myelopathy, and paraplegia. An increased incidence of renal
calculi and gout is seen, especially in men with Pagets disease.
Rarely, the formation of new bone is so great that high-output
cardiac failure secondary to increased blood flow may result. As

Figure 76-1 Although the disease is often asymptomatic, pain is the


most common clinical symptom that ultimately leads to the diagnosis
of Pagets disease.

224 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes

mentioned earlier, pagetic lesions undergo malignant transformation in approximately 1% of patients with Pagets disease.

Clinical Pearls
Careful evaluation of patients with Pagets disease is mandatory to help avoid the potential complications of the disease.
The clinician must look carefully for subtle signs of brainstem or spinal cord compression. Epidural and intercostal
injection of local anesthetics and steroids can provide excellent palliation of the pain associated with Pagets disease
that fails to respond to pharmacological treatment.

SUGGESTED READINGS
Ralston SH: Pathogenesis of Pagets disease of bone, Bone 43:819825, 2008.
Rousire M, Michou L, Cornlis F, Orcel P: Pagets disease of bone, Best Pract Res
Clin Rheumatol 17:10191041, 2003.
Rousire M, Michou L, Cornlis F, Orcel P: Pagets disease. In Waldman SD,
Campbell RSD, editors: Imaging of pain, Philadelphia, 2010, Saunders.
Walsh JP, Attewell R, Stuckey BGA, et al: Eisman treatment of Pagets disease
of bone: a survey of clinical practice in Australia, Bone 42:12191225, 2008.
Whitten CR, Saifuddin A: MRI of Pagets disease of bone, Clin Radiol 58:
763769, 2003.

Chapter 77
DIFFUSE IDIOPATHIC SKELETAL
HYPEROSTOSIS
ICD-9 CODE 733.99
ICD-10 CODE M89.30
The Clinical Syndrome
Diffuse idiopathic skeletal hyperostosis (DISH) is a disease of
the ligamentous structures of the spine. The cause of DISH is
unknown. The hallmark of this disease is confluent ossification of
the spinal ligamentous structures that spans at least three spinal
interspaces (Table 77-1). DISH occurs most commonly in the
thoracolumbar spine, but it also can affect the cervical spine, ribs,
and bones of the pelvis.
DISH causes stiffness and pain of the cervical and thoracolumbar spine. The symptoms are worse on wakening and at night.
When the disease affects the cervical spine, cervical myelopathy
may result. If anterior spurring of the cervical spine occurs, dysphagia may result. DISH is a disease of the late fifth and early
sixth decades. It also can cause a relative spinal stenosis with
intermittent claudication. It affects men twice as commonly as
women. DISH is a disease that affects primarily whites. Patients
with DISH have a higher incidence of diabetes mellitus, hypertension, and obesity than the general population. DISH usually is
diagnosed by plain radiographs of the spine.

Signs and Symptoms


A patient with DISH reports stiffness and pain in the area of
the affected spinal segments or bone. Patients also may note

TABLE 77-1

Causes of Abnormal Bone Growth in and About the


Axial Skeleton
Seronegative spondyloathropathies
Acromegaly
Charcot neuroarthropathy
Trauma
Degenerative changes
Diffuse idiopathic skeletal hyperostosis
Abnormal urate deposition
Excessive fluoride intake
Onchronosis

numbness, weakness, and lack of coordination in the extremities subserved by the spinal segments affected by DISH. Muscle
spasms, back pain, and pain referred to the buttocks are common
(Figure 77-1). Occasionally, a patient with DISH experiences
compression of the spinal cord, nerve roots, and cauda equina,
resulting in myelopathy or cauda equina syndrome. DISH is the
second most common cause of cervical myelopathy after cervical
spondylosis. Patients with lumbar myelopathy or cauda equina
syndrome experience varying degrees of lower extremity weakness
and bowel and bladder symptoms; this represents a neurosurgical
emergency and should be treated as such.

Testing
DISH is diagnosed by plain radiographs. Confluent ossification of
the spinal ligamentous structures spanning at least three interspaces
is pathognomonic for the disease. Disk space height is preserved
in patients with DISH. If myelopathy is suspected, magnetic resonance imaging (MRI) of the spine provides the best information
regarding the status of the spinal cord and nerve roots. MRI is
highly accurate and helps identify other abnormalities that may
put the patient at risk for the development of permanent spinal
cord injury (Figure 77-2). In patients who cannot undergo MRI,
such as a patient with a pacemaker, computed tomography (CT)
or myelography is a reasonable second choice. Radionucleotide
bone scanning and plain radiographs are indicated if fracture or
bony abnormality, such as metastatic disease, is being considered.
Although this testing provides useful neuroanatomical information, electromyography and nerve conduction velocity testing provide neurophysiological information that can delineate
the actual status of each individual nerve root and the lumbar
plexus. Screening laboratory tests, consisting of complete blood
cell count, erythrocyte sedimentation rate, and automated blood
chemistry testing, should be performed if the diagnosis of DISH
is in question.

Differential Diagnosis
DISH is a radiographic diagnosis that is supported by a combination of clinical history, physical examination, and MRI. Pain syndromes that may mimic DISH include neck and low back strain;
bursitis; fibromyositis; inflammatory arthritis; ankylosing spondylitis; and disorders of the spinal cord, roots, plexus, and nerves.
Thirty percent of patients with multiple myeloma or Pagets
disease also have DISH. Screening laboratory tests consisting of
complete blood cell count, erythrocyte sedimentation rate, antinuclear antibody testing, human leukocyte antigen (HLA) B-27
antigen screening, and automated blood chemistry testing should
225

226 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes

Figure 77-2 Ankylosing spondylitis. Sagittal T1-weighted (600/20/4)


magnetic resonance imaging of the upper lumbar spine, including the
lower thoracic segments, also shows the characteristic squaring of the
vertebral body corners. (From Stark DD, Bradley WG Jr: Magnetic resonance imaging, ed 3, St Louis, 1999, Mosby, p 1877.)
Figure 77-1 Patients with DISH report stiffness and pain in the area of
the affected spinal segments or bone. They also may note numbness,
weakness, and lack of coordination in the extremities subserved by the
spinal segments affected by DISH. Muscle spasms, back pain, and pain
referred to the buttocks are common.

be performed if the diagnosis of DISH is in question to help rule


out other causes of the patients pain.

Treatment
DISH is best treated with a multimodality approach. Physical
therapy, including heat modalities, range-of-motion exercises,
and deep sedative massage, combined with nonsteroidal antiinflammatory drugs (NSAIDs) and skeletal muscle relaxants
represents a reasonable starting point. The addition of steroid
epidural nerve blocks is a reasonable next step if pain remains a
problem. Underlying sleep disturbance and depression are best
treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls


Failure to diagnose DISH accurately may put the patient at risk for
the development of myelopathy, which, if untreated, may progress

to paraparesis or paraplegia. Electromyography helps distinguish


between plexopathy from radiculopathy and helps identify coexistent entrapment neuropathy, which may confuse the diagnosis.

Clinical Pearls
Given the association of DISH with multiple myeloma and
Pagets disease, these potentially life-threatening diseases
must always be included in the differential diagnosis. DISH
and degenerative arthritis and discogenic disease may coexist.
Each disease process may require its own specific course of
treatment.

SUGGESTED READINGS
Hannallah D, White AP, Goldberg G, Albert TJ: Diffuse idiopathic skeletal
hyperostosis, Operat Techn Orthop 17:174177, 2007.
Kasper D, Hermichen H, Koster R, Schultz-Coulon HJ: Clinical manifestations
of diffuse idiopathic skeletal hyperostosis (DISH), HNO 50:978983, 2002.
Mader R: Diffuse idiopathic skeletal hyperostosis: a distinct clinical entity, Isr Med
Assoc J 5:506508, 2003.
Mader R: Current therapeutic options in the management of diffuse idiopathic
skeletal hyperostosis, Exp Opin Pharmacother 6:13131318, 2005.

Chapter 78
SPONDYLOLISTHESIS

ICD-9 CODE 756.12


ICD-10 CODE Q76.2
The Clinical Syndrome
Spondylolisthesis is a degenerative disease of the lumbar spine that
results in pain and functional disability. It occurs more commonly
in women and is most often seen after age 40. This disease is caused
by the slippage of one vertebral body onto another as a result of
degeneration of the facet joints and intervertebral disk. Usually,
the upper vertebral body moves anteriorly relative to the vertebral
body below it, which causes narrowing of the spinal canal. This
narrowing results in a relative spinal stenosis and back pain. Occasionally, the upper vertebral body slides posteriorly relative to the
vertebral body below it, which compromises the neural foramina.
Clinically, a patient with spondylolisthesis reports back pain
with lifting, twisting, or bending of the lumbar spine. Patients
may state that they feels like they have a catch in their back.
Patients with spondylolisthesis often report radicular pain of the
lower extremity and often experience pseudoclaudication with
walking. Rarely, the slippage of the vertebra is so extreme that
myelopathy or cauda equina syndrome develops.

imaging (MRI) of the lumbar spine provides the best information


regarding the contents of the lumbar spine (Figure 78-3). MRI is
highly accurate and helps identify abnormalities that may put the
patient at risk for the development of lumbar myelopathy, such as
the trefoil spinal canal of congenital spinal stenosis (Figure 78-4).
In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) and myelography are reasonable second choices. Radionucleotide bone scanning and plain
radiographs are indicated if fracture or bony abnormality, such as
metastatic disease, is being considered.
Although this testing provides useful neuroanatomical information, electromyography and nerve conduction velocity testing
provide neurophysiological information that can delineate the
actual status of each individual nerve root and the lumbar plexus.
Screening laboratory tests, consisting of complete blood cell count,
erythrocyte sedimentation rate, and automated blood chemistry

Signs and Symptoms


Patients with spondylolisthesis report back pain with motion of
the lumbar spine. Rising from a sitting to a standing position often
reproduces the pain (Figure. 78-1). Many patients with spondylolisthesis experience radicular symptoms that manifest on physical
examination as weakness and sensory abnormality in the affected
dermatomes. Often, more than one dermatome is affected. Occasionally, a patient with spondylolisthesis experiences compression
of the lumbar spinal nerve roots and cauda equina, resulting in
myelopathy or cauda equina syndrome. Lumbar myelopathy is
most commonly due to midline herniated lumbar disk, spinal stenosis, tumor, or, rarely, infection. Patients with lumbar myelopathy or cauda equina syndrome experience varying degrees of lower
extremity weakness and bowel and bladder symptoms; this represents a neurosurgical emergency and should be treated as such.

Testing
Plain radiographs of the lumbar spine usually are sufficient to
diagnose spondylolisthesis (Figure 78-2). The lateral view shows
the slippage of one vertebra onto another. Magnetic resonance

Figure 78-1 Patients with spondylolisthesis often report back pain with
motion of the lumbar spine. Rising from a sitting to a standing position
often reproduces the pain.

227

228 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes

L4

Figure 78-2 Type 2a isthmic spondylolisthesis. Lateral radiograph of the


lumbar spine demonstrates bilateral L4 pars defects (arrow) with associated grade 1 L4/5 isthmic spondylolisthesis. The L4/5 disk has degeneration. (From Butt S, Saifuddin A: The imaging of lumbar spondylolisthesis,
Clin Radiol 60:533546, 2005.)

testing, should be performed if the diagnosis of spondylolisthesis


is in question.

Differential Diagnosis
Spondylolisthesis is a radiographic diagnosis that is supported by a
combination of clinical history, physical examination, radiography,
and MRI. Pain syndromes that may mimic spondylolisthesis include
lumbar radiculopathy; low back strain; lumbar bursitis; lumbar fibromyositis; inflammatory arthritis; and disorders of the lumbar spinal
cord, roots, plexus, and nerves. MRI of the lumbar spine should be
performed in all patients thought to have spondylolisthesis. Screening
laboratory tests consisting of complete blood cell count, erythrocyte
sedimentation rate, antinuclear antibody testing, human leukocyte
antigen (HLA) B-27 antigen screening, and automated blood chemistry testing should be performed if the diagnosis of spondylolisthesis
is in question to help rule out other causes of pain.

Treatment
Spondylolisthesis is best treated with a multimodality approach.
Physical therapy, including flexion exercises, heat modalities,
and deep sedative massage, combined with nonsteroidal anti-
inflammatory drugs (NSAIDs) and skeletal muscle relaxants represents a reasonable starting point. The addition of steroid epidural
nerve blocks is a reasonable next step. Caudal or lumbar epidural
blocks with a local anesthetic and steroid have been shown to be
extremely effective in the treatment of pain secondary to spondylolisthesis. Underlying sleep disturbance and depression are best

L5

Figure 78-3 Spondylolisthesis. Grade II spondylolisthesis of L4 on L5.


This leads to the false impression of L4-5 disk herniation. The posterior
disk margin has not extended beyond the L5 vertebral margin (arrows),
however. (From Haaga JR, Lanzieri CF, Gilkeson RC, editors: Computed
tomography and magnetic resonance imaging of the whole body, ed 4,
St Louis, 2003, Mosby, p 732.)

treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.

Complications and Pitfalls


Failure to diagnose spondylolisthesis accurately may put the
patient at risk for the development of lumbar myelopathy, which,
if untreated, may progress to paraparesis or paraplegia. Electromyography helps distinguish plexopathy from radiculopathy and
helps identify coexistent entrapment neuropathy, such as tarsal
tunnel syndrome, which can confuse the diagnosis.

Clinical Pearls
The diagnosis of spondylolisthesis should be considered in
any patient reporting back pain, radicular pain, or both or
symptoms of pseudoclaudication. Patients with symptoms
of myelopathy should undergo MRI on an urgent basis.
Physical therapy may help prevent recurrent episodes of
pain, but, ultimately, surgical stabilization of the affected
segments may be required.

78 Spondylolisthesis 229
SUGGESTED READINGS
Agabegi SA, Fischgrund JS: Contemporary management of isthmic spondylolisthesis: pediatric and adult, Spine J 10:530543, 2010.
Butt S, Saifuddin A: The imaging of lumbar spondylolisthesis, Clin Radiol
60:533546, 2005.
Denard PJ, Holton KF, Miller J, etal: Lumbar spondylolisthesis among elderly
men: prevalence, correlates and progression, Spine 35:10721078, 2010.
Denard PJ, Holton KF, Miller J, etal: Osteoporotic Fractures in Men (MrOS)
Study Group: Back pain, neurogenic symptoms, and physical function in relation to spondylolisthesis among elderly men, Spine J 10:865873, 2010.

L4

B
Figure 78-4 Congenital spinal stenosis. A 12-year-old boy developed
leg numbness and pain after a soccer game. A, Sagittal T2-weighted
magnetic resonance imaging shows progressive narrowing of the sagittal dimension of the lumbar spinal canal from the upper to lower levels. B, On an axial proton densityweighted image at L4, short stubby
pedicles are the primary cause of small lateral recesses and congenital
spinal stenosis. (From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors:
Clinical magnetic resonance imaging, ed 3, Philadelphia, 2006, Saunders,
p 2227.)

Chapter 79
ANKYLOSING SPONDYLITIS

ICD-9 CODE 720.0


ICD-10 CODE M45.9

the knee. Spinal fracture with resultant spinal cord injury may
occur as a result of the rigid and inflexible nature of the spine.
Anterior uveitis manifests with photophobia, decreased visual acuity, and excessive lacrimation and represents an ophthalmological
emergency.

The Clinical Syndrome

Testing

Ankylosing spondylitis is an inflammatory disease of the spine,


sacroiliac joints, and occasionally extra-articular structures,
including the eye. It also is known as Marie-Strmpell disease. The
cause of ankylosing spondylitis is unknown, but autoimmunemediated mechanisms have been implicated. Approximately 90%
of patients with ankylosing spondylitis have the histocompatibility antigen human leukocyte antigen (HLA) B-27 in contrast
to 7% of the general population. The significance of this fact is
unknown, but this antigen provides the basis for a test to aid in
diagnosis of the disease. Ankylosing spondylitis occurs three times
more frequently in men, and symptoms usually appear by the
third decade of life. Onset of the disease after age 40 is rare.
Sacroiliitis is often one of the earliest manifestations of ankylosing spondylitis. This finding usually manifests as morning stiffness and a deep aching pain of insidious onset in the low back and
over the sacroiliac joints. This stiffness improves with activity and
reappears with periods of inactivity. The pain worsens as the disease progresses, and nocturnal exacerbations with significant sleep
disturbance are common. Tenderness over the spine, sacroiliac
joints, costosternal junction, and greater trochanters is common.
Pain and stiffness of the peripheral joints, including the hips and
shoulders, are present in 30% to 40% of patients with ankylosing
spondylitis. The character of the pain of ankylosing spondylitis is
dull and aching, and its intensity is mild to moderate. Occasionally, acute uveitis can occur, as can aortic valvular disease.

Plain radiographs of the sacroiliac joints usually allow the clinician to diagnose ankylosing spondylitis. Erosion of the sacroiliac
joints produces a characteristic symmetrical pseudowidening
that is diagnostic of the disease (Figure 79-2), as is squaring of
the vertebral bodies, sclerosis of the anterior margins of the vertebral bodies (so-called shiny corners), and the classic trolley track
sign resulting from ankylosis of the facet joints (Figure 79-3).
Magnetic resonance imaging (MRI) of the spine provides the
best information regarding the contents of the lumbar spine and
sacroiliac joints. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for the development of
myelopathy (Figure 79-4). In patients who cannot undergo MRI,
such as patients with pacemakers, computed tomography (CT)
or myelography is a reasonable second choice. Radionucleotide
bone scanning and plain radiography are indicated if fracture or
bony abnormality, such as metastatic disease, is being considered
in the differential diagnosis.
Although no test is diagnostic for ankylosing spondylitis, finding of the HLA B-27 antigen is highly suggestive of the disease
in patients with the previously mentioned clinical findings. This
antigen is present in 90% of patients with ankylosing spondylitis.
Complete blood cell count may reveal normocytic normochromic
anemia. The erythrocyte sedimentation rate is usually elevated, as
is the serum immunoglobulin A level.

Signs and Symptoms


Clinically, a patient with ankylosing spondylitis reports back and
sacroiliac pain and stiffness that are worse in the morning and
after periods of prolonged activity (Figure 79-1). The patient may
report a limitation of range of motion of the lateral spine and
occasionally chest expansion. This limitation of range of motion
is due to a combination of bony ankylosis and muscle spasm that
the clinician may be able to identify on physical examination.
Tenderness to palpation of the iliac crests, greater trochanter, and
axial skeleton is a common finding in ankylosing spondylitis. As
the disease progresses, the lumbar lordosis disappears, and atrophy
of the gluteal muscles may occur. A thoracic kyphosis develops,
and the neck is forward flexed. Hip ankylosis may occur with hip
involvement, and the patient often compensates with flexion at
230

Differential Diagnosis
Ankylosing spondylitis is a radiographic diagnosis that is supported by a combination of clinical history, physical examination,
and laboratory testing. Pain syndromes that may mimic ankylosing spondylitis include low back strain; lumbar bursitis; lumbar
fibromyositis; inflammatory arthritis; Reiters syndrome; collagenvascular diseases; and disorders of the lumbar spinal cord, roots,
plexus, and nerves as well as the sacroiliac joints. The clinician
should be aware that many other causes of sacroiliitis-related pain
exist (Table 79-1). Screening laboratory tests, consisting of complete blood cell count, erythrocyte sedimentation rate, antinuclear
antibody testing, HLA B-27 antigen screening, and automated
blood chemistry testing, should be performed if the diagnosis of
ankylosing spondylitis is in question to help rule out other causes
of the patients pain.

79 Ankylosing Spondylitis 231

Spine

Ilium
Sacroiliac
joint
Sacrum
Greater
trochanter

Figure 79-1 Patients with ankylosing spondylitis often report back and sacroiliac pain and stiffness that are worse in the morning and after periods
of prolonged activity.

Figure 79-2 Anteroposterior Ferguson view of the sacroiliac joints in


a patient with ankylosing spondylitis, showing bilateral symmetrical
involvement. Small, succinct erosions involve both sides of the joint,
with limited bone repair. (From Brower AC: Disorders of the sacroiliac joint,
Radiology 1:3, 1978.)

Treatment
Ankylosing spondylitis is best treated with a multimodality
approach. Physical therapy, including exercises to maintain function, heat modalities, and deep sedative massage, combined with
nonsteroidal anti-inflammatory drugs (NSAIDs) and skeletal muscle relaxants represents a reasonable starting point. Sulfasalazine
may be useful in managing the arthritis associated with the disease.
The addition of steroid epidural nerve blocks is a reasonable next
step. Caudal or lumbar epidural blocks with a local anesthetic and
steroid have been shown to be extremely effective in the treatment
of pain secondary to ankylosing spondylitis. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at

Figure 79-3 Lateral radiograph of the lumbar spine with squaring of the
vertebral bodies, which is typical of early ankylosing spondylitis. Generalized osteopenia and early inflammatory arthropathy of the lower facet
joints are seen. (From Waldman SD: Seronegative spondyloarthropathy. In
Waldman SD, Campbell RSD, editors: Imaging of pain, Philadelphia, 2011,
Saunders, pp 141144.)

232 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes

Figure 79-4 Ankylosing spondylitis. Parasagittal and sagittal T1-weighted images show extensive L2-L3 discovertebral erosion and associated marrow
edema and a posterior neural arch pseudofracture that is depicted as a horizontal dark signal abnormality (arrows). Note the presence of a prevertebral
inflammatory mass. Magnetic resonance imaging findings are indistinguishable from the findings of infectious spondylodiscitis. Plain film radiographs
showing characteristic findings of ankylosing spondylitis and clinical history are useful for differentiating the two entities. (From Edelman RR, Hesselink
JR, Zlatkin MB, etal, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2006, Saunders, p 2346.)

TABLE 79-1

Common Causes of Sacroilitis-Related Pain


Psoriatic arthritis
Reiters syndrome

disability. Myelopathy, which may progress to paraplegia or quadriplegia, is a serious problem if diagnosis is delayed. Electromyography helps distinguish plexopathy from radiculopathy and helps
identify coexistent entrapment neuropathy, such as tarsal tunnel
syndrome, which confuse the diagnosis.

Septic arthritis
Ulcerative colitis
Crohns disease
Synovitis-acne-pustulosis hyperostosis-osteomyelitis (SAPHO)
syndrome
Seronegative arthropathies, including ankylosing spondylitis
Tuberculosis
Intestinal bypassinduced arthritis
Sarcoidosis
Whipples disease

Clinical Pearls
The diagnosis of ankylosing spondylitis should be considered in any patient reporting back or sacroiliac pain and
stiffness that are worse in the morning or after prolonged
periods of inactivity. Patients with symptoms of myelopathy
should undergo MRI on an urgent basis. Physical therapy
combined with NSAIDs may help prevent recurrent episodes of pain and help preserve function.

Brucellosis
Hyperparathyroidism

a single bedtime dose of 25 mg. Acute uveitis should be managed


with corticosteroids and mydriatic agents.

Complications and Pitfalls


Failure to diagnose ankylosing spondylitis accurately may put
the patient at risk for the development of significant functional

SUGGESTED READINGS
Joseph A, Brasington R, Kahl L, etal: Immunologic rheumatic disorders, J Allergy
Clin Immunol 125(Suppl 2):S204S215, 2010.
Mansour M, Cheema GS, Naguwa SM, et al: Ankylosing spondylitis: a contemporary perspective on diagnosis and treatment, Semin Arthritis Rheum
36:210223, 2007.
Reveille JD, Arnett FC: Spondyloarthritis: update on pathogenesis and management,
Am J Med 118:592603, 2005.
Waldman SD: Seronegative spondyloarthropathy. In Waldman SD, Campbell
RSD, editors: Imaging of pain, Philadelphia, 2011, Saunders, pp 141144.

Chapter 80
SUPERIOR CLUNEAL NERVE
ENTRAPMENT SYNDROME
ICD-9 CODE 355.9
ICD-10 CODE G58.9
The Clinical Syndrome
Entrapment of the superior cluneal nerve is an uncommon cause
of low back and buttocks pain. Comprising the terminal branches
of the posterior rami of L1, L2, and L3 nerve roots, the superior
cluneal nerves provide cutaneous innervation to the upper part of
the buttocks and are susceptible to entrapment as it passes over
the iliac crest through a tunnel formed by the thoracolumbar fascia and the superior rim of the iliac crest in a manner analogous
to compression of the median nerve as it passes through the carpal tunnel (Figure 80-1). The middle branch is most commonly
affected.
This entrapment neuropathy presents as pain, numbness, and
dysesthesias in the distribution of the superior cluneal nerve. The
symptoms often begin as a burning pain in the upper buttocks
with associated cutaneous sensitivity. Patients with superior cluneal
nerve entrapment note that sitting, squatting, or wearing tight jeans
with a low rise causes the symptoms to worsen. Although traumatic
lesions to the superior cluneal nerve during bone harvesting procedures and pelvic fractures have been implicated in superior cluneal
nerve entrapment, in most patients, no obvious antecedent trauma
can be identified.

Signs and Symptoms


Physical findings include tenderness over the superior cluneal
nerves as they passes over the posterior iliac crest. A positive
Tinels sign over the superior cluneal nerves as they pass over the
posterior iliac crest may be present. Patients may report burning
dysesthesias in the nerves distribution (Figure 80-1). Careful
sensory examination of the upper buttocks may reveal a sensory
deficit in the distribution of the superior cluneal nerves; no motor
deficit should be present. Sitting or the wearing of low-cut jeans
with tight waistbands or wide belts can compress the nerve and
exacerbate the symptoms of superior cluneal nerve entrapment.

Testing
Electromyography can distinguish lumbar radiculopathy and
plexopathy from superior cluneal nerve entrapment. Plain radiographs of the back, hip, and pelvis are indicated in all patients who
present with superior cluneal nerve entrapment to rule out occult

bony pathological processes. Based on the patients clinical presentation, additional testing may be warranted, including a complete
blood count, uric acid level, erythrocyte sedimentation rate, and
antinuclear antibody testing. Magnetic resonance imaging (MRI)
of the back is indicated if herniated disk, spinal stenosis, or spaceoccupying lesion is suspected. The injection technique described
later serves as both a diagnostic and therapeutic maneuver.

Differential Diagnosis
Superior cluneal nerve entrapment is often misdiagnosed as lumbar
radiculopathy, sacroiliac joint pain, gluteal bursitis, or primary hip
pathological conditions. Radiographs of the hip and electromyography can distinguish superior cluneal nerve entrapment from
radiculopathy or pain emanating from the hip. In addition, most
patients with lumbar radiculopathy have back pain associated with
reflex, motor, and sensory changes, whereas patients with superior cluneal nerve entrapment have no back pain and no motor
or reflex changes. The sensory changes of superior cluneal nerve
entrapment are limited to the distribution of the superior cluneal
nerve and should not extend below the upper buttocks. It should
be remembered that lumbar radiculopathy and superior cluneal
nerve entrapment may coexist as the double crush syndrome.
Occasionally, lumbar plexopathy produces buttocks pain, which
may confuse the diagnosis.

Treatment
Patients with superior cluneal nerve entrapment should be
instructed in avoidance techniques to reduce the symptoms and
pain associated with this entrapment neuropathy. A short course
of conservative therapy consisting of simple analgesics, nonsteroidal anti-inflammatory drugs (NSAIDs), or cyclooxygenase-2
(COX-2) inhibitors is a reasonable first step in the treatment of
superior cluneal nerve entrapment. If patients do not experience
rapid improvement, injection is the next step.
To treat the pain of superior cluneal nerve entrapment, the
patient is placed in the prone position. The posterior iliac crest is
identified by palpation, as are the spinous processes of the adjacent
lumbar vertebra. A point 7 cm lateral to midline, along the posterior iliac crest, is identified and prepared with antiseptic solution.
A 112-inch, 25-gauge needle is slowly advanced perpendicular to
the skin until the needle is felt to pop through the fascia. A paresthesia is often elicited. After careful aspiration, a solution of 5 to 7
mL of 1% preservative-free lidocaine and 40 mg methylprednisolone is injected in a fanlike pattern as the needle pierces the fascia
gluteal muscle. After injection of the solution, pressure is applied
to the injection site to decrease the incidence of ecchymosis and
233

234 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes

Entrapped, inflamed,
and flattened
cluneal nerves
Gluteus medius
Gluteus maximus

Figure 80-1 Distribution of the superior cluneal nerves as they pass over the posterior iliac crest and provide cutaneous innervation of the buttocks.
The medial superior cluneal nerve is shown crossing the iliac crest 7 cm from midline. The wearing of low-cut jeans with tight waistbands or wide
belts can compress the nerve and exacerbate the symptoms of superior cluneal nerve entrapment.

hematoma formation, which can be quite dramatic, especially in


anticoagulated patients. If anatomical landmarks are difficult to
identify, the use of fluoroscopic or ultrasound guidance should
be considered.

Complications and Pitfalls


Care must be taken to rule out other conditions that may mimic
the pain of superior cluneal nerve entrapment. The main complications of the injection technique are ecchymosis and hematoma.
Rarely, infection may occur. Early detection of infection is crucial
to avoid potentially life-threatening sequelae.

Clinical Pearls
Superior cluneal nerve entrapment is a common condition
that is often misdiagnosed as lumbar radiculopathy, sacroiliac
pain, or gluteal bursitis. The injection technique described
can produce dramatic pain relief; however, if a patient has
pain suggestive of superior cluneal nerve entrapment but
does not respond to superior cluneal nerve block, a lesion
more proximal in the lumbar plexus or an L1-3 radiculopathy should be considered. Such patients often respond to
epidural block with steroid. Electromyography and MRI of
the lumbar plexus are indicated in this patient population to
rule out other causes of pain, including malignancy invading
the lumbar plexus or epidural or vertebral metastatic disease
at L1-3.

SUGGESTED READINGS
Akbas M, Yegin A, Karsli B: Superior cluneal nerve entrapment eight years after
decubitus surgery, Pain Pract 5:364366, 2005.
Aly TA, Tanaka Y, Aizawa T, Ozawa H, Kokubun S: Medial superior cluneal
nerve entrapment neuropathy in teenagers: a report of two cases, Tohoku J Exp
Med 197:229231, 2002.
Herring A, Price DD, Nagdev A, Simon B: Superior cluneal nerve block for
treatment of buttock abscesses in the emergency department, J Emerg Med
39:8385, 2010.
Lu J, Ebraheim NA, Huntoon M, Heck BE, Yeasting RA: Anatomic considerations of superior cluneal nerve at posterior iliac crest region, Clin Orthop Relat
Res 347:224228, 1998.
Maigne JY, Doursounian L: Entrapment neuropathy of the medial superior cluneal
nerve: nineteen cases surgically treated, with a minimum of 2 years follow-up,
Spine 22:11561159, 1997.
Talu GK, zyalin S, Talu U: Superior cluneal nerve entrapment, Reg Anesth Pain
Med 25:648650, 2000.

Chapter 81
LUMBAR MYOFASCIAL PAIN
SYNDROME

ICD-9 CODE 724.2


ICD-10 CODE M54.5
The Clinical Syndrome
The muscles of the back work together as a functional unit to
stabilize and allow coordinated movement of the low back and
allow maintaining an upright position. Trauma to an individual
muscle can result in dysfunction of the entire functional unit.
The rhomboids, latissimus dorsi, iliocostalis quadratus lumborum, multifidus, and psoas muscles are frequent sites of myofascial pain syndrome. The points of origin and attachments of
these muscles are particularly susceptible to trauma and the subsequent development of myofascial trigger points (Figure 81-1).
Injection of these trigger points serves as a diagnostic and therapeutic maneuver.
The muscles of the back are particularly susceptible to the development of myofascial pain syndrome. Flexion/extension injuries
to the back or repeated microtrauma secondary to improper lifting

and bending may result in the development of myofascial pain in


the muscles of the back. Myofascial pain syndrome is a chronic
pain syndrome that affects a focal or regional portion of the body.
The sine qua non of myofascial pain syndrome is the finding
of myofascial trigger points on physical examination. Although
these trigger points are generally localized to the regional part of
the body affected, the pain of myofascial pain syndrome often is
referred to other areas. This referred pain often is misdiagnosed or
attributed to other organ systems, leading to extensive evaluations
and ineffective treatment. Patients with myofascial pain syndrome
involving the muscles of the low back often have referred pain into
the hips, sacroiliac joint, and buttocks.

Signs and Symptoms


The trigger point is the pathognomonic lesion of myofascial pain
and is thought to be the result of microtrauma to the affected
muscles. This pathological lesion is characterized by a local point
of exquisite tenderness in affected muscle. Mechanical stimulation
of the trigger point by palpation or stretching produces not only
intense local pain, but also referred pain. In addition to this local
and referred pain, an involuntary withdrawal of the stimulated

Multifidus m.

Figure 81-1 Myofascial pain syndrome is a chronic pain syndrome that affects a focal or regional portion of the body.

235

236 SECTION 8 Lumbar Spine and Sacroiliac Joint Pain Syndromes

muscle often occurs that is called a jump sign. This jump sign also
is characteristic of myofascial pain syndrome.
Taut bands of muscle fibers often are identified when myofascial trigger points are palpated. Despite this consistent physical finding in patients with myofascial pain syndrome, the
pathophysiology of the myofascial trigger point remains elusive,
although many theories have been advanced. Common to all
of these theories is the belief that trigger points are the result of
microtrauma to the affected muscle. This microtrauma may occur
as a single injury to the affected muscle or as the result of repetitive microtrauma or chronic deconditioning of the agonist and
antagonist muscle unit.
In addition to muscle trauma, a variety of other factors seem
to predispose to development of myofascial pain syndrome. The
weekend athlete who subjects his or her body to unaccustomed
physical activity often develops myofascial pain syndrome. Poor
posture while sitting at a computer keyboard or watching television
has been implicated as a predisposing factor to the development of
myofascial pain syndrome. Previous injuries may result in abnormal
muscle function and predispose to the subsequent development of
myofascial pain syndrome. All of these predisposing factors may be
intensified if the patient also has poor nutritional status or coexisting psychological or behavioral abnormalities, including chronic
stress and depression. The muscles of the low back seem to be particularly susceptible to stress-induced myofascial pain syndrome.
Stiffness and fatigue often coexist with the pain of myofascial
pain syndrome, increasing the functional disability associated with
this disease and complicating its treatment. Myofascial pain syndrome may occur as a primary disease state or may occur in conjunction with other painful conditions, including radiculopathy
and chronic regional pain syndromes. Psychological or behavioral
abnormalities, including depression, frequently coexist with the
muscle abnormalities associated with myofascial pain syndrome.
Treatment of these psychological and behavioral abnormalities
must be an integral part of any successful treatment plan for myofascial pain syndrome.

spondylolisthesis also may mimic the clinical presentation of lumbar myofascial pain syndrome.

Treatment
Lumbar myofascial pain syndrome is best treated with a multimodality approach. Physical therapy, including correction of
functional abnormalities (e.g., poor posture, improper chair or
computer height) and the use of heat modalities and deep sedative
massage, combined with nonsteroidal anti-inflammatory drugs
(NSAIDs) and skeletal muscle relaxants represents a reasonable
starting point. If these treatments fail to provide rapid symptomatic
relief, local trigger point injection of anesthetic and steroid into the
myofascial trigger point area is a reasonable next step. Underlying
diffuse muscle pain and sleep disturbance and depression are best
treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.
When performing trigger point injections, careful preparation of the patient before injection helps optimize results. Trigger
point injections are directed at the primary trigger point, rather
than the area of referred pain. It should be explained to the patient
that the goal of trigger point injection is to block the trigger of the
persistent pain and, it is hoped, provide long-lasting relief. It is
important that the patient understand that for most patients with
myofascial pain syndrome, more than one treatment modality is
required to provide optimal pain relief. The use of the prone or
lateral position when identifying and marking trigger points and
when performing the actual trigger point injection helps decrease
the incidence of vasovagal reactions. The skin overlying the trigger point to be injected should always be prepared with antiseptic
solution before injection to avoid infection.
After the goals of trigger point injection are explained to the
patient and proper preparation of the patient has been carried out,
the trigger point to be injected is reidentified by palpation with a
sterile gloved finger (Figure 81-2). A syringe containing 10 mL of

Testing
No specific test exists for lumbar myofascial pain syndrome. Testing is aimed primarily at identifying occult pathology or other
diseases that may mimic myofascial pain syndrome (see discussion
of differential diagnosis). Plain radiographs help delineate bony
abnormality of the lumbar spine, including arthritis, fracture,
congenital abnormalities (e.g., trefoil spinal canal), and tumor. All
patients with recent onset of myofascial pain syndrome should
undergo magnetic resonance imaging (MRI) of the lumbar spine
to rule out occult pathological processes. Screening laboratory
tests, consisting of complete blood count, erythrocyte sedimentation rate, antinuclear antibody testing, and automated blood
chemistry testing, should be performed to rule out occult inflammatory arthritis, infection, and tumor.

Trapezius m.

Erector spinae m.

Latissimus dorsi m.

Serratus posterior m.

Trigger points

Differential Diagnosis
Lumbar myofascial pain syndrome is a clinical diagnosis of exclusion that is supported by a combination of clinical history, physical examination, radiography, and MRI. Pain syndromes that
may mimic lumbar myofascial pain syndrome include lumbar
strain, inflammatory arthritis, and disorders of the lumbar spinal cord, roots, plexus, and nerves. Congenital abnormalities,
such as arteriovenous malformations and trefoil spinal canal, and

Carrico & Shavell


Figure 81-2 Injection technique to relieve lumbar myofascial pain.
(From Waldman SD: Atlas of pain management injection techniques, ed
2, Philadelphia, 2007, Saunders, p 330.)

81 Lumbar Myofascial Pain Syndrome 237

0.25% preservative-free bupivacaine and 40 mg of methylprednisolone to be injected is attached to a 25-gauge needle of a length
adequate to reach the trigger point. For the deeper muscles of
posture in the low back, a 312-inch needle is required. A volume
of 0.5 to 1 mL of solution is injected into each trigger point. The
patient should be informed that a series of two to five treatment
sessions may be required to abolish the trigger point completely.

Complications and Pitfalls


The proximity to the spinal cord and exiting nerve roots makes
it imperative that this procedure be performed only by clinicians
well versed in the regional anatomy and experienced in performing interventional pain management techniques. Many patients
report a transient increase in pain after injection of trigger points.
If long needles are used, pneumothorax or damage to the retroperitoneal organs, including the kidneys, also may occur.

Clinical Pearls
Trigger point injections are an extremely safe procedure if
careful attention is paid to the clinically relevant anatomy in
the areas to be injected. Care must be taken to use sterile technique to avoid infection and universal precautions to avoid
risk to the operator. Most side effects of trigger point injection are related to needle-induced trauma to the injection
site and underlying tissues. The incidence of ecchymosis and
hematoma formation can be decreased if pressure is placed
on the injection site immediately after trigger point injection. The avoidance of overly long needles helps decrease the
incidence of trauma to underlying structures. Special care
must be taken to avoid pneumothorax when injecting trigger points in proximity to the underlying pleural space.
Antidepressant compounds are the primary pharmacological treatment for myofascial pain syndrome. Tricyclic
antidepressants are thought to be more effective than selective serotonin reuptake inhibitors in the treatment of this
painful condition. The precise mechanism of action of antidepressant compounds in the treatment of myofascial pain
syndrome is unknown. Some investigators believe that the
primary effect of this class of drugs is to treat the underlying
depression that is present in many patients with myofascial
pain syndrome. Drugs such as amitriptyline and nortriptyline represent good first choices and should be given as a
single bedtime dose, starting with 10 to 25 mg and titrating
upward as side effects allow.

SUGGESTED READINGS
Bradley LA: Pathophysiology of fibromyalgia, Am J Med 122:S22S30, 2009.
Ge H-Y, Nie HL, Madeleine P, et al: Contribution of the local and referred
pain from active myofascial trigger points in fibromyalgia syndrome, Pain
147:233240, 2009.
Krismer M, van Tulder M: The Low Back Pain Group of the Bone and Joint
Health Strategies for Europe Project: Low back pain (non-specific), Best Pract
Res Clin Rheumatol 21:7791, 2007.
Mens JMA: The use of medication in low back pain, Best Pract Res Clin Rheumatol
19:609621, 2005.
Stanos SP, PM, Harden RN: The physiatric approach to low back pain, Semin
Pain Med 2:186196, 2004.

SECTION 9 Pelvic Pain Syndromes

Chapter 82
PROCTALGIA FUGAX

ICD-9 CODE 564.6


ICD-10 CODE K59.4
The Clinical Syndrome
Proctalgia fugax is a disease of unknown cause characterized by
paroxysms of rectal pain with pain-free periods between attacks.
The pain-free periods between attacks can last seconds to minutes.
Similar to cluster headache, spontaneous remissions of the disease
occur and may last weeks to years. Proctalgia fugax is more common in women and occurs with greater frequency in patients with
irritable bowel syndrome.
The pain of proctalgia fugax is sharp or gripping and severe.
Similar to other urogenital focal pain syndromes, such as vulvodynia and prostadynia, the causes remain obscure. Stress and
sitting for prolonged periods often increase the frequency and
intensity of attacks of proctalgia fugax. Patients often feel an urge
to defecate with the onset of the paroxysms of pain (Figure 82-1).
Depression often accompanies the pain of proctalgia fugax but is
not thought to be the primary cause. The symptoms of proctalgia
fugax can be so severe as to limit the patients ability to perform
activities of daily living.

Signs and Symptoms


The physical examination of a patient with proctalgia fugax is usually normal. The patient may be depressed or appear anxious. Rectal
examination is normal, although deep palpation of the surrounding musculature may trigger paroxysms of pain. The patient often
reports that he or she can abort the attack of pain by placing a finger
in the rectum. Rectal suppositories also may interrupt the attacks.

Testing
Similar to the physical examination, testing in patients with proctalgia fugax is usually normal. Because of the risk for overlooking rectal malignancy that may be responsible for pain that may
be attributed to a benign cause, by necessity proctalgia fugax is
238

a diagnosis of exclusion. Rectal examination is mandatory in all


patients thought to have proctalgia fugax. Sigmoidoscopy or colonoscopy is strongly recommended in such patients. Testing of the
stool for occult blood is indicated. Screening laboratory studies,
consisting of a complete blood cell count, automated chemistries,
and erythrocyte sedimentation rate, should be performed. Magnetic resonance imaging (MRI) or computed tomography (CT)
of the pelvis should be considered in all patients with proctalgia
fugax to rule out occult pathology (Figure 82-2). If psychological
problems are suspected or the patient has a history of sexual abuse,
psychiatric evaluation is indicated concurrently with laboratory
and radiographic testing.

Differential Diagnosis
As mentioned previously, because of the risk for overlooking serious pathology of the anus and rectum, proctalgia fugax must be
a diagnosis of exclusion. The clinician first must rule out rectal
malignancy to avoid disaster. Proctitis can mimic the pain of proctalgia fugax and can be diagnosed on sigmoidoscopy or colonoscopy. Hemorrhoids usually manifest with bleeding associated with
pain and can be distinguished from proctalgia fugax on physical
examination. Prostadynia sometimes may be confused with proctalgia fugax, but the pain is more constant, more dull, and aching.

Treatment
Initial treatment of proctalgia fugax should include a combination of simple analgesics and nonsteroidal anti-inflammatory drugs
(NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. If these medications do not control the symptoms adequately, a tricyclic antidepressant or gabapentin should be added. Traditionally, tricyclic
antidepressants have been a mainstay in the palliation of pain secondary to proctalgia fugax. Controlled studies have shown the efficacy
of amitriptyline for this indication. Other tricyclic antidepressants,
including nortriptyline and desipramine, also have been shown to be
clinically useful. This class of drugs is associated with significant anticholinergic side effects, including dry mouth, constipation, sedation,
and urinary retention. These drugs should be used with caution in
patients with glaucoma, cardiac arrhythmia, and prostatism.
To minimize side effects and encourage compliance, the primary care physician should start amitriptyline or nortriptyline at

82 Proctalgia Fugax 239

Rectum

Anal canal

Figure 82-1 The pain of proctalgia fugax is sharp or gripping and severe. Increased stress and sitting for prolonged periods can increase the
frequency and intensity of attacks.

Figure 82-2 Ulcerative colitis. The bowel wall of the rectum and sigmoid colon is minimally thickened with a target appearance (arrowheads). (From Haaga JR, Lanzieri CF, Gilkeson RC, editors: CT and MR
imaging of the whole body, 4th ed, Philadelphia, 2003, Mosby, p 1245.)

a 10-mg dose at bedtime. The dose can be titrated upward to 25


mg at bedtime, as side effects allow. Upward titration of dosage
in 25-mg increments can be done each week as side effects allow.
Even at lower doses, patients generally report a rapid improvement in sleep disturbance and begin to experience some pain relief
in 10 to 14 days. If the patient does not experience any improvement in pain as the dose is being titrated upward, the addition
of gabapentin alone or in combination with nerve blocks of the
intercostal nerves with local anesthetics, steroid, or both is recommended. Selective serotonin reuptake inhibitors, such as fluoxetine, also have been used to treat the pain of diabetic neuropathy;

although better tolerated than the tricyclic antidepressants, they


seem to be less efficacious.
If antidepressant compounds are ineffective or contraindicated, gabapentin is a reasonable alternative. Gabapentin should
be started with a 300-mg dose at bedtime for 2 nights. The patient
should be cautioned about potential side effects, including dizziness, sedation, confusion, and rash. The drug is increased in 300mg increments, given in equally divided doses over 2 days, as side
effects allow, until pain relief is obtained or a total dose of 2400
mg daily is reached. At this point, if the patient has experienced
partial pain relief, blood values are measured, and the drug is carefully titrated upward using 100-mg tablets. More than 3600 mg
daily rarely is required.
Local application of heat and cold also may be beneficial to
provide symptomatic relief of the pain of proctalgia fugax. The
use of bland rectal suppositories may help provide symptomatic
relief. For patients who do not respond to these treatment modalities, injection of the peroneal nerves or caudal epidural nerve block
using a local anesthetic and steroid may be a reasonable next step.
Anecdotal reports indicate that calcium channel blockers, topical
nitroglycerin, and inhalation of albuterol provide symptomatic
relief of the pain of proctalgia fugax.

Complications and Pitfalls


The major problem in the care of patients thought to have
proctalgia fugax is the failure to identify potentially serious
pathology of the anus or rectum secondary to primary tumor or
invasion of these structures by pelvic tumor. Although uncommon, occult rectal infection remains a possibility, especially in

240 SECTION 9 Pelvic Pain Syndromes

an immunocompromised patient with cancer. Early detection of


infection is crucial to avoid potentially life-threatening sequelae.

Clinical Pearls
Proctalgia fugax is a distressing disease for patients. The
paroxysms of pain may occur without warning and make
the patient afraid to leave the house. The main focus of
the clinician caring for a patient with proctalgia fugax is
to ensure that occult malignancy has not been overlooked.
Given the psychological implications of pain involving the
genitals and rectum, the clinician should not overlook the
possibility of psychological abnormality in patients with
pain in the rectum.

SUGGESTED READINGS
Bharucha AE, Wald A, Enck P, Rao A: Functional anorectal disorders, Gastroenterology 130:15101518, 2006.
Karras JD, Angelo G: Proctalgia fugax, Am J Surg 82:616625, 1951.
Vincent C: Anorectal pain and irritation: anal fissure, levator syndrome, proctalgia
fugax, and pruritus ani, Prim Care 26:5368, 1999.
Waldman SD: Proctalgia fugax. In Waldman SD, editor: Pain review, Philadelphia,
2009, Saunders, pp 307308.
Weizman Z, Binsztok M: Proctalgia fugax in teenagers, J Pediatr 114:813814,
1989.

Chapter 83
PROSTATODYNIA

ICD-9 CODE 608.9


ICD-10 CODE R10.2

neuropathy can occur after radiation therapy for the treatment of


malignancy of the prostate and rectum and can mimic the pain of
prostatodynia.

Testing
Prostatodynia is an uncommon cause of perineal pain in men.
Also known as chronic nonbacterial prostatitis and chronic pelvic pain syndrome, prostatodynia probably is not a single clinical entity, but rather the conglomeration of a variety of disorders
that can cause pain in this anatomical region. Included in these
disorders are chronic infections of the prostate, chronic inflammation of the prostate without demonstrable infection, bladder
outflow abnormalities, pelvic floor muscle disorders, reflex sympathetic dystrophy, and psychogenic causes. All have in common
the ability to cause chronic, ill-defined perineal pain, which is the
hallmark of prostatodynia.
The pain of prostatodynia is characterized by dull, aching,
or burning pain of the perineum and underlying structures
(Figure 83-1). The intensity of pain is mild to moderate and
may worsen with urination or sexual activity. The pain may be
referred to the penis, testicles, scrotum, or inner thigh. Irritative
urinary outflow symptoms and sexual dysfunction often coexist with the pain of prostatodynia. The history of all patients
with chronic prostatodynia should include specific questioning
regarding a history of sexual abuse.

Digital examination of the prostate is the cornerstone of the


diagnosis of patients with prostatodynia. Careful examination
for tenderness, nodules, or tumor is crucial to avoid overlooking
prostatic malignancy. Ultrasound examination of the prostate is
indicated in all patients with prostatodynia. If any question of
occult malignancy of the prostate or pelvic contents exists, magnetic resonance imaging (MRI) or computed tomography (CT) of
the pelvis is mandatory, as is laboratory determination of prostatespecific antigen level (Figure 83-2). Acute infection of the prostate can elevate the prostate-specific antigen level. Urinalysis to
rule out urinary tract infection is indicated in all patients with
prostatodynia. The role of laboratory examination of postprostatic massage prostatic fluid in the evaluation of prostatodynia is
unclear, although anecdotal reports exist of the consistent finding
of an elevated uric acid level in the prostatic fluid of patients with
prostatodynia.
Electromyography helps distinguish radiation neuropathy
from lumbar plexopathy or lumbar radiculopathy. Based on the
patients clinical presentation, additional tests, including complete blood cell count, uric acid, erythrocyte sedimentation rate,
and antinuclear antibody testing, may be indicated. MRI of the
lumbar plexus is indicated if tumor or hematoma is suspected.

Signs and Symptoms

Differential Diagnosis

Physical examination of patients with acute prostatodynia is


directed at identifying acute bacterial infection of the prostate,
urinary tract, or both. Patients with acute orchitis secondary to
infections, including sexually transmitted diseases, have a prostate
that is exquisitely tender to palpation. For patients with chronic
prostatodynia, the physical findings are often nonspecific, with
the prostate mildly tender to palpation, unless specific pathological processes are present. Allodynia of the perineum also often is
present. Prostate malignancy always should be considered in any
patient presenting with prostatodynia. Physical findings in this
setting vary, but prostate enlargement is often an early finding.
Extraprostate pathological processes can occur with the primary symptom of prostatodynia. One of the most common causes
of prostatodynia of extraprostate origin is malignancy involving
pelvic contents other than the prostate. Tumor involving the lumbar plexus, cauda equina, or hypogastric plexus rarely can manifest as pain localized to the prostate and perineum. Postradiation

Extraprostate pathology, including reflex sympathetic dystrophy


and lesions of the lumbar plexus, nerve roots, and spinal cord,
can mimic the pain of prostatodynia and must be included in the
differential diagnosis. As mentioned earlier, because of the disastrous results of missing a diagnosis of prostatic malignancy when
evaluating and treating patients thought to have prostatodynia, it
is mandatory that malignancy be high on the list of differential
diagnostic possibilities (Figure 83-3). It is also important to correctly diagnose any underlying causes of prostatitis because some
are readily amenable to treatment with antibiotics (Table 83-1).

The Clinical Syndrome

Treatment
Initial treatment of the pain associated with prostatodynia should
include a combination of nonsteroidal anti-inflammatory drugs
(NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. The local
application of heat and cold with sitz baths also may be beneficial.
241

242 SECTION 9 Pelvic Pain Syndromes

Sitz bath

Prostate
Urethra
Penis

Testicle
Scrotum

Figure 83-1 The pain of prostatodynia is characterized by dull, aching, or burning pain of the perineum and underlying structures.

Figure 83-2 Coronal T2-weighted magnetic resonance imaging of


the prostate in a patient with a prostate-specific antigen level of 9.2,
Gleason cancer score of 7 on biopsy, and organ-confined disease on
digital rectal examination. The patient was being assessed for radical
prostatectomy. Low T2 signal intensity is seen extending into the left
seminal vesicle (arrow), consistent with T3B disease. In view of this
unequivocal finding, the patient opted to undergo radiation treatment instead of surgery. (From Edelman RR, Hesselink JR, Zlatkin MB,
etal, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2006, Saunders, p 2925.)

Figure 83-3 Prostate cancer (arrows) infiltrating and displacing the normal high signal intensity peripheral zone. The fibrous prostate capsule
is intact, separating the cancer from the high signal intensity lateral
periprostatic venous plexus. (From Stark DD, Bradley WG Jr: Magnetic
resonance imaging, 3rd ed, St Louis, 1999, Mosby, p 626.)

An arbitrary treatment course of antibiotics, such as doxycycline


100 mg twice daily for 2 weeks, may be worth trying, even though
urine cultures are negative. Anecdotal reports of decreased pain
after treatment with allopurinol make this drug a consideration
for patients who continue to have pain. For patients who do not
respond to these treatment modalities, caudal epidural nerve

83 Prostatodynia 243
TABLE 83-1

Distinguishing Features of Prostate Syndromes


Prostatic Fluid

Syndrome

Confirmed UTI

Prostate
Examination

WBC

Culture

Response to
Antibiotics

Impaired
Urinary Flow

Acute bacterial
prostatitis

Yes

Tender, warm

Yes

Yes

Yes

Yes

Chronic bacterial
prostatitis

Usually

Varied

Yes

Yes

Slow

Nonbacterial
prostatitis

No

Varied

Yes

No

Poor

Prostatodynia

No

Usually normal

No

No

No

Yes

From Lummus WE, Thompson I: Prostatitis, Emerg Med Clin North Am 19:691707, 2001.
UTI, Urinary tract infection.

blocks with a local anesthetic and steroid may be a reasonable next


step. Psychological evaluation and interventions should occur
concurrently with the aforementioned treatment modalities, given
the high incidence of coexistent psychological issues associated
with all pelvic pain syndromes.

Complications and Pitfalls


The major pitfalls in the care of a patient with prostatodynia are
threefold: (1) the misdiagnosis of extraprostate pathological processes responsible for the patients pain, (2) the failure to identify
prostate malignancy, and (3) the failure to address the psychological issues surrounding the patients pain.

Clinical Pearls
The clinician should be aware that the relationship of the
genitalia to the male psyche presents some unique challenges for the clinician treating patients with prostatodynia.
The behavioral and psychological issues must be addressed
concurrently with the medical issues if treatment is to be
successful. The possibility for prostate malignancy is ever
present and should be carefully sought in all patients with
prostatodynia.

SUGGESTED READINGS
Lummus WE, Thompson I: Prostatitis, Emerg Med Clin North Am 19:691707,
2001.
Rarbalias GA: Prostatodynia or painful male urethral syndrome? Urology
36:146153, 1990.
Turner JA, Hauge S, Von Korff M, et al: Primary care and urology patients
with the male pelvic pain syndrome: symptoms and quality of life, J Urol
167:17681773, 2002.
Wesselmann U, Burnett AL, Heinberg LJ: The urogenital and rectal pain
syndromes, Pain 73:269294, 1997.

Chapter 84
GLUTEUS MAXIMUS PAIN
SYNDROME

ICD-9 CODE 729.1


ICD-10 CODE M79.1
The Clinical Syndrome
The primary function of the gluteus maximus muscle is as a hip
extensor. The gluteus maximus muscle has its origin at the posterior aspect of the dorsal ilium, the posterior superior iliac crest,
the posterior inferior aspect of the sacrum and coccyx, and the
sacrotuberous ligament (Figure 84-1). The muscle inserts on the
fascia lata at the iliotibial band and the gluteal tuberosity on the
femur. The muscle is innervated by the inferior gluteal nerve. The

Gluteus maximus m.

Figure 84-1 The primary function of the gluteus maximus muscle is as a


hip extensor. It originates at the posterior aspect of the dorsal ilium, the
posterior superior iliac crest, the posterior inferior aspect of the sacrum
and coccyx, and the sacrotuberous ligament.

244

gluteus maximus muscle is susceptible to trauma and to wear and


tear from overuse and misuse and may develop myofascial pain
syndrome, which also may be associated with gluteal bursitis. The
pain of myofascial pain syndrome most often occurs as a result of
repetitive microtrauma to the muscle from activities such as running on soft surfaces and overuse of exercise equipment or other
repetitive activities that require hip extension. Blunt trauma to
the muscle also may incite gluteus maximus myofascial pain syndrome.
Myofascial pain syndrome is a chronic pain syndrome that
affects a focal or regional portion of the body. The sine qua
non of myofascial pain syndrome is the finding of myofascial
trigger points on physical examination. Although these trigger
points generally are localized to the regional part of the body
affected, the pain of myofascial pain syndrome often is referred
to other anatomical areas. This referred pain often is misdiagnosed or attributed to other organ systems, leading to extensive
evaluations and ineffective treatment. Patients with myofascial
pain syndrome involving the gluteus maximus often have primary pain in the medial and lower aspects of the muscle that
is referred across the buttocks and into the coccygeal area (Figure 84-2). Taut bands of muscle fibers often are identified when
myofascial trigger points are palpated. Despite this consistent
physical finding in patients who have myofascial pain syndrome,
the pathophysiology of the myofascial trigger point remains elusive, although many theories have been advanced. Common to
all of these theories is the belief that trigger points are the result
of microtrauma to the affected muscle. This microtrauma may
occur as a single injury to the affected muscle or as the result of
repetitive microtrauma or chronic deconditioning of the agonist
and antagonist muscle unit.
In addition to muscle trauma, a variety of other factors seem to
predispose the patient to develop myofascial pain syndrome. The
weekend athlete who subjects his or her body to unaccustomed
physical activity often develops myofascial pain syndrome. Poor
posture while sitting at a computer keyboard or while watching
television also has been implicated as a predisposing factor to the
development of myofascial pain syndrome. Previous injuries may
result in abnormal muscle function and predispose to the subsequent development of myofascial pain syndrome. All of these
predisposing factors may be intensified if the patient also has poor
nutritional status or coexisting psychological or behavioral abnormalities, including chronic stress and depression. The muscles of
the low back and hip seem to be particularly susceptible to stressinduced myofascial pain syndrome.
Stiffness and fatigue often coexist with the pain of myofascial
pain syndrome, increasing the functional disability associated
with this disease and complicating its treatment. Myofascial pain

84 Gluteus Maximus Pain Syndrome 245

syndrome may occur as a primary disease state or may occur in


conjunction with other painful conditions, including radiculopathy and chronic regional pain syndromes. Psychological or behavioral abnormalities, including depression, frequently coexist with
the muscle abnormalities associated with myofascial pain syndrome. Treatment of these psychological and behavioral abnormalities must be an integral part of any successful treatment plan
for myofascial pain syndrome.

Signs and Symptoms


The trigger point is the pathognomonic lesion of myofascial pain
and is thought to be the result of microtrauma to the affected
muscles. This pathological lesion is characterized by a local point
of exquisite tenderness in affected muscle. Mechanical stimulation
of the trigger point by palpation or stretching produces not only
intense local pain but also referred pain. In addition to this local
and referred pain, an involuntary withdrawal of the stimulated
muscle, termed the jump sign, often occurs. The jump sign also is
characteristic of myofascial pain syndrome. Patients with gluteus
maximus pain syndrome exhibit trigger points in the medial and
lower aspects of the muscle that are referred across the buttocks
and into the coccygeal area.

Testing
No specific test exists for gluteus maximus pain syndrome. Testing
is aimed primarily at identifying occult pathological conditions or
other diseases that may mimic myofascial pain syndrome (see discussion of differential diagnosis). Plain radiographs help delineate
bony abnormality of the pelvis and hip, including arthritis, avascular necrosis of the hip, fracture, congenital abnormalities, and
tumor. All patients with the recent onset of myofascial pain syndrome should undergo magnetic resonance imaging (MRI) of the
lumbar spine and pelvis to rule out occult pathological processes
(Figure 84-3). Screening laboratory tests, consisting of complete
blood count, erythrocyte sedimentation rate, antinuclear antibody

testing, and automated blood chemistry testing, should be performed to rule out occult inflammatory arthritis, infection, and
tumor.

Differential Diagnosis
Gluteus maximus pain syndrome is a clinical diagnosis of exclusion supported by a combination of clinical history, physical
examination, radiography, and MRI. Pain syndromes that may
mimic gluteus maximus pain syndrome include lumbosacral
radiculopathy and plexopathy, stress fractures of the pelvis and
hip, muscle strain, inflammatory arthritis, and disorders of the
lumbar spinal cord, roots, plexus, and nerves. Intrapelvic tumors
also may mimic the clinical presentation of gluteus maximus pain
syndrome.

Treatment
Gluteus maximus pain syndrome is best treated with a multimodality approach. Physical therapy, including correction of
functional abnormalities (e.g., poor posture, improper chair or
computer height) and the use of heat modalities and deep sedative
massage, combined with nonsteroidal anti-inflammatory drugs
(NSAIDs) and skeletal muscle relaxants, represents a reasonable
starting point. If these treatments fail to provide rapid symptomatic relief, local trigger point injection of local anesthetic and steroid into the myofascial trigger point area is a reasonable next step.
Underlying diffuse muscle pain and sleep disturbance and depression are best treated with a tricyclic antidepressant compound,
such as nortriptyline, which can be started at a single bedtime
dose of 25 mg.
When performing trigger point injections, careful preparation of the patient before trigger point injection helps optimize
results. Trigger point injections are directed at the primary trigger point, rather than in the area of referred pain. It should be
explained to the patient that the goal of trigger point injection is
to block the trigger of the persistent pain and, it is hoped, provide

Trigger point
Referred pain
Gluteus maximus m.

Figure 84-2 Injection technique to relieve gluteus maximus pain. (From Waldman SD: Atlas of pain management injection techniques, 2nd ed, Philadelphia, 2007, Saunders, p 379.)

246 SECTION 9 Pelvic Pain Syndromes

Figure 84-3 Gluteal intramuscular hematoma. A, Axial T1-weighted magnetic resonance imaging. Subacute left gluteal region hematoma manifests
as a hyperintense rim, consistent with the presence of methemoglobin. B, Axial T2-weighted image. The left gluteal hematoma exhibits a hyperintense signal pattern. (From Edelman RR, Hesselink JR, Zlatkin MB, et al, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2006,
Saunders, p 3387.)

long-lasting relief. It is important that the patient understand


that for most patients with myofascial pain syndrome, more
than one treatment modality is required to provide optimal pain
relief. The use of the prone or lateral position when identifying and marking trigger points and when performing the actual
trigger point injection helps decrease the incidence of vasovagal
reactions. The skin overlying the trigger point to be injected
always should be prepared with antiseptic solution before injection to avoid infection.
After the goals of trigger point injection are explained to the
patient and proper preparation of the patient has been carried
out, the trigger point to be injected is identified again by palpation with the sterile gloved finger (see Figure 84-2). A syringe
containing 10 mL of 0.25% preservative-free bupivacaine and
40 mg of methylprednisolone to be injected is attached to a
25-gauge needle of a length adequate to reach the trigger point.
A volume of 0.5 to 1 mL of solution is injected into each trigger point. The patient should be informed that two to five
treatment sessions may be required to abolish the trigger point
completely.

Complications and Pitfalls


The proximity to the sciatic nerve makes it imperative that this
procedure be performed only by clinicians well versed in the
regional anatomy and experienced in performing interventional
pain management techniques. Many patients also complain of a
transient increase in pain after injection of trigger points. If long
needles are used, damage to the retroperitoneal organs also may
occur.

Clinical Pearls
Trigger point injections are an extremely safe procedure if
careful attention is paid to the clinically relevant anatomy
in the areas to be injected. Care must be taken to use sterile
technique to avoid infection; universal precautions should
be used to avoid risk to the operator. Most side effects of
trigger point injection are related to needle-induced trauma
to the injection site and underlying tissues. The incidence
of ecchymosis and hematoma formation can be decreased
if pressure is placed on the injection site immediately after
trigger point injection. The avoidance of overly long needles
helps decrease the incidence of trauma to underlying structures. Special care must be taken to avoid pneumothorax
when injecting trigger points in proximity to the underlying pleural space. The antidepressant compounds represent
the primary pharmacological treatment for myofascial pain
syndrome. Tricyclic antidepressants are thought to be more
effective than selective serotonin reuptake inhibitors in the
treatment of this painful condition. The precise mechanism
of action of the antidepressant compounds in the treatment
of myofascial pain syndrome is unknown. Some investigators believe that the primary effect of this class of drugs is
to treat the underlying depression present in many patients
with myofascial pain syndrome. Drugs such as amitriptyline
and nortriptyline represent good first choices and should be
given as a single bedtime dose, starting with 10 to 25 mg
and titrating upward as side effects allow.

84 Gluteus Maximus Pain Syndrome 247


SUGGESTED READINGS
Arnold LM: The pathophysiology, diagnosis and treatment of fibromyalgia,
Psychiatr Clin North Am 33:375408, 2010.
Bradley LA: Pathophysiology of fibromyalgia, Am J Med 122(Suppl 1):S22S30,
2009.
Imamura M, Cassius DA, Fregni F: Fibromyalgia: from treatment to rehabilitation, Eur J Pain Suppl 3:117122, 2009.

Marsh M: Milnacipran. The comprehensive pharmacology reference, Philadelphia, 2008,


Elsevier, pp 14.
Waldman SD: Atlas of pain management injection techniques, Philadelphia, 2007,
Saunders, pp 378380.

Chapter 85
GLUTEUS MEDIUS SYNDROME

ICD-9 CODE 729.1


ICD-10 CODE M79.7
The Clinical Syndrome
The gluteus medius muscles primary function is as a hip abductor, and the muscle also assists in medial and lateral rotation of the
hip. The gluteus medius muscle finds its origin at the dorsal ilium
just below the iliac crest. The gluteus medius muscle is susceptible
to the development of myofascial pain syndrome. Such pain most
often occurs as a result of repetitive microtrauma to the muscle
from activities such as running on soft surfaces and overuse of
exercise equipment or other repetitive activities that require hip
abduction (Figure 85-1). Blunt trauma to the muscle may also
incite gluteus medius myofascial pain syndrome.
Myofascial pain syndrome is a chronic pain syndrome that
affects a focal or regional portion of the body. The sine qua non of
myofascial pain syndrome is the finding of myofascial trigger points
on physical examination. Although these trigger points generally

Figure 85-1 Gluteus medius syndrome usually results from repetitive


microtrauma to the muscle during such activities as running on soft
surfaces, overuse of exercise equipment, or other repetitive activities
that require hip extension.

248

are localized to the regional part of the body affected, the pain
of myofascial pain syndrome often is referred to other anatomical areas. This referred pain often is misdiagnosed or attributed
to other organ systems, thereby leading to extensive evaluations
and ineffective treatment. Patients with myofascial pain syndrome
involving the gluteus medius often have primary pain along the
posterior iliac crest that is referred down the buttocks across the
sacroiliac joint and into the posterior lower extremity.
The trigger point is the pathognomonic lesion of myofascial
pain and is thought to be the result of microtrauma to the affected
muscles. This pathological lesion is characterized by a local point
of exquisite tenderness in affected muscle. Mechanical stimulation
of the trigger point by palpation or stretching produces not only
intense local pain but also referred pain. In addition to this local
and referred pain, often an involuntary withdrawal of the stimulated muscle, termed a jump sign, occurs. The jump sign also is
characteristic of myofascial pain syndrome. Patients with gluteus
medius syndrome will exhibit a trigger point along the posterior
iliac crest.
Taut bands of muscle fibers often are identified when myofascial trigger points are palpated. In spite of this consistent
physical finding in patients with myofascial pain syndrome, the
pathophysiology of the myofascial trigger point remains elusive,
although many theories have been advanced. Common to all
of these theories is the belief that trigger points are the result of
microtrauma to the affected muscle. This microtrauma may occur
as a single injury to the affected muscle or as the result of repetitive microtrauma or chronic deconditioning of the agonist and
antagonist muscle unit.
In addition to muscle trauma, a variety of other factors seem to
predispose the patient to develop myofascial pain syndrome. The
weekend athlete who subjects his or her body to unaccustomed
physical activity often may develop myofascial pain syndrome.
Poor posture while sitting at a computer keyboard or while watching television also has been implicated as a predisposing factor
to the development of myofascial pain syndrome. Previous injuries may result in abnormal muscle function and predispose to
the subsequent development of myofascial pain syndrome. All of
these predisposing factors may be intensified if the patient also has
poor nutritional status or coexisting psychological or behavioral
abnormalities, including chronic stress and depression. The gluteus medius muscle seems to be particularly susceptible to stressinduced myofascial pain syndrome.
Stiffness and fatigue often coexist with the pain of myofascial pain syndrome, increasing the functional disability associated with this disease and complicating its treatment. Myofascial
pain syndrome may occur as a primary disease state or in conjunction with other painful conditions, including radiculopathy

85 Gluteus Medius Syndrome 249

and chronic regional pain syndromes. Psychological or behavioral


abnormalities, including depression, frequently coexist with the
muscle abnormalities associated with myofascial pain syndrome.
Treatment of these psychological and behavioral abnormalities
must be an integral part of any successful treatment plan for myofascial pain syndrome.

Signs and Symptoms


The trigger point is the pathological lesion of gluteus medius syndrome, and it is characterized by a local point of exquisite tenderness in gluteus medius muscle. Mechanical stimulation of the
trigger point by palpation or stretching produces both intense
local pain in the medial and lower aspects of the muscle and
referred primary pain along the posterior iliac crest that is referred
down the buttocks across the sacroiliac joint and into the posterior
lower extremity. In addition, the jump sign is often present.

Testing
Biopsies of clinically identified trigger points have not revealed
consistently abnormal histological findings. The muscle hosting
the trigger points has been described as moth eaten and as containing waxy degeneration. Increased plasma myoglobin has
been reported in some patients with gluteus medius syndrome, but
this finding has not been corroborated by other investigators. Electrodiagnostic testing has revealed an increase in muscle tension in
some patients, but again, this finding has not been reproducible.
Because of the lack of objective diagnostic testing, the clinician
must rule out other coexisting disease processes that may mimic
gluteus medius syndrome (see discussion of differential diagnosis).

Differential Diagnosis
The diagnosis of gluteus medius syndrome is based on clinical
findings rather than specific laboratory, electrodiagnostic, or
radiographic testing. For this reason, a targeted history and physical examination, with a systematic search for trigger points and
identification of a positive jump sign, must be carried out in every

patient thought to have gluteus medius syndrome. It is incumbent


on the clinician to rule out other coexisting disease processes that
may mimic gluteus medius syndrome, including primary inflammatory muscle disease, primary hip pathological processes, gluteal bursitis, and superior cluneal and gluteal nerve entrapment
(Figure 85-2). The use of electrodiagnostic and radiographic testing can identify coexisting pathological conditions such as rectal
or pelvic tumors or lumbosacral nerve lesions. The clinician must
also identify coexisting psychological and behavioral abnormalities that may mask or exacerbate the symptoms associated with
gluteus medius syndrome.

Treatment
Treatment is focused on eliminating the myofascial trigger and
achieving relaxation of the affected muscle. It is hoped that interrupting the pain cycle in this way will allow the patient to obtain
prolonged pain relief. The mechanism of action of the treatment
modalities used is poorly understood, so an element of trial and
error is involved in developing a treatment plan.
Conservative therapy consisting of trigger point injection with
local anesthetic or saline is the initial treatment of gluteus medius
syndrome. Because underlying depression and anxiety are present
in many patients, antidepressants are an integral part of most treatment plans. Other methods, including physical therapy, therapeutic heat and cold, transcutaneous nerve stimulation, and electrical
stimulation, may be helpful on a case-by-case basis. For patients
who do not respond to these traditional measures, consideration
should be given to the use of botulinum toxin type A. Although
not currently approved by the Food and Drug Administration for
this indication, the injection of minute quantities of botulinum
toxin type A directly into trigger points has been successful in the
treatment of persistent gluteus medius syndrome.

Complications and Pitfalls


Trigger point injections are extremely safe if careful attention is
paid to the clinically relevant anatomy. Sterile technique must
be used to avoid infection, along with universal precautions to

Figure 85-2 Possible entrapment of the superior gluteal nerve. A, Transverse, T1-weighted, spin echo magnetic resonance imaging (MRI) shows
denervation hypertrophy of the tensor fasciae latae muscle (arrow). B, Similar hypertrophy and high signal intensity are seen in the muscle (arrow)
on transverse, fat-suppressed, T1-weighted, spin echo MRI obtained after intravenous gadolinium administration. (From Resnick D: Diagnosis of bone
and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 3551.)

250 SECTION 9 Pelvic Pain Syndromes

minimize any risk to the operator. Most complications of trigger


point injection are related to needle-induced trauma at the injection site and in underlying tissues. The incidence of ecchymosis
and hematoma formation can be decreased if pressure is applied
to the injection site immediately after injection. The avoidance
of overly long needles can decrease the incidence of trauma to
underlying structures. Special care must be taken to avoid trauma
to the sciatic nerve.

Clinical Pearls
Although gluteus medius syndrome is a common disorder,
it is often misdiagnosed. Therefore, in patients thought
to have gluteus medius syndrome, a careful evaluation to
identify underlying disease processes is mandatory. Gluteus
medius syndrome often coexists with a variety of somatic
and psychological disorders.

SUGGESTED READINGS
Arnold LM: The pathophysiology, diagnosis and treatment of fibromyalgia, Psychiatr
Clin North Am 33:375408, 2010.
Bradley LA: Pathophysiology of fibromyalgia, Am J Med 122(Suppl 1):S22S30,
2009.
Imamura M, Cassius DA, Fregni F: Fibromyalgia: from treatment to rehabilitation,
Eur J Pain Suppl 3:117122, 2009.
Marsh M: Milnacipran, The comprehensive pharmacology reference, Philadelphia, 2008,
Elsevier, pp 14.
Waldman SD: Atlas of pain management injection techniques, Philadelphia, 2007,
Saunders, pp 378380.

Chapter 86
ORCHIALGIA

ICD-9 CODE 608.9


ICD-10 CODE R10.2
The Clinical Syndrome
Orchialgia, or testicular pain, can be a difficult clinical situation
for the patient and clinician because of the unique significance the
testicle has as part of the male psyche. This fact is crucial if the clinician is to evaluate and treat successfully patients with orchialgia.
Acute orchialgia represents a medical emergency and may be the
result of trauma, infection, or inflammation of the testes or torsion of the testes and spermatic cord. Chronic orchialgia is defined
as testicular pain that is of more than 3 months duration and
significantly interferes with the patients activities of daily living.
Chronic orchialgia can be the result of pathological processes that
are extrascrotal in origin (e.g., ureteral calculi, inguinal hernia,
ilioinguinal or genitofemoral nerve entrapment), diseases of the
lumbar spine and roots, or pathological processes that are intrascrotal in origin (e.g., chronic epididymitis, hydrocele, varicocele).
The history of all patients with chronic orchialgia should include
specific questioning regarding a history of sexual abuse.

Signs and Symptoms


Physical examination of patients with acute orchialgia is directed
at identifying acute torsion of the testes and spermatic cord, which
is a surgical emergency. Patients with acute orchitis secondary to
infections, including sexually transmitted diseases, present with
testes that are exquisitely tender to palpation. For patients with
chronic orchialgia, the physical findings are often nonspecific, with
the testicle mildly tender to palpation, unless specific pathological
processes are present (Figure 86-1). Patients with chronic testicular pain secondary to varicocele present with a scrotum that feels
like a bag of worms. Patients with chronic epididymitis present with tenderness that is localized to the epididymis. Testicular
malignancy always should be considered in any patient presenting
with orchialgia. Physical findings in this setting vary, but testicular
enlargement is often an early finding.
As mentioned earlier, extrascrotal pathological processes also
can manifest with the primary symptom of orchialgia. One of the
most common causes of orchialgia of extrascrotal origin is ilioinguinal or genitofemoral neuralgia. Ilioinguinal neuralgia manifests
as a sensory deficit in the inner thigh and scrotum in the distribution of the ilioinguinal nerve. Weakness of the anterior abdominal
wall musculature may be present. Tinels sign may be elicited by

tapping over the ilioinguinal nerve at the point it pierces the transverse abdominis muscle. A patient with ilioinguinal or genitofemoral neuralgia may assume a bent-forward novice skiers position
to eliminate pressure on the affected nerve.

Testing
Ultrasound examination of the scrotal contents is indicated in all
patients with orchialgia. Radionucleotide and Doppler studies are
indicated if vascular compromise is suspected. Transillumination
of the scrotal contents also can help identify varicocele. Electromyography helps distinguish ilioinguinal nerve entrapment from
lumbar plexopathy, lumbar radiculopathy, and diabetic polyneuropathy. Based on the patients clinical presentation, additional
tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may
be indicated. Magnetic resonance imaging (MRI) of the lumbar
plexus and pelvis is indicated if tumor or hematoma is suspected
(Figure 86-2).

Differential Diagnosis
Extrascrotal pathology, including inguinal hernia, ilioinguinal neuralgia, and lesions of the lumbar plexus, nerve roots, and spinal cord,
can mimic the pain of orchialgia and must be included in the differential diagnosis, as can a variety of systemic diseases (Table 86-1).
Considerable intrapatient variability exists in the anatomy of the
ilioinguinal and genitofemoral nerves, which can result in variation
in patients clinical presentation. The ilioinguinal nerve is a branch
of the L1 nerve root with contribution from T12 in some patients.
The nerve follows a curvilinear course that takes it from its origin of
the L1 and occasionally T12 somatic nerves to inside the concavity
of the ilium. The ilioinguinal nerve continues anteriorly to perforate
the transverse abdominis muscle at the level of the anterior superior iliac spine. The nerve may interconnect with the iliohypogastric
nerve as it continues along its course medially and inferiorly, where
it accompanies the spermatic cord through the inguinal ring and
into the inguinal canal. The distribution of the sensory innervation
of the ilioinguinal nerves varies among patients because considerable
overlap may occur with the iliohypogastric nerve. In general, the
ilioinguinal nerve provides sensory innervation to the upper portion
of the skin of the inner thigh and the root of the penis and upper
scrotum in men.

Treatment
Many treatments have been advocated for orchialgia, with varying degrees of success (Table 86-2). Initial treatment of the pain
251

252 SECTION 9 Pelvic Pain Syndromes

Testicle

Figure 86-1 For patients with chronic orchialgia, the physical findings are often nonspecific, with the testicle mildly tender to palpation unless specific
pathological processes are present.

TABLE 86-1

Causes of Chronic Orchialgia

Figure 86-2 Tumor in an undescended testis. Computed tomography


scan through the pelvis shows a large cystic-necrotic mass (M) invading
the left iliopsoas muscles. Excision revealed testicular teratoma within a
nodal mass. (From Haaga JR, Lanzieri CF, Gilkeson RC, editors: CT and MR
imaging of the whole body, 4th ed, Philadelphia, 2003, Mosby, p 1727.)

Diabetic neuropathy
Epididymal cyst/spermatocele
Epididymitis
Infectious (e.g., Chlamydia trachomatis, Neisseria gonorrhea, Ureaplasma urealyticum, coliform bacteria)
Noninfectious (e.g., reflux of urine)
Fourniers gangrene
Henoch-Schnlein purpura
Hydrocele
Idiopathic swelling
Inguinal hernia
Interstitial cystitis
Nephrolithaisis in the mid-ureter
Orchitis (e.g., mumps)
Polyarteritis nodosa
Previous surgical interventions (e.g., vasectomy, herniorrhaphy,
scrotal procedures)
Prostatitis
Psychogenic (e.g., history of sexual abuse, relationship stress)
Referred pain from abdomen or pelvis resulting from entrapment of
genitofemoral or ilioinguinal nerve roots (T10-L1), with or without
a history of surgery
Testicular torsion or torsion of the appendix testis (intermittent)
Testicular vasocongestion from sexual arousal without ejaculation
Trauma
Tumor (e.g., testicle, epididymis, spermatic cord)
Statin use
Varicocele
Vasectomy (postvasectomy pain syndrome)
From Heidelbaugh JJ: Academic mens health: case studies in clinical practice:
chronic orchialgia, J Mens Health 6:220225, 2009.

86 Orchialgia 253
TABLE 86-2

Treatment Options for Chronic Testicular Pain


Nonsurgical management
Antibiotics and nonsteroidal anti-inflammatory drugs
Alpha-adrenergic antagonists
Tricyclic antidepressants, gabapentin, carbamazepine
Allopurinol
Transcutaneous electrical nerve stimulation
Pulsed radiofrequency
Minimal invasive treatment options
Needle aspiration or enucleation of cystic lesion that might be
relevant to the site of pain
Local anesthetic infiltration of the spermatic cord with or without
methylprednisolone
Local anesthetic infiltration of the pelvic plexus under transrectal
ultrasound guidance
Direct intraprostatic injection of antibiotic and methylprednisolone
Surgical intervention
Denervation of the spermatic cord
Vasovasostomy or vasoepididymostomy in the postvasectomy pain
syndrome
Orchiectomy

Complications and Pitfalls


The major pitfalls in the care of a patient with orchialgia are fourfold: (1) the misdiagnosis of extrascrotal pathology responsible for
the patients pain, (2) the failure to identify testicular malignancy,
(3) the failure to identify vascular compromise or infectious causes
of acute orchialgia, and (4) the failure to address the psychological
issues surrounding the patients pain.

Clinical Pearls
The clinician should be aware that the relationship of the
genitalia to the male psyche presents some unique challenges when treating patients suffering from orchialgia.
The behavioral and psychological issues must be addressed
concurrently with the medical issues if treatment is to be
successful. The possibility for testicular malignancy is ever
present and should be carefully sought in all patients with
orchialgia.

From Granitsiotis P, Kirk D: Chronic testicular pain: an overview, Eur Urol


45:430436, 2004.

associated with orchialgia should include a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2
(COX-2) inhibitors and physical therapy. Local application of
heat and cold may be beneficial. The use of supportive undergarments or an athletic supporter may provide symptomatic relief.
For patients who do not respond to these treatment modalities,
injection of the spermatic cord or ilioinguinal and genitofemoral
nerves with a local anesthetic and steroid may be a reasonable next
step. If the symptoms of orchialgia persist, surgical exploration of
the scrotal contents should be considered. Psychological evaluation and interventions should take place concurrently with the
previously mentioned treatment modalities.

SUGGESTED READINGS
Christiansen CG, Sandlow JI: Testicular pain following vasectomy: a review of
postvasectomy pain syndrome, J Androl 24:293298, 2003.
Heidelbaugh JJ: Academic mens health: case studies in clinical practice: chronic
orchialgia, J Mens Health 6:220225, 2009.
Linnebur S, Hiatt WH: Probable statin-induced testicular pain, Ann Pharmacother
41:138142, 2007.
Masarani M, Cox R: The aetiology, pathophysiology and management of chronic
orchialgia, BJU Int 91:435437, 2003.
Waldman SD: Orchialgia. In Waldman SD, editor: Pain review, Philadelphia,
2009, Saunders, pp 304305.
Wampler SM, Llanes M: Common scrotal and testicular problems primary care,
Clin Office Pract 37:613626, 2010.
Wesselman U, Burnett AL, Heinburg LJ: The urogenital and rectal pain syndromes,
Pain 73:269294, 1997.

Chapter 87
VULVODYNIA

ICD-9 CODE 625.9


ICD-10 CODE R10.2
The Clinical Syndrome
Vulvodynia is an uncommon cause of pelvic pain encountered
in clinical practice. Vulvodynia probably is not a single clinical entity, but rather the conglomeration of a variety of disorders that can cause pain in this anatomical region. Included in
these disorders are chronic infections of the female urogenital
tract; chronic inflammation of the skin and mucosa of the vulva
without demonstrable bacterial, viral, or fungal infection; and
bladder abnormalities, including interstitial cystitis, pelvic floor
muscle disorders, reflex sympathetic dystrophy, and psychogenic
causes. All of these disorders have in common the ability to
cause chronic, ill-defined pain of the vulva that is the hallmark
of vulvodynia.
The pain of vulvodynia is characterized by dull, stinging, aching,
or burning pain of the vulva. The intensity of pain is mild to moderate and may worsen with bathing, urination, or sexual activity.
The pain may be referred to the perineum, rectum, or inner thigh.
Irritative urinary outflow symptoms and sexual dysfunction often
coexist with the pain of vulvodynia, with vulvodynia being one
of the leading causes of dyspareunia (Figure 87-1). All patients
with chronic vulvodynia should be questioned regarding a history
of sexual abuse, sexually transmitted diseases, and psychological
abnormalities related to sexuality.

Signs and Symptoms


Physical examination of patients with acute vulvodynia is directed
at identifying acute infections of the vulva, urinary tract, or both
that may be readily treatable. Patients with acute infections, including yeast infections and sexually transmitted diseases, have a vulva
that is irritated, inflamed, raw to the touch, and tender to palpation. For patients with chronic vulvodynia, the physical findings
are often nonspecific, with the vulva mildly tender to palpation
and an otherwise normal pelvic examination. Changes of the skin
and mucous membranes of the vulva resulting from herpes infection, chronic itching, irritation, or douching also may be present.
In a few patients with vulvodynia, spasm of the muscles of the
pelvic floor may be shown on pelvic examination. Allodynia of the
vulva and perineum may be present, especially if the patient has a
history of trauma, such as surgery, radiation therapy, or straddle
injuries. Vulva malignancy always should be considered in any
patient with vulvodynia.
254

Extravulvar pathological processes can manifest with the primary symptom of vulvodynia. One of the most common causes
of vulvodynia of extravulvar origin is malignancy involving the
pelvic contents other than the vulva. Tumor involving the lumbar plexus, cauda equina, or hypogastric plexus rarely can manifest as pain localized to the vulva and perineum. Postradiation
neuropathy can occur after radiation therapy for the treatment of
malignancy of the vulva and rectum and can mimic the pain of
vulvodynia. Ilioinguinal or genitofemoral entrapment neuropathy
also can manifest clinically as vulvodynia.

Testing
Pelvic examination is the cornerstone of the diagnosis of patients
with vulvodynia. Careful examination for infection, cutaneous or
mucosal abnormalities, tenderness, muscle spasm, or tumor is crucial to avoid overlooking vulvar malignancy. Ultrasound examination of the pelvis is indicated in all patients with vulvodynia. If any
question of occult malignancy of the vulva or pelvic contents exists,
magnetic resonance imaging (MRI) or computed tomography
(CT) of the pelvis is mandatory to rule out malignancy or disease of
the pelvic organs, such as endometriosis, which may be responsible
for the pain symptoms (Figure 87-2). Urinalysis to rule out urinary
tract infection also is indicated in all patients with vulvodynia. Culture for sexually transmitted diseases, including herpes, is indicated
in the evaluation of all patients thought to have vulvodynia.
Electromyography helps distinguish entrapment neuropathy of
the genitofemoral or ilioinguinal nerves from lumbar plexopathy or
lumbar radiculopathy. Based on the patients clinical presentation,
additional tests, including complete blood cell count, erythrocyte
sedimentation rate, and antinuclear antibody testing, may be indicated. MRI of the lumbar plexus is indicated if tumor or hematoma
is suspected.

Differential Diagnosis
Extravulvar pathological findings, including reflex sympathetic dystrophy and lesions of the lumbar plexus, nerve roots, and spinal
cord, can mimic the pain of vulvodynia and must be included in
the differential diagnosis. As mentioned earlier, because of the disastrous results of missing a diagnosis of pelvic or vulvar malignancy
when evaluating and treating patients thought to have vulvodynia,
it is mandatory that malignancy be high on the list of differential
diagnostic possibilities.

Treatment
A variety of treatments have been advocated in the treatment of
vulvodynia with varying degrees of success (Tables 87-1 and 87-2).

87 Vulvodynia 255

Rectum
Bladder

Perineum
Vulva
Inner thigh

Figure 87-1 The pain of vulvodynia is characterized by dull, stinging, aching, or burning pain of the vulva. The pain may be referred to the perineum,
rectum, or inner thigh.

Initial treatment of the pain associated with vulvodynia should


include implementation of the approaches listed in Table 87-1
along with a combination of nonsteroidal anti-inflammatory drugs
(NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors. Local application of heat and cold with sitz baths may be beneficial. An arbitrary
treatment course of antibiotics, such as doxycycline 100 mg twice
daily for 2 weeks, may be worth trying, even though urine cultures
are negative. A course of treatment for vaginal yeast infection concurrently with the antibiotics also should be considered. Anecdotal
reports of decreased pain after treatment with adjuvant analgesics
such as a tricyclic antidepressant (e.g., nortriptyline 25 mg at bedtime and titrating upward as side effects allow) or gabapentin make
these drugs a consideration for patients who continue to have pain
in the absence of demonstrable treatable disease.
For patients who do not respond to these treatment modalities,
caudal epidural nerve blocks with a local anesthetic and steroid may
be a reasonable next step. If the symptoms of vulvodynia persist,
laparoscopy should be considered. Psychological evaluation and
interventions should take place concurrently with the previously
mentioned treatment modalities given the high incidence of coexistent psychological issues associated with all pelvic pain syndromes.

Complications and Pitfalls


The major pitfalls in the care of a patient with vulvodynia are
threefold: (1) the misdiagnosis of extravulvar pathological processes responsible for the patients pain, (2) the failure to identify
vulvar or pelvic malignancy or both, and (3) the failure to address
the psychological issues surrounding the patients pain.

Clinical Pearls
The clinician should be aware that the relationship of
the genitalia to the female psyche presents some unique
challenges when treating patients with vulvodynia. The
behavioral and psychological issues must be addressed
concurrently with the medical issues if treatment is to be
successful. The possibility for vulvar or pelvic malignancy
is ever present and should be carefully sought out in all
patients with vulvodynia.

256 SECTION 9 Pelvic Pain Syndromes

*
C

Figure 87-2 A 41-year-old woman with chronic pelvic pain and a suspicious multicystic pelvic mass at sonography. A, Axial T2-weighted magnetic
resonance imaging shows a complex cystic mass associated with normal hyperintense follicles at the right posterior (large arrow) and anterior left side
suggesting a bilateral adnexal origin. Shadowing of the left part of the cyst suggests blood products. The anterior rectal wall is abnormally thickened
and contains hyperintense spots (small arrow). B, Axial T1-weighted image at the same level as in A shows hyperintense portions (asterisks). C, Fatsuppressed T1-weighted image shows that the hyperintense areas seen in B persist, confirming the suspicion of bilateral endometrioma. Multiple
smaller, high-intensity foci are better seen with fat suppression (arrows). D, Sagittal T2-weighted image confirms abnormal thickening of the anterior
rectal wall posterior to the ovary (arrow) and a hypointense nodule in the pouch of Douglas posterior to the cervix (asterisk). Subsequent laparotomy
with bilateral oophorectomy and anterior rectal resection confirmed bilateral endometriomas (8 cm rectal and 2 cm peritoneal endometriosis of the
pouch of Douglas), associated with functional ovarian cysts. (From Edelman RR, Hesselink JR, Zlatkin MB, etal, editors: Clinical magnetic resonance
imaging, 3rd ed, Philadelphia, 2006, Saunders, p 2980.)

TABLE 87-1

First-Line Treatment Options for Vulvodynia


Cease use of potentially irritating perfumed soaps, lotions, sprays,
or douches
After urination, rinse vulvar skin with distilled water
Rinse all underwear in a separate cycle with only water after
laundering with detergent
Do not use fabric softener on underwear
Use only 100% cotton menstrual pads and tampons
Wear only 100% cotton underwear and stockings, not nylon
pantyhose
Bathe in water with oatmeal, baking soda, Aveeno, or sitz baths
with tea; no bubble baths; cold compresses
Lubricate with vitamin E oil or vegetable oil, Desitin, or A&D
ointment
From Glazer HI, Ledger WJ: Clinical management of vulvodynia, Rev Gynaecol
Pract 2:8390, 2002.

87 Vulvodynia 257
TABLE 87-2

SUGGESTED READINGS

Specific Treatment Options for Vulvodynia

Edwards L: New concepts in vulvodynia, Am J Obstet Gynecol 189(Suppl 1):S24S30,


2003.
Glazer HI, Ledger WJ: Clinical management of vulvodynia, Rev Gynaecol Pract
2:8390, 2002.
Groysman V: Vulvodynia: new concepts and review of the literature, Dermatol
Clin 28:681696, 2010.
Masheb RM, Nash JM, Brondolo E, Kerns RD: Vulvodynia: an introduction and
critical review of a chronic pain condition, Pain 86:310, 2000.

Treat abnormal visible conditions such as infections, dermatoses,


and both malignant and premalignant conditions
Vulvar care measures; avoidance of irritants
Topical medications
Lidocaine 5% jelly at introitus at bedtime
Nitroglycerine
Amitriptyline 2%, baclofen 2% ( ketofen 2%)
Capsaicin
Oral medications
Antidepressant class
Tricyclic medications (150 mg/day)
Venlafaxine extended release (150 mg/day)
Duloxetine (60 mg twice daily)
Anticonvulsant class
Gabapentin (3600 mg/day)
Pregabalin (300 twice daily)
Injections
Triamcinolone 10 mg/mL, 0.2-0.4 mL into trigger point
Botulinum toxin A injections
Intralesional interferon- (no longer used)
Pelvic floor physical therapy
Pelvic floor surface electromyography and biofeedback
Low-oxalate diet with calcium citrate supplementation
(controversial)
Cognitive behavioral therapy, sexual counseling
Surgery (for vestibulodynia only) localized excision, vestibulectomy,
or perineoplasty
From Groysman V: Vulvodynia: new concepts and review of the literature, Dermatol Clin 28:681696, 2010.

Chapter 88
CLITORAL PRIAPISM

ICD-9 CODE 625.8


ICD-10 CODE N94.89
The Clinical Syndrome
In health, an integral part of the human sexual response is tumescence of the penis in males and the clitoris and vulva in females,
known as erection. The physiological process that results in tumescence is the result of a complex interplay of the parasympathetic
and sympathetic nervous system and vasoactive neurotransmitters,
including prostaglandin E1 and the vasoactive intestinal polypeptide, as well as nitric oxide. Rarely, tumescence of the penis and
clitoris can occur in the absence of sexual arousal and can be the
result of systemic disease, for example, sickle cell disease or drugs
such as sildenafil and trazodone (Table 88-1). Occasionally, no
causative factor can be identified. If the duration of tumescence
is prolonged, it is termed priapism. Although most attention has
been paid to cases of priapism occurring in males, more recently
cases of clitoral priapism have been identified as an uncommon
cause of female pelvic pain.

Signs and Symptoms


Priapism of the clitoris is defined as a painful and often prolonged erection of the clitoris in the absence of sexual arousal

(Figure 88-1). The erection may last from minutes to hours and
is often described as painful, with the pain being characterized
as burning and often involving not only the clitoris but also the
vulva. The patient may be hesitant to describe the exact nature
or location of the painful erection because of embarrassment or
a lack of understanding as to what is actually causing the pain.
Often, the patient may report a painful swelling of her vagina
and attribute her symptoms to an insect bite, urinary tract or
vaginal infection, or allergic reaction. On physical examination,
the examiner will note that the clitoris is erect and firm, with the
glans of the clitoris retracted beneath the engorged clitoral hood.
Rubor is often present, as well as significant allodynia. Vaginal
transudation, which is seen as part of female sexual arousal, is
usually absent. It should be noted that enlargement of the clitoris
has other causes, some of which are painful and some not, such
as infiltrative tumors (Table 88-2).

Testing
Pelvic examination is the cornerstone of the diagnosis of patients
with vulvodynia. Careful examination for infection, cutaneous
or mucosal abnormalities, tenderness, muscle spasm, or tumor
is crucial to avoid overlooking clitoral, vulvar, or pelvic malignancy. Ultrasound examination of the pelvis is indicated in all
patients with clitoral priapism. If any question exists regarding occult malignancy of the vulva or pelvic contents, magnetic

TABLE 88-1

Drugs Implicated in Priapism in Men and Women


Trazodone
Bupropion
Risperidone
Olanzapine
Fluoxetine
Bromocriptine
Nefazodone
Citalopram
Papaverine
Cocaine
Sildenafil
Vardenafil
Tadalafil

258

Figure 88-1 Clitoral tumescence. (From Bruni V, Pontello V, Dei M, etal:


Hemangioma of the clitoris presenting as clitoromegaly: a case report,
J Pediatr Adolesc Gynecol 22:el37e138, 2009.)

88 Clitoral Priapism 259

resonance imaging (MRI) or computed tomography (CT) of the


pelvis is mandatory to rule out malignancy or disease of the pelvic
organs that may be responsible for the symptoms (Figure 88-2).
Urinalysis to rule out urinary tract infection also is indicated in
all patients with vulvodynia. Culture for sexually transmitted
diseases, including herpes, is indicated in the evaluation of all
patients thought to have clitoral priapism.
Based on the patients clinical presentation, additional tests,
including complete blood cell count, erythrocyte sedimentation
rate, and antinuclear antibody testing, may be indicated. MRI and
electromyography of the lumbar plexus are indicated if tumor or
hematoma is suspected.

TABLE 88-2

Causes of Clitoromegaly
Congenital
Congenital adrenal hyperplasia, classical
Ambiguous genitalia, isolated or in syndromic conditions
Acquired
Hormonal
Congenital adrenal hyperplasia, late onset
Ovarian or adrenal tumors (androgen secreting)
Iatrogenic androgen exposure
Nonhormonal
Neurofibromatosis
Epidermoid cyst (spontaneous or traumatic, female genital
mutilation)
Hemangioma of the clitoris or the prepuce
Metastatic infiltration
Idiopatic
Modified from Bruni V, Pontello V, Dei M, etal: Hemangioma of the clitoris
presenting as clitoromegaly: a case report, J Pediatr Adolesc Gynecol
22:el37e138, 2009.

Differential Diagnosis
Extravulvar pathological findings, including reflex sympathetic
dystrophy and lesions of the lumbar plexus, nerve roots, and spinal cord, can mimic the pain of vulvodynia and must be included
in the differential diagnosis. As mentioned earlier, because of the
disastrous results of missing a diagnosis of pelvic or vulvar malignancy when evaluating and treating patients thought to have vulvodynia, it is mandatory that malignancy be high on the list of
differential diagnostic possibilities.

Treatment
The foundation of treatment of clitoral priapism is to first identify the factor responsible for the symptoms and then immediately
remove it. Because the vast majority of cases of both male and
female priapism are drug induced, a careful drug history looking at
both legal and illegal drugs is mandatory (see Table 88-1). A history of spider bite or painful insect stings also should be ascertained
because the venom of both black widow spiders and scorpions can
cause priapism. Empiric treatment with alpha-adrenergic drugs
such as phenylephrine and phenylpropanolamine should be initiated with careful monitoring of the patients cardiovascular status.

Complications and Pitfalls


The major pitfalls in the care of a patient with clitoral priapism are
threefold: (1) the misdiagnosis of extraclitoral pathological processes responsible for the pain, (2) the failure to identify clitoral or
vulvar or pelvic malignancy or both, and (3) the failure to address
the psychological issues surrounding the pain.

Clinical Pearls
The most common cause of clitoral priapism is drug-induced
clitoral dysfunction. The clinician should be aware that the
relationship of the genitalia to the female psyche presents
some unique challenges when treating patients with clitoral
priapism. The behavioral and psychological issues must be
addressed concurrently with the medical issues if treatment
is to be successful. The possibility for vulvar or pelvic malignancy is ever present and should be carefully sought in all
patients thought to have clitoral priapism.

SUGGESTED READINGS

Figure 88-2 Clitoral carcinoma. (From Matsuo K, Hew KE, Im DD, Rosenshein NB: Clitoral metastasis of anal adenocarcinoma associated with rectovaginal fistula in long standing Crohns disease, Eur J Obstet Gynecol
Reprod Biol 144:182183, 2009.)

Compton MT, Miller AH: Priapism associated with conventional and atypical
antipsychotic medications: a review, J Clin Psychiatry 62:362366, 2001.
Fedele L, Fontana E, Bianchi S, Frontino G, Berlanda N: An unusual case of clitoromegaly, Eur J Obstet Gynecol Reprod Biol 140:287288, 2008.
Gharahbaghian L: Clitoral priapism with no known risk factors, West J Emerg Med
9:235237, 2008.
Levin R, Riley A: The physiology of human sexual function, Psychiatry 6:9094,
2007.
Rosenberg I, Aniskin D, Bernay L: Psychiatric treatment of patients predisposed to
priapism induced by quetiapine, trazodone and risperidone: a case report, Gen
Hosp Psychiatry 31:98, 2009.

Chapter 89
GLUTEAL BURSITIS

ICD-9 CODE 726.5


ICD-10 CODE M70.70
The Clinical Syndrome
Gluteal bursitis is an uncommon cause of buttock pain that is
frequently misdiagnosed as primary hip pathological conditions.
A patient with gluteal bursitis frequently reports pain at the
upper outer quadrant of the buttock and with resisted abduction
and extension of the lower extremity. The pain is localized to the
area over the upper outer quadrant of the buttock, with referred
pain noted into the sciatic notch. Often, the patient is unable to
sleep on the affected hip and may report a sharp, catching sensation when extending and abducting the hip, especially on first
awakening.
The gluteal bursae lie between the gluteal maximus, medius,
and minimus muscles and between these muscles and the
underlying bone. These bursae may exist as a single bursal sac
or in some patients may exist as a multisegmented series of
sacs that may be loculated. The gluteal bursae are vulnerable
to injury from acute trauma and repeated microtrauma. The
action of the gluteus maximus muscle includes the flexion of
trunk on thigh when maintaining a sitting position when riding
a horse (Figure 89-1). This action can irritate the gluteal bursae
and result in pain and inflammation. Acute injuries frequently
take the form of direct trauma to the bursa from falls directly
onto the buttocks or repeated intramuscular injections and from
overuse such as running for long distances, especially on soft
or uneven surfaces. If the inflammation of the gluteal bursae
becomes chronic, calcification of the bursae may occur.

Testing
Plain radiographs of the hip may reveal calcification of the bursa
and associated structures consistent with chronic inflammation.
Magnetic resonance imaging (MRI) is indicated if occult mass or
tumor of the hip is suspected. Electromyography should be performed if neurological findings are present to rule out plexopathy, radiculopathy, or nerve entrapment syndromes of the lower
extremity. Based on the patients clinical presentation, additional
tests, including complete blood cell count; human leukocyte antigen (HLA) B-27 testing; automated serum chemistries, including
uric acid; erythrocyte sedimentation rate; and antinuclear antibody
testing, may be indicated. The injection technique described here
serves as a diagnostic and therapeutic maneuver for patients with
gluteal bursitis.

Differential Diagnosis
Gluteal bursitis is often misdiagnosed as sciatica or attributed to
primary hip pathological processes. Radiographs of the hip and
electromyography help distinguish gluteal bursitis from radiculopathy of pain emanating from the hip. Most patients with a lumbar
radiculopathy have back pain associated with reflex, motor, and
sensory changes, whereas patients with gluteal bursitis have only
secondary back pain and no neurological changes. Piriformis syndrome sometimes may be confused with gluteal bursitis, but can

Signs and Symptoms


Physical examination of patients with gluteal bursitis may reveal
point tenderness in the upper outer quadrant of the buttocks.
Passive flexion and adduction and active resisted extension and
abduction of the affected lower extremity reproduce the pain.
Sudden release of resistance during this maneuver markedly
increases the pain.
Examination of the hip and sacroiliac joint is normal. Careful
neurological examination of the affected lower extremity should
reveal no neurological deficits. If neurological deficits are present,
evaluation for plexopathy, radiculopathy, or entrapment neuropathy should be undertaken. These neurological symptoms can
coexist with gluteal bursitis, confusing the clinical diagnosis.
260

Figure 89-1 The action of the gluteus maximus muscle includes the
flexion of trunk on thigh when maintaining a sitting position when
riding a horse.

89 Gluteal Bursitis 261

be distinguished by the presence of motor and sensory changes


involving the sciatic nerve. These motor and sensory changes are
limited to the distribution of the sciatic nerve below the sciatic
notch. Lumbar radiculopathy and sciatic nerve entrapment may
coexist as the double crush syndrome. The pain of gluteal bursitis may cause alteration of gait, which may result in secondary
back and radicular symptoms that may coexist with this entrapment neuropathy.

Treatment
Initial treatment of the pain and functional disability associated
with gluteal bursitis should include a combination of nonsteroidal
anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2)
inhibitors and physical therapy. Local application of heat and cold
may be beneficial. The repetitive movements that incite the syndrome should be avoided. For patients who do not respond to
these treatment modalities, injection of gluteal bursa with a local
anesthetic and steroid may be a reasonable next step.
To inject the gluteal bursae, the patient is placed in the lateral
position with the affected side up and the affected leg flexed at the
knee. Preparation with antiseptic solution of the skin overlying
the upper outer quadrant of the buttocks is carried out. A syringe
containing 4 mL of 0.25% preservative-free bupivacaine and 40
mg of methylprednisolone is attached to a 25-gauge, 112-inch
needle. The point of maximal tenderness within the upper, outer
quadrant of the buttocks is identified with a sterile gloved finger.
Before needle placement, the patient should be advised to say
There! immediately if he or she feels a paresthesia into the lower
Gluteal
medius
Ilium

Gluteal
maximus
Gluteal
minimus
Gluteal
bursae
Sciatic
nerve

Figure 89-2 Injection technique to relieve the pain resulting from gluteal
bursitis.

extremity, indicating that the needle has impinged on the sciatic


nerve. Should a paresthesia occur, the needle should be withdrawn
immediately and repositioned more medially. The needle is carefully advanced perpendicular to the skin at the previously identified point until it impinges on the wing of the ilium (Figure 89-2).
Care must be taken to keep the needle medial and not to advance
it laterally, or it could impinge on the sciatic nerve. After careful
aspiration and if no paresthesia is present, the contents of the
syringe are gently injected into the bursa. Minimal resistance to
injection should be felt.

Complications and Pitfalls


The proximity to the sciatic nerve makes it imperative that this
procedure be performed only by clinicians well versed in the
regional anatomy and experienced in performing injection techniques. Many patients also report a transient increase in pain after
injection of the bursae.

Clinical Pearls
This injection technique is extremely effective in the treatment of gluteal bursitis. It is a safe procedure if careful attention is paid to the clinically relevant anatomy in the areas
to be injected. Care must be taken to use sterile technique
to avoid infection and universal precautions to avoid risk to
the operator. Most side effects of this injection technique
are related to needle-induced trauma to the injection site
and underlying tissues. The incidence of ecchymosis and
hematoma formation can be decreased if pressure is placed
on the injection site immediately after injection. The avoidance of overly long needles helps decrease the incidence of
trauma to underlying structures. Special care must be taken
to avoid trauma to the sciatic nerve.
The use of physical modalities, including local heat and
gentle stretching exercises, should be introduced several
days after the patient undergoes this injection technique.
Vigorous exercises should be avoided because they would
exacerbate the symptoms. Simple analgesics, NSAIDs, and
antimyotonic agents such as tizanidine may be used concurrently with this injection technique.

SUGGESTED READINGS
Bancroft LW, Peterson JJ, Kransdorf MJ: Cysts, geodes, and erosions, Radiol Clin
North Am 42:7387, 2004.
Hodnett PA, Shelly MJ, MacMahon PJ, Kavanagh EC, Eustace SJ: MR imaging of
overuse injuries of the hip, MRI Clin North Am 17:667679, 2009.
Tibor LM, Sekiya JK: Differential diagnosis of pain around the hip joint, Arthroscopy
24:14071421, 2008.
Waldman SD: Injection technique for gluteal bursitis. In Waldman SD, editor:
Pain review, Philadelphia, 2009, Saunders, pp 549551.
Waldman SD: The gluteal bursa. In Waldman SD, editor: Pain review, Philadelphia, 2009, Saunders, pp 139140.

Chapter 90
LEVATOR ANI PAIN SYNDROME

ICD-9 CODE 729.1


ICD-10 CODE M79.7
The Clinical Syndrome
The primary function of the levator ani muscle is active support
of the pelvic contents, compressing the urethra and vagina by elevating the pelvic floor, and maintaining a physiological anorectal
angle by pulling the anorectal junction forward. The levator ani
muscle originates at the posterior surface of the body of the pubis,
the fascia of the obturator internus muscle, and the ischial spine
(Figure 90-1). The muscle inserts on the anococcygeal raphe and
coccyx. The muscle is innervated by the branches of the ventral
primary rami of spinal nerves S3-4. The levator ani muscle is susceptible to trauma and to wear and tear from overuse and misuse.
It may develop myofascial pain syndrome that may also be associated with gluteal bursitis and coccygodynia, which may confuse
the clinical picture further.
Myofascial pain syndrome is a chronic pain syndrome that
affects a focal or regional portion of the body. The sine qua non
of myofascial pain syndrome is the finding of myofascial trigger
points on physical examination. Although these trigger points
generally are localized to the regional part of the body affected,
the pain of myofascial pain syndrome often is referred to other
anatomical areas. This referred pain often is misdiagnosed or

attributed to other organ systems, leading to extensive evaluations and ineffective treatment. Patients with levator ani syndrome
exhibit a trigger point along the rectum or perineum (Figure 90-2).
Taut bands of muscle fibers often are identified when myofascial
trigger points are palpated. Despite this consistent physical finding
in patients who have myofascial pain syndrome, the pathophysiology of the myofascial trigger point remains elusive, although many
theories have been advanced. Common to all of these theories is
the thought that trigger points are the result of microtrauma to the
affected muscle. This microtrauma may occur as a single injury to
the affected muscle or may occur as the result of repetitive microtrauma or chronic deconditioning of the agonist and antagonist
muscle unit.
In addition to muscle trauma, a variety of other factors seem to
predispose the patient to develop myofascial pain syndrome. The
weekend athlete who subjects his or her body to unaccustomed
physical activity often develops myofascial pain syndrome. Poor
posture while sitting at a computer keyboard or while watching
television also has been implicated as a predisposing factor to the

Inflamed levator ani m.

Trigger point
Referred pain

Levator ani m.
Figure 90-1 The levator ani muscle originates at the posterior surface of
the body of the pubis, the fascia of the obturator internus muscle, and
the ischial spine.

262

Figure 90-2 Patients with levator ani syndrome exhibit a trigger point
along the rectum or perineum. (From Waldman SD: Atlas of pain management injection techniques, 2nd ed, Philadelphia, 2007, Saunders, p 385.)

90 Levator Ani Pain Syndrome 263

development of myofascial pain syndrome. Previous injuries may


result in abnormal muscle function and predispose to the subsequent development of myofascial pain syndrome. All of these
predisposing factors may be intensified if the patient also has poor
nutritional status or coexisting psychological or behavioral abnormalities, including chronic stress and depression. The levator
ani muscle seems to be particularly susceptible to stress-induced
myofascial pain syndrome.
Stiffness and fatigue often coexist with the pain of myofascial
pain syndrome, increasing the functional disability associated with
this disease and complicating its treatment. Myofascial pain syndrome may occur as a primary disease state or may occur in conjunction with other painful conditions, including radiculopathy
and chronic regional pain syndromes. Psychological or behavioral
abnormalities, including depression, frequently coexist with the
muscle abnormalities associated with myofascial pain syndrome.
Treatment of these psychological and behavioral abnormalities
must be an integral part of any successful treatment plan for myofascial pain syndrome.

Signs and Symptoms


The trigger point is the pathognomonic lesion of myofascial pain
and is thought to be the result of microtrauma to the affected

muscles. This pathological lesion is characterized by a local point


of exquisite tenderness in affected muscle. Mechanical stimulation of the trigger point by palpation or stretching produces not
only intense local pain but also referred pain. In addition to this
local and referred pain, an involuntary withdrawal of the stimulated muscle, termed a jump sign, may occur. The jump sign also
is characteristic of myofascial pain syndrome. Patients with gluteus medius syndrome exhibit a trigger point along the rectum or
perineum (see Figure 90-2).

Testing
No specific test exists for levator ani pain syndrome. Testing
is aimed primarily at identifying occult pathological conditions
or other diseases that may mimic myofascial pain syndrome
(see discussion of differential diagnosis). Plain radiographs
help delineate bony abnormality of the pelvis and hip, including arthritis, avascular necrosis of the hip, fracture, congenital
abnormalities, and tumor. All patients with recent onset of
myofascial pain syndrome should undergo magnetic resonance
imaging (MRI) of the lumbar spine and pelvis to rule out occult
pathological processes. Screening laboratory tests, consisting of
complete blood count, erythrocyte sedimentation rate, antinuclear antibody testing, and automated blood chemistry testing,

C
Figure 90-3 Crohns disease with enterorectal fistula. A, Small-bowel follow-through examination shows an enterorectal fistula (arrow). B, Computed tomography (CT) scan shows diffuse pericolonic, perirectal, and perienteric inflammatory infiltrates with bowel wall thickening of pelvic ileal
loops. C, Gadolinium-enhanced MR image shows marked contrast enhancement in the thickened rectal wall (arrows) and inflammatory tissues
(arrowheads) surrounding the fistula. (From Haaga JR, Lanzieri CF, Gilkeson RC, editors: CT and MR imaging of the whole body, 4th ed, Philadelphia,
2003, Mosby, p 1244.)

264 SECTION 9 Pelvic Pain Syndromes

should be performed to rule out occult inflammatory arthritis,


infection, and tumor.

Differential Diagnosis
Levator ani pain syndrome is a clinical diagnosis of exclusion
supported by a combination of clinical history, physical examination, radiography, and MRI. Pain syndromes that may mimic
levator ani pain syndrome include lumbosacral radiculopathy and
plexopathy; stress fractures of the pelvis and hip; myofascial pain
syndromes such as gluteus medius pain syndrome; pelvic floor
muscle strain; inflammatory arthritis; and disorders of the lumbar
spinal cord, roots, plexus, and nerves. Intrapelvic tumors also may
mimic the clinical presentation of gluteus medius pain syndrome
(Figure 90-3).

Treatment
Levator ani pain syndrome is best treated with a multimodality
approach. Physical therapy, including correction of functional
abnormalities (e.g., poor posture, improper chair or computer
height) and use of heat modalities and deep sedative massage,
combined with nonsteroidal anti-inflammatory drugs (NSAIDs)
and skeletal muscle relaxants is a reasonable starting point. If these
treatments fail to provide rapid symptomatic relief, local trigger
point injection of local anesthetic and steroid into the myofascial trigger point area is a reasonable next step. Underlying diffuse
muscle pain, sleep disturbance, and depression are best treated
with a tricyclic antidepressant compound, such as nortriptyline,
which can be started at a single bedtime dose of 25 mg.
When performing trigger point injections, careful preparation
of the patient before trigger point injection helps optimize results.
Trigger point injections are directed at the primary trigger point,
rather than in the area of referred pain. It should be explained
to the patient that the goal of trigger point injection is to block
the trigger of the persistent pain and, it is hoped, provide longlasting relief. It is important that the patient understand that with
most patients who have myofascial pain syndrome, more than one
treatment modality is required to provide optimal pain relief. The
use of the prone or lateral position when identifying and marking
trigger points and when performing the actual trigger point injection helps decrease the incidence of vasovagal reactions. The skin
overlying the trigger point to be injected always should be prepared with antiseptic solution before injection to avoid infection.

Complications and Pitfalls


The proximity to the rectum, vagina, and pelvic viscera makes it
imperative that this procedure be performed only by clinicians

well versed in the regional anatomy and experienced in performing interventional pain management techniques. Many patients
also report a transient increase in pain after injection of trigger
points. If long needles are used, damage to the retroperitoneal
organs also may occur.

Clinical Pearls
Trigger point injections are an extremely safe procedure if
careful attention is paid to the clinically relevant anatomy
in the areas to be injected. Care must be taken to use sterile technique to avoid infection and universal precautions
to avoid risk to the operator. Most side effects of trigger
point injection are related to needle-induced trauma to
the injection site and underlying tissues. The incidence of
ecchymosis and hematoma formation can be decreased if
pressure is placed on the injection site immediately after
trigger point injection. The avoidance of overly long needles helps decrease the incidence of trauma to underlying
structures. Special care must be taken to avoid pneumothorax when injecting trigger points in proximity to the
underlying pleural space. The antidepressant compounds
represent the primary pharmacological treatment for
myofascial pain syndrome. Tricyclic antidepressants are
thought to be more effective than selective serotonin reuptake inhibitors in the treatment of this painful condition.
The precise mechanism of action of the antidepressant
compounds in the treatment of myofascial pain syndrome
is unknown. Some investigators believe that the primary
effect of this class of drugs is to treat the underlying depression that is present in many patients who have myofascial pain syndrome. Drugs such as amitriptyline and
nortriptyline represent good first choices and should be
given as a single bedtime dose, starting with 10 to 25 mg
and titrating upward as side effects allow.

SUGGESTED READINGS
Arnold LM: The pathophysiology, diagnosis and treatment of fibromyalgia,
Psychiatr Clin North Am 33:375408, 2010.
Bradley LA: Pathophysiology of fibromyalgia, Am J Med 122(Suppl 1):S22S30,
2009.
Imamura M, Cassius DA, Fregni F: Fibromyalgia: from treatment to rehabilitation,
Eur J Pain Suppl 3:117122, 2009.
Shobeiri SA, Chesson RR, Gasser RF: The internal innervation and morphology of
the human female levator ani muscle, Am J Obstet Gynecol 199:686.e1686.e6,
2008.
Singh K, Reid WMN, Berger LA: Magnetic resonance imaging of normal levator
ani anatomy and function, Obstet Gynecol 99:433438, 2002.

SECTION 10 Hip and Lower Extremity Pain Syndromes

Chapter 91
AVASCULAR NECROSIS OF THE HIP
ICD-9 CODE 733.40
ICD-10 CODE M87.00
The Clinical Syndrome
Avascular necrosis of the hip is an often missed diagnosis. It is
also known as osteonecrosis. Similar to the scaphoid, the hip is
extremely susceptible to this disease because of its tenuous blood
supply. The blood supply of the hip is easily disrupted, often leaving the proximal portion of the bone without nutrition, thereby
leading to osteonecrosis. Avascular necrosis of the hip is a disease
of the fourth and fifth decades of life and is more common in
men, with an 8:1 male-to-female preponderance (Figure 91-1),
except for patients with avascular necrosis of the hip secondary to

collagen-vascular disease. The disease is bilateral in 50% to 55%


of cases.
Predisposing factors to avascular necrosis of the hip are listed
in Table 91-1 and include trauma to the proximal femur and
acetabulum; corticosteroid use; Cushings disease; alcohol abuse;
connective tissue diseases, especially systemic lupus erythematous; osteomyelitis; human immunodeficiency virus (HIV); organ
transplantation; Legg-Calv-Perthes disease; hemoglobinopathies,
including sickle cell disease; hyperlipidemia; gout; renal failure;
pregnancy; and radiation therapy involving the femoral head. A
patient with avascular necrosis of the hip reports pain over the
affected hip or hips, which may radiate into the groin, buttocks,
and proximal lower extremity. The pain is deep and aching,
and the patient often reports a catching sensation with range of
motion of the affected hip or hips. Range of motion decreases as
the disease progresses.

Signs and Symptoms


Physical examination of patients with avascular necrosis of the hip
reveals pain to deep palpation of the hip joint. The pain becomes
TABLE 91-1

Predisposing Factors for Avascular Necrosis of the Hip


Trauma to proximal femur and acetabulum
Corticosteroid use
Cushings disease
Alcohol abuse
Connective tissue diseases, especially systemic lupus erythematosus
Osteomyelitis
Human immunodeficiency virus
Organ transplantation
Legg-Calv-Perthes disease
Hemoglobinopathies, including sickle cell disease
Hyperlipidemia
Gout
Renal failure
Figure 91-1 Avascular necrosis of the hip is a disease of the fourth and
fifth decades of life and is more common in men, with an 8:1 male-tofemale ratio.

Pregnancy
Radiation therapy

265

266 SECTION 10 Hip and Lower Extremity Pain Syndromes

Figure 91-2 Osteonecrosis of the right hip in a 35-year-old man. A left hip replacement was performed because of advanced osteonecrosis and
secondary osteoarthritis. A, Coronal T1-weighted magnetic resonance imaging. A well-defined reactive rim surrounds a zone of osteonecrosis that
appears isointense with normal fatty marrow. B, Axial T2-weighted image. The region of femoral head osteonecrosis remains relatively isointense with
normal marrow. C, Coronal fat-saturated T2-weighted image. The double line sign represents an inner hyperintense component, corresponding with
hypervascular granulation tissue and fibrovascular proliferation, and an outer, hypointense, fibrotic band. (From Edelman RR, Hesselink JR, Zlatkin MB,
etal, editors: Clinical magnetic resonance imaging, 3rd ed, Philadelphia, 2006, Saunders, p 3369.)

worse with passive range of motion and weight bearing on a single


extremity. A click or crepitus may be appreciated by the examiner
when putting the hip joint through range of motion. A Trendelenburg gait may be noted, and decreased range of motion is
invariably present.

Testing
Plain radiographs are indicated in all patients with avascular
necrosis of the hip to rule out underlying occult bony pathological
processes and identify sclerosis and fragmentation of the femoral
head, although early in the course of the disease, plain radiographs are unreliable. Based on the patients clinical presentation,
additional tests, including complete blood cell count, uric acid,

erythrocyte sedimentation rate, and antinuclear antibody testing,


may be indicated. Magnetic resonance imaging (MRI) of the hip is
indicated in all patients suspected to have avascular necrosis of the
hip or if other causes of joint instability, infection, or tumor are
suspected (Figures 91-2 and 91-3). Administration of gadolinium
followed by postcontrast imaging may help delineate the adequacy
of blood supply, with contrast enhancement of the proximal hip
being a good prognostic sign. Electromyography is indicated if
coexistent lumbar radiculopathy, plexopathy, or both are suspected. A gentle injection of the hip joint with small volumes of
local anesthetic provides immediate improvement of the pain and
helps show the nidus of the patients pain is, in fact, the hip. Ultimately, total joint replacement is required in most patients with
avascular necrosis of the hip.

91 Avascular Necrosis of the Hip 267

occult metastatic disease also may mimic the pain of avascular


necrosis of the hip.

Treatment

Initial treatment of the pain and functional disability associated


with avascular necrosis of the hip should include a combination
of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2 (COX-2) inhibitors and decreased weight bearing of the
affected hip or hips. Local application of heat and cold may be
beneficial. For patients who do not respond to these treatment
modalities, an injection of a local anesthetic into the hip joint
may be a reasonable next step to provide palliation of acute pain.
Vigorous exercises should be avoided because they would exacerbate the patients symptoms. Ultimately, surgical repair in the
form of total joint arthroplasty is the treatment of choice.

Complications and Pitfalls


Failure to treat significant avascular necrosis of the hip surgically usually results in continued pain and disability and in most
patients leads to ongoing damage to the hip (see Figure 91-2).
Injection of the joint with local anesthetic is a relatively safe technique if the clinician is attentive to detail, specifically using small
amounts of local anesthetic and avoiding high injection pressures,
which may damage the joint further. Another complication of
this injection technique is infection. This complication should be
exceedingly rare if the clinician adheres to strict aseptic technique.
Approximately 25% of patients report a transient increase in pain
after this injection technique and should be warned of such.

Clinical Pearls

B
Figure 91-3 Osteonecrosis of the left hip with subchondral osseous collapse and osteoarthritis. A, Coronal T1-weighted magnetic resonance
imaging. Decreased marrow signal intensity is identified within the
superior lateral aspect of the left femoral head and the adjacent lateral
aspect of the acetabulum. Lateral subluxation of the femoral head also is
present. B, Coronal fat-saturated T2-weighted image. Flattening of the
articular surface of the superior lateral portion of the left femoral head
is evident, indicating subchondral osseous collapse. Increased marrow
signal intensity involving the lateral acetabulum and the proximal femur
is observed, which is compatible with reactive edema and fibrovascular
proliferation. A large left hip joint effusion also is present. (From Edelman
RR, Hesselink JR, Zlatkin MB, et al, editors: Clinical magnetic resonance
imaging, 3rd ed, Philadelphia, 2006, Saunders, p 3371.)

Differential Diagnosis
Coexistent arthritis and gout of the hip joints, bursitis, and tendinitis may coexist with avascular necrosis of the hips and exacerbate the pain and disability of the patient. Tears of the labrum,
ligament tears, bone cysts, bone contusions, bone fractures, and

Avascular necrosis of the hip is a diagnosis that is often


missed, leading to much unnecessary pain and disability.
The clinician should include avascular necrosis of the hip
in the differential diagnosis with all patients with hip pain,
especially if any of the predisposing factors listed in Table
91-1 are present. Coexistent arthritis, tendinitis, and gout
may contribute to the pain and may require additional treatment. The use of physical modalities, including local heat
and cold and decreased weight bearing, may provide symptomatic relief. Vigorous exercises should be avoided because
they would exacerbate the symptoms and may cause further
damage to the hip. Simple analgesics and NSAIDs may be
used concurrently with this injection technique.

SUGGESTED READINGS
Israelite CL, Garino JP: Osteonecrosis of the hip, Semin Arthroplasty 16:2732,
2005.
Malizos KN, Karantanas AH, Varitimidis SE, etal: Osteonecrosis of the femoral
head: etiology, imaging and treatment, Eur J Radiol 63:1628, 2007.
Waldman SD: Osteonecrosis of the hip. In Waldman SD, Campbell RSD, editors:
Imaging of pain, Philadelphia, 2011, Saunders, pp 339341.
Zibis AH, Karantanas AH, Roidis NT, etal: The role of MR imaging in staging
femoral head osteonecrosis, Eur J Radiol 63:39, 2007.

Chapter 92
PSOAS BURSITIS

ICD-9 CODE 727.3


ICD-10 CODE M71.50
The Clinical Syndrome
Psoas bursitis is an uncommon cause of hip and groin pain that is
frequently misdiagnosed in clinical practice. A patient with psoas
bursitis frequently reports pain in the groin. The pain is localized to
the area just below the crease of the groin anteriorly, with referred
pain noted into the hip joint. Often, the patient is unable to sleep
on the affected hip and may report a sharp, catching sensation with
range of motion of the hip.
The psoas muscle flexes the thigh on the trunk or, if the thigh is
fixed, flexes the trunk on the thigh as when moving from a supine
to a sitting position. This action can irritate the psoas bursa, as
can repeated trauma from repetitive activity, including running

up stairs or overuse of exercise equipment for lower extremity


strengthening (Figure 92-1). The psoas muscle is innervated by
the lumbar plexus. The psoas bursa lies medially in the femoral
triangle between the psoas tendon and the anterior aspect of the
neck of the femur. This bursa may exist as a single bursal sac or
in some patients may exist as a multisegmented series of sacs that
may be loculated in nature. The psoas bursa is vulnerable to injury
from acute trauma and repeated microtrauma. Acute injuries frequently take the form of direct trauma to the bursa from seat belt
injuries and from overuse injuries requiring repeated hip flexion,
such as javelin throwing and ballet. If the inflammation of the
psoas bursa becomes chronic, calcification of the bursa may occur.

Signs and Symptoms


Physical examination may reveal point tenderness in the upper
thigh just below the crease of the groin in patients with psoas bursitis. Passive flexion, adduction, and abduction and active resisted
flexion and adduction of the affected lower extremity reproduce
the pain. Sudden release of resistance during this maneuver

Psoas muscle
Psoas bursa

Figure 92-1 The psoas muscle flexes the thigh on the trunk or, if the thigh is fixed, flexes the trunk on the thigh as when moving from a supine to
a sitting position. This action can irritate the psoas bursa, as can repeated trauma from repetitive activity, including running up stairs or overuse of
exercise equipment for lower extremity strengthening.

268

92 Psoas Bursitis 269

markedly increases the pain. Examination of the hip is normal,


unless there is coexistent internal derangement of the hip.

nerve. These motor and sensory changes are limited to the distribution of the femoral nerve below the inguinal ligament. Ilioinguinal and genitofemoral neuropathy also can be confused with
psoas bursitis. Lumbar radiculopathy and these nerve entrapments
may coexist as the double crush syndrome. The pain of psoas
bursitis also may cause alteration of gait, which may result in secondary back and radicular symptoms that may coexist with this
entrapment neuropathy.

Testing
Plain radiographs of the hip may reveal calcification of the bursa
and associated structures consistent with chronic inflammation
(Figure 92-2). Magnetic resonance imaging (MRI) is indicated
if occult mass, abscess, or tumor of the hip or groin is suspected.
Complete blood cell count and automated chemistry profile,
including uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, are indicated if collagen-vascular disease
is suspected. Injection of the psoas bursa with a local anesthetic
and steroid serves as a diagnostic maneuver and a therapeutic
maneuver.

Treatment
Initial treatment of the pain and functional disability associated
with psoas bursitis should include a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) or cyclooxygenase-2
(COX-2) inhibitors and physical therapy. Local application of
heat and cold also may be beneficial. The repetitive movements
that incite the syndrome should be avoided. For patients who do
not respond to these treatment modalities, injection of the psoas
bursa with a local anesthetic and steroid may be a reasonable
next step.

Differential Diagnosis
Psoas bursitis is often misdiagnosed as an inguinal hernia or
attributed to a primary hip pathological process. Radiographs of
the hip and electromyography help distinguish psoas bursitis from
radiculopathy of pain emanating from the hip. Most patients
with lumbar radiculopathy have back pain associated with reflex,
motor, and sensory changes, whereas patients with psoas bursitis have only secondary back pain as a result of altered gait and
no neurological changes. Femoral diabetic neuropathy sometimes
may be confused with psoas bursitis, but can be distinguished by
the presence of motor and sensory changes involving the femoral

Complications and Pitfalls


The proximity to the femoral nerve of the psoas bursa makes it
imperative that the injection procedure be done only by clinicians
well versed in the regional anatomy and experienced in performing injection techniques. Many patients report a transient increase
in pain after injection of the bursa.

Figure 92-2 Tuberculous spondylitis: Psoas abscess. A, The typical appearance of bilateral and fusiform psoas abscesses is illustrated in a cross-
sectional drawing through a lumbar vertebral body. B, A large left noncalcified psoas abscess (arrows) can be seen. C, Diffusely calcified psoas
abscesses are noted in association with spinal abnormalities. (From Resnick D, editor: Diagnosis of bone and Joint disorders, 4th ed, Philadelphia, 2002,
Saunders, p 2530.)

270 SECTION 10 Hip and Lower Extremity Pain Syndromes

Clinical Pearls
It is important to rule out other causes of groin pain, including inguinal hernia and entrapment neuropathies of the
ilioinguinal, genitofemoral, and femoral nerves. Injection
of the psoas bursa is extremely effective in the treatment of
psoas bursitis. Special care must be taken to avoid trauma
to the femoral nerve.
The use of physical modalities, including local heat and
gentle stretching exercises, should be introduced several
days after the patient undergoes this injection technique.
Vigorous exercises should be avoided because they would
exacerbate the symptoms. Simple analgesics, NSAIDs, and
antimyotonic agents such as tizanidine may be used concurrently with injection of the bursa.

SUGGESTED READINGS
Ilizaliturri VM Jr, Camacho-Galindo J, Evia Ramirez AN, etal: Soft tissue pathology around the hip, Clin Sports Med 30:391415, 2011.
Patel K, Wallace R, Busconi BD: Radiology, Clin Sports Med 30:239283, 2011.
Valeriano-Marcet J, Carter JD, Vasey FB: Soft tissue disease, Rheum Dis Clin
North Am 29:7788, 2003.
Waldman SD: Injection technique for psoas bursitis. In Waldman SD, editor:
Pain review, Philadelphia, 2009, Saunders, pp 551552.

Chapter 93
FEMORAL NEUROPATHY

ICD-9 CODE 355.8


ICD-10 CODE G57.90
The Clinical Syndrome
Femoral neuropathy is an uncommon cause of anterior thigh and
medial calf pain that has many causes. Femoral neuropathy may
be due to compression by tumor, retroperitoneal hemorrhage, or
abscess. Stretch injuries to the femoral nerve as it passes under the
inguinal ligament from extreme extension or flexion at the hip
also may produce the symptoms of femoral neuropathy. Direct
trauma to the nerve from surgery or during cardiac catheterization and diabetes, which can produce vascular lesions of the nerve
itself, also can produce this clinical syndrome.
A patient with femoral neuropathy has pain that radiates into
the anterior thigh and midcalf and is associated with weakness
of the quadriceps muscle. This weakness can result in significant
functional deficit, with the patient unable to extend the knee fully,
which can allow the knee to buckle, resulting in inexplicable falls.
A patient with femoral neuropathy also may experience weakness
of the hip flexors, making walking up stairs quite difficult.

(MRI) of the spine and pelvis is indicated if tumor or hematoma


is suspected (Figure 93-3). Injection of the femoral nerve at the
femoral triangle serves as a diagnostic and therapeutic maneuver.

Differential Diagnosis
It is difficult to separate femoral neuropathy from an L4 radiculopathy on purely clinical grounds. Subtle differences may exist because
the L4 radiculopathy may manifest with sensory changes into the
foot and weakness of the dorsiflexors of the foot. Intrapelvic or retroperitoneal tumor or hematoma may compress the lumbar plexus
and mimic the clinical presentation of femoral neuropathy.

Treatment
Mild cases of femoral neuropathy usually respond to conservative therapy, and surgery should be reserved for more severe cases.
Femoral
nerve
Inguinal
ligament

Signs and Symptoms


The patient with femoral neuropathy has pain that radiates into
the anterior thigh and medial calf (Figure 93-1). This pain may be
paresthetic or burning; the intensity is moderate to severe. Weakness of the quadriceps muscle can be quite marked, and over time
atrophy of the quadriceps may occur, especially in patients with
diabetes (Figure 93-2). Patients with femoral neuropathy may
report a sunburned feeling over the anterior thigh. Patients also
may report that the knee feels like it is giving way.

Quadriceps
muscles

Testing
Electromyography can help identify the exact source of neurological dysfunction and clarify the differential diagnosis and should be
the starting point of the evaluation of all patients thought to have
femoral neuropathy. Plain radiographs of the spine, hip, and pelvis
are indicated in all patients with femoral neuropathy to rule out
occult bony pathological conditions. Based on the patients clinical
presentation, additional tests, including complete blood cell count,
uric acid level, erythrocyte sedimentation rate, and antinuclear
antibody testing, may be indicated. Magnetic resonance imaging

Figure 93-1 Patients with femoral neuropathy present with pain that
radiates into the anterior thigh and medial calf.

271

272 SECTION 10 Hip and Lower Extremity Pain Syndromes

Initial treatment of femoral neuropathy should consist of treatment with simple analgesics, nonsteroidal anti-inflammatory
drugs (NSAIDs), or cyclooxygenase-2 (COX-2) inhibitors and
avoidance of repetitive activities that exacerbate the symptoms.
If diabetes is thought to be the cause of the patients femoral
neuropathy, tight control of blood glucose levels is mandatory.
Avoidance of repetitive activities thought to be responsible for the
exacerbation of femoral neuropathy (e.g., repetitive hip extension
and flexion) also helps ameliorate the symptoms. If the patient
fails to respond to these conservative measures, a next reasonable
step is injection of the femoral nerve with a local anesthetic and
steroid.

Complications and Pitfalls


It is imperative that the clinician rule out causes of femoral neuropathy that, if undiagnosed, could harm the patient, such as
uncontrolled diabetes and retroperitoneal or pelvic tumor. The
main side effect of femoral nerve block is postblock ecchymosis
and hematoma. The potential exists for needle-induced trauma to
the femoral nerve. By advancing the needle slowly and then withdrawing the needle slightly away from the nerve, needle-induced
trauma to the femoral nerve can be avoided.

Clinical Pearls
Figure 93-2 Amyotrophy of the left quadriceps femoris. (From Jellad A,
Boudokhane S, Ezzine S, etal: Femoral neuropathy caused by compressive
iliopsoas hydatid cyst: a case report and review of the literature, Joint Bone
Spine 77:371372, 2010.)

Femoral neuropathy always should be differentiated from


lumbar plexopathy and radiculopathy of the nerve roots,
which sometimes may mimic femoral nerve compression.
Lumbar radiculopathy and femoral nerve entrapment
may coexist in the double crush syndrome. The double
crush syndrome is seen most commonly with median nerve
entrapment at the wrist.
Injection of the femoral nerve is a simple and safe technique in the evaluation and treatment of the previously
mentioned painful conditions. Careful neurological examination to identify preexisting neurological deficits that may
later be attributed to the nerve block should be performed
on all patients before beginning femoral nerve block, especially in patients with clinical symptoms of diabetes or clinically significant femoral neuropathy.

SUGGESTED READINGS

Figure 93-3 T2-Weighted coronal magnetic resonance imaging showing the hematoma as an area of increased signal intensity in the muscle belly (arrowhead). Fascial edema/hemorrhage is depicted as linear
hyperintensity. An ill-defined area of high signal intensity can be seen
at the distal myotendinous junction of the left psoasiliacus complex,
indicating a partial injury (arrow). (From Seijo-Martnez M, Castro del Ro
M, Fontoira E, Fontoira M: Acute femoral neuropathy secondary to an iliacus
muscle hematoma, J Neurol Sci 209:119122, 2003.)

Busis NA: Femoral and obturator neuropathies, Neurol Clin 17:633653, 1999.
Hsin HT, Hwang JJ: Isolated femoral nerve neuropathy after intra-aortic balloon
pump treatment, J Formos Med Assoc 106:S29S32, 2007.
Parmer SS, Carpenter JP, Fairman RM, etal: Femoral neuropathy following retroperitoneal hemorrhage: case series and review of the literature, Ann Vasc Surg
20:536540, 2006.
Seijo-Martnez M, Castro del Ro M, Fontoira E, Fontoira M: Acute femoral neuropathy secondary to an iliacus muscle hematoma, J Neurol Sci 209:119122,
2003.

Chapter 94
SAPHENOUS NEURALGIA

ICD-9 CODE 355.8


ICD-10 CODE G57.90
The Clinical Syndrome
Saphenous neuralgia is an uncommon cause of medial calf pain
that may occur after vascular surgery on the lower extremity
(Figure 94-1). With the increased number of total knee arthroplasties being performed, trauma to the infrapatellar branch of
the saphenous nerve may cause damage, producing pain and
numbness over the patellar tendon. Patients with saphenous
neuralgia often experience the medial pseudoclaudication type
of pain that may confuse the clinical evaluation and lead the
clinician to suspect lumbar spinal stenosis. Saphenous neuralgia
also may be due to compression of the nerve by tumor, hemorrhage, or abscess. This compression usually occurs at the level
at which the nerve exits from Hunters canal. Stretch injuries to
the saphenous nerve also can occur at this point. The nerve is
subject to compression as it crosses to the medial knee. Compression of the saphenous nerve at the knee is known as surfers knee
because of compression of the saphenous nerve by the edge of
the surfboard. Diabetes can affect the saphenous nerve, but this
is usually in conjunction with neuropathy of the other nerves of
the lower extremity.

Signs and Symptoms


A patient with saphenous neuralgia presents with pain that radiates into the medial calf to the medial malleolus (Figure 94-2).
This pain may be paresthetic or burning; the intensity is moderate
to severe. There is no motor deficit associated with saphenous neuropathy, unless the spinal nerve roots or plexus or other peripheral
nerves are involved. Patients with saphenous neuralgia may report
a sunburned feeling over the distribution of the saphenous nerve.

Testing
Electromyography can help identify the exact source of neurological dysfunction and clarify the differential diagnosis and should
be the starting point of the evaluation of all patients suspected
to have saphenous neuralgia. Plain radiographs of the spine, hip,
pelvis, and femur are indicated in all patients who present with
saphenous neuralgia to rule out occult bony pathological processes. Based on the patients clinical presentation, additional

tests, including complete blood cell count, uric acid level, erythrocyte sedimentation rate, and antinuclear antibody testing, may be
indicated. Magnetic resonance imaging (MRI) of the spine, pelvis,
and proximal lower extremity is indicated if tumor or hematoma
is suspected. Injection of the saphenous nerve with a local anesthetic and steroid as it exits Hunters canal serves as a diagnostic
and therapeutic maneuver.

Differential Diagnosis
It is difficult to separate saphenous neuralgia from a lumbar radiculopathy on purely clinical grounds, and electromyography is
strongly recommended. Electromyography and nerve conduction
testing also help rule out the presence of peripheral neuropathy.
Intrapelvic or retroperitoneal tumor or hematoma may compress
the lumbar plexus and mimic the clinical presentation of saphenous neuralgia.

Treatment
Mild cases of saphenous neuralgia usually respond to conservative
therapy, and surgery should be reserved for more severe cases. Initial treatment of saphenous neuralgia should consist of treatment
with simple analgesics, nonsteroidal anti-inflammatory drugs
(NSAIDs), or cyclooxygenase-2 (COX-2) inhibitors and avoidance of repetitive activities that exacerbate the symptoms. If diabetes is thought to be the cause of the patients saphenous neuralgia,
tight control of blood glucose levels is mandatory. Avoidance of
repetitive activities thought to be responsible for the exacerbation
of saphenous neuralgia helps ameliorate the symptoms. The use
of gabapentin or a tricyclic antidepressant such as nortriptyline
as an adjuvant analgesic also may help ameliorate the symptoms
of saphenous neuralgia. If the patient fails to respond to these
conservative measures, a next reasonable step is injection of the
saphenous nerve with a local anesthetic and steroid. Ultrasound
guidance may be useful in patients in whom anatomical landmarks are difficult to identify (Figure 94-3).

Complications and Pitfalls


It is imperative that the clinician rule out causes of saphenous
neuralgia that, if undiagnosed, could harm the patient, such as
uncontrolled diabetes and retroperitoneal or pelvic tumor. The
main side effect of saphenous nerve block is postblock ecchymosis
and hematoma. Potential exists for needle-induced trauma to the
saphenous nerve. By advancing the needle slowly and then withdrawing the needle slightly away from the nerve, needle-induced
trauma to the saphenous nerve can be avoided.
273

274 SECTION 10 Hip and Lower Extremity Pain Syndromes

Sartorius

Gracilis

SSV

GSV

Medial condyle
of femur

Femur

Tibia

Tibia

D
Figure 94-1 Axial T1-weighted (A) and PD-weighted fat-suppressed (B) images, sagittal PD-weighted fat-suppressed (C) and ultrasound (arrows
delineate postsurgical neuroma) (D) images of a right knee. Postsurgery neuroma of the sartorial branch of the saphenous nerve at the point where
it becomes superficial between the sartorius (S) and gracilis (G) tendons. GSV, Great saphenous vein; SSV, small saphenous vein. (From Damarey B,
Demondion X, Wavreille G, etal: Imaging of the nerves of the knee region, Eur J Radiol 82:27-37, 2013.)

94 Saphenous Neuralgia 275

Saphenous
nerve

Patellar
tendon

Medial
malleolus

Figure 94-2 Patients with saphenous neuralgia present with pain that radiates into the medial calf to the medial malleolus.

Local anesthetic

Anterior

Posterior

Sartorius muscle

A
Vastus medialis
muscle

Needle
tip

Femoral
artery

Subsartorial plexus

Anterior

Posterior

Sartorius muscle

Subsartorial
plexus

B
Vastus medialis
muscle

Local
anesthetic

Femoral
artery

Figure 94-3 Image sequence showing subsartorial block of the saphenous nerve in the midthigh with the sartorius muscle and subsartorial plexus
imaged in short-axis view. An in-plane approach is demonstrated in which the needle tip is placed through the sartorius muscle, targeting the fascial
plane on the anterior side of the femoral artery (A). In this example, after injection, the local anesthetic is distributed around a single nerve complex
underneath the sartorius muscle (B). (From Gray AT: Atlas of ultrasound-guided regional anesthesia, Philadelphia, 2010, Saunders, p 158.)

276 SECTION 10 Hip and Lower Extremity Pain Syndromes

Clinical Pearls
Saphenous neuralgia always should be differentiated from
lumbar plexopathy and radiculopathy of the nerve roots,
which may sometimes mimic saphenous nerve compression.
Lumbar radiculopathy and saphenous nerve entrapment
may coexist in the double crush syndrome. The double
crush syndrome is seen most commonly with median nerve
entrapment at the wrist.
Injection of the saphenous nerve is a simple and safe
technique in the evaluation and treatment of the aforementioned painful conditions. Careful neurological examination to identify preexisting neurological deficits that later
may be attributed to the nerve block should be performed
on all patients before beginning saphenous nerve block,
especially in patients with clinical symptoms of diabetes or
clinically significant saphenous neuralgia.

SUGGESTED READINGS
Dayan V, Cura L, Cubas S, Carriquiry G: Surgical anatomy of the saphenous
nerve, Ann Thorac Surg 85:896900, 2008.
Iizuka M, Yao R, Wainapel S: Saphenous nerve injury following medial knee joint
injection: a case report, Arch Phys Med Rehabil 86:20622065, 2005.
Kalenak A: Saphenous nerve entrapment, Oper Tech Sports Med 4045, 1996.
Mountney J, Wilkinson GAL: Saphenous neuralgia after coronary artery bypass
grafting, Eur J Cardiothorac Surg 16:440443, 1999.
Waldman SD: Saphenous nerve block at the knee. In Waldman SD, editor: Pain
review, Philadelphia, 2009, Saunders, pp 573574.

Chapter 95
OBTURATOR NEURALGIA

ICD-9 CODE 355.8


ICD-10 CODE G57.90
The Clinical Syndrome
Obturator neuralgia is an uncommon cause of medial thigh pain
that does not extend below the knee and occurs most often after
trauma. Pelvic fractures, gunshot wounds, and occasionally childbirth have been implicated in the evolution of obturator neuralgia. With the increased number of total hip arthroplasties being
performed, trauma to the branches of the obturator nerve may
occur, producing pain and numbness over the medial thigh.
Obturator neuralgia also may be due to compression of the nerve
by tumor, hemorrhage, bone cement from total hip arthroplasties, endometriosis, or abscess. Stretch injuries to the obturator
nerve can cause the symptoms of obturator neuralgia. Diabetes
can affect the obturator nerve, but this is usually in conjunction
with neuropathy of the other nerves of the lower extremity, especially the femoral nerve.

Signs and Symptoms

Differential Diagnosis
It is sometimes difficult to separate obturator neuralgia from a
lumbar plexopathy or radiculopathy on purely clinical grounds,
and electromyography is strongly recommended. Electromyography and nerve conduction testing also help rule out the presence
of peripheral neuropathy. Intrapelvic or retroperitoneal tumor or
hematoma may compress the lumbar plexus and mimic the clinical presentation of obturator neuralgia (Figure 95-3).

Treatment
Mild cases of obturator neuralgia usually respond to conservative
therapy, and surgery should be reserved for more severe cases. Initial treatment of obturator neuralgia should consist of treatment
with simple analgesics, nonsteroidal anti-inflammatory drugs
(NSAIDs), or cyclooxygenase-2 (COX-2) inhibitors and avoidance

Obturator
nerve

A patient with obturator neuralgia presents with pain that radiates


into the medial thigh and, except in rare patients, does not extend
below the knee (Figure 95-1). This pain may be paresthetic or
burning, and the intensity is moderate to severe. No significant
feeling of sunburn over the distribution of the obturator nerve has
been reported.

Testing
Electromyography can help identify the exact source of neurological dysfunction and clarify the differential diagnosis and should
be the starting point of the evaluation of all patients thought to
have obturator neuralgia. Plain radiographs of the spine, hip, pelvis, and proximal femur are indicated in all patients with obturator neuralgia to rule out occult bony pathology. Based on the
patients clinical presentation, additional tests, including complete blood cell count, uric acid level, erythrocyte sedimentation
rate, and antinuclear antibody testing, may be indicated. Magnetic resonance imaging (MRI) of the spine, pelvis, and proximal
lower extremity is indicated if tumor or hematoma is suspected
(Figure 95-2). Injection of the obturator nerve with a local anesthetic and steroid serves as a diagnostic and therapeutic maneuver.

Figure 95-1 Patients with obturator neuralgia have pain that radiates
into the medial thigh and does not extend below the knee.

277

Sigmoid colon

Endometriosis tumor in
right obturator fossae

Left obturator nerve

Figure 95-2 Transversal magnetic resonance image of the pelvis, with entrapment of the obturator nerve on the right side. (From Langebrekke A,
Qvigstad E: Endometriosis entrapment of the obturator nerve after previous cervical cancer surgery, Fertil Steril 91:622623, 2009.)

Figure 95-3 Skeletal metastasis: Medulloblastoma. A, Radiograph of the pelvis was obtained in a 23-year-old woman 2 years after a craniotomy with
excision of a medulloblastoma. Patchy osteosclerosis is evident in the left iliac crest, right acetabulum, symphyseal regions, ischial tuberosities, and
left femoral neck. B, After the removal of a medulloblastoma in a 20-year-old man, extensive osteoblastic metastases developed in the spine and,
as shown here, throughout the pelvic bones and proximal portions of the femora. C, In a 12-year-old boy who had undergone excision of a medulloblastoma, widespread osteoblastic skeletal metastases developed, shown here in the tubular bones of the lower extremity. (From Resnick D, editor:
Diagnosis of bone and joint disorders, 4th ed, Philadelphia, 2002, Saunders, p 4313.)

95 Obturator Neuralgia 279

of repetitive activities that exacerbate the symptoms. If diabetes is


thought to be the cause of the patients obturator neuralgia, tight
control of blood glucose levels is mandatory. Avoidance of repetitive activities thought to be responsible for the exacerbation of
obturator neuralgia also helps ameliorate the symptoms. The use
of gabapentin or a tricyclic antidepressant such as nortriptyline as
an adjuvant analgesic also may help ameliorate the symptoms of
obturator neuralgia. If the patient fails to respond to these conservative measures, a reasonable next step is injection of the obturator nerve with a local anesthetic and steroid.

Complications and Pitfalls


It is imperative that the clinician rule out causes of obturator
neuralgia that, if undiagnosed, could harm the patient, such as
uncontrolled diabetes and retroperitoneal or pelvic tumor. The
main side effect of obturator nerve block is postblock ecchymosis
and hematoma. Potential exists for needle-induced trauma to the
obturator nerve. By advancing the needle slowly and then withdrawing the needle slightly away from the nerve, needle-induced
trauma to the obturator nerve can be avoided.

Clinical Pearls
Obturator neuralgia always should be differentiated from
lumbar plexopathy and radiculopathy of the nerve roots
that may sometimes mimic obturator nerve compression.
Lumbar radiculopathy and obturator nerve entrapment
may coexist in the double crush syndrome. The double
crush syndrome is seen most commonly with median nerve
entrapment at the wrist.
Injection of the obturator nerve is a simple and safe
technique in the evaluation and treatment of the previously
mentioned painful conditions. Careful neurological examination to identify preexisting neurological deficits that may
later be attributed to the nerve block should be performed
on all patients before beginning obturator nerve block,
especially in patients with clinical symptoms of diabetes or
clinically significant obturator neuralgia.

SUGGESTED READINGS
Cardosi RJ, Cox CS, Hoffman MS: Postoperative neuropathies after major pelvic
surgery, Obstet Gynecol 100:240244, 2002.
Langebrekke A, Qvigstad E: Endometriosis entrapment of the obturator nerve
after previous cervical cancer surgery, Fertil Steril 91:622623, 2009.
Toth C: Peripheral nerve injuries attributable to sport and recreation, Phys Med
Rehabil Clin North Am 20:77100, 2009.
Toussaint CP, Perry EC III,