Otolaryngol Clin N Am

37 (2004) 435–451

Isolated sphenoid sinus disease

Gregory A. Grillone, MDa,*, Peter Kasznica, BAb
a
The Department of Otolaryngology–Head and Neck Surgery, Boston University School
of Medicine, 88 East Newton Street, Boston, MA 02118, USA
b
Boston University School of Medicine, 88 East Newton Street, Boston, MA 02118, USA

The sphenoid sinus has often been referred to as the neglected sinus
because of its isolated position and difficult accessibility. Disease restricted
to the sphenoid sinus is rare and often manifests with nonspecific or subtle
signs and symptoms. The early and accurate diagnosis of isolated sphenoid
sinus disease may thus be difficult. Typically, patients are referred to the
otolaryngologist because of a finding of isolated sinus opacification on CT
scans ordered by the patient’s primary care physician or neurologist to
evaluate vague symptoms such as headache. Occasionally, the opacified
sinus is an incidental finding on CT scans performed for unrelated reasons.
In either case, the otolaryngologist must have a thorough knowledge of the
clinical spectrum of sphenoid sinus disease and the radiologic characteristics
to evaluate and manage these patients properly.

Embryology and anatomy of the sphenoid sinuses

Thorough knowledge of the embryology and anatomy of the sphenoid
sinus is a prerequisite for the understanding of its various disease processes
and their treatment. The sphenoid sinus develops in the third month of
embryologic life. The predecessor of the sphenoid sinus is the cartilaginous
cupolar recess of the nasal cavity. The cartilaginous cupolar recess is a pouch
formed when the nasal mucosa invaginates into the nasal capsule. The
ossiculum Bertini is the wall of the cartilage that becomes partially ossified
in subsequent months. In the second and third years of life the cartilage
becomes resorbed, and the ossiculum Bertini is fused to the body of the
sphenoid. Pneumatization of the sinus occurs in the sixth or seventh year of
life. The anterior clinoid processes and the pterygoid process can also

* Corresponding author.
E-mail address: Gregory.Grillone@bmc.org (G.A. Grillone).

0030-6665/04/$ - see front matter Ó 2004 Elsevier Inc. All rights reserved.
doi:10.1016/S0030-6665(03)00160-9
436 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451

become pneumatized by the twelfth year. The sphenoid sinus reaches its
final size and shape between nine and twelve years of age.
The sphenoid sinuses are located in the center of the skull. They are
found within a bony shell known as the sphenoidal concha that is anterior
and lateral to the body of the sphenoid bone. The sinuses occupy a midline
position in the coronal plane, and they are separated by an intersinus
septum. The position of the sphenoid sinus septum varies greatly, and it can
often be found well off the midline (Fig. 1). The ostium occupies a medial
and superior position on either side of the nasal septum, where it commu-
nicates with the nasal cavity.
The internal carotid artery lies on the lateral aspect of the sphenoid sinus
complex. It emerges from the carotid canal and tracks medially over the
foramen lacerum and then superiorly along the wall of the sphenoid sinus. It
courses through the cavernous sinus to reach the meningohypophyseal
trunk. The cavernous sinus and its contents, the oculomotor, trochlear,
abducens, and V1 and V2 branches of the trigeminal nerve also lie along the
lateral border of the sphenoid sinus. The optic nerve lies on the surface of

Fig. 1. Coronal CT of the sphenoid sinus showing an offset intersinus septum.

 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451 437

the sphenoidal concha, and it can occasionally indent the sinus itself in well-
pneumatized specimens with thin walls. The bone of the lateral wall of the
sphenoid sinus that overlies the optic nerve and internal carotid artery can
occasionally be dehiscent.
A sphenoethmoid cell, known as the Onodi cell, occurs when the most
posteriorly located ethmoid cell is highly pneumatized and extends
posteriorly toward the anterior border of the sphenoid sinus. It follows
the lamina papyracea of the medial orbital wall en route to the sphenoid
sinus. The overall result is a seeming continuity of the ethmoid and sphenoid
sinuses. The internal carotid artery and the optic nerve may lie in close
proximity to the Onodi cell and be prone to damage in cases of trauma or
surgical manipulation.

Classification of sphenoid sinus disease

Isolated sphenoid sinus disease can be broadly divided into the
inflammatory, neoplastic, and miscellaneous categories. The inflammatory
processes include acute and chronic sinusitis, mucoceles, and fungal
infections. The neoplastic lesions include the anatomically intrinsic
(primary) and extrinsic (secondary) tumors. Neoplastic lesions can be
further subdivided according to their primary anatomic site, malignant
potential, and embryologic tissue derivation. Fibro-osseous disorders also
fall in this general category. Cerebrospinal fluid leaks and encephaloceles
account for most of the miscellaneous disorders (Box 1).

Clinical signs and symptoms of isolated sphenoid sinus disease

In general, the symptoms of sphenoid sinus disease tend to be variable
and nonspecific. The various pathologic processes that affect the sphenoid
sinus share many of the same symptoms. Headaches, visual compromise,
and other nonspecific complaints that expand rather than focus the
differential diagnosis are typical (Table 1). Unless the disease process (eg,
neoplastic or fungal disease) disrupts the anatomy of the surrounding bone,
the patient seldom reports symptoms related to the structures surrounding
the sphenoid sinus. Therefore, it is unlikely to see patients with cranial nerve
palsies or vascular compromise as the initial or sole symptom.
The most common symptom of sphenoid sinus disease is headache. In
fact, many cases of sphenoid sinus disease are discovered on imaging studies
ordered for the evaluation of headache. The headache can be nonspecific in
location and quality, and thus the physician must maintain a high index of
suspicion. Pain originating in the sphenoid sinuses can be localized to the
vertex, occipital, posterior parietal, retro-orbital area, or other areas of the
head and face. The pain sensation is carried by the afferent nerve fibers
through the sphenopalatine ganglion and trigeminal nerve and ultimately
Box 1. Classification of sphenoid sinus disease
Inflammatory lesions
Sinusitis
Acute
Chronic
Fungal infections
Invasive: Mucormycoses, disseminated Aspergillosis
Noninvasive: Mycetoma, Aspergilloma
Mucoceles
Pyoceles
Neoplastic lesions
Benign
 Intrinsic:
1. Inverting papillomas
2. Myofibroma
3. Schwannoma
4. Osteochondroma
5. Fibro-osseous disorders (fibrous dysplasia, ossifying
fibroma)
 Extrinsic:
1. Meningioma
2. Paraganglioma
3. Pituitary macroadenomas
Malignant
 Primary:
1. Squamous cell carcinoma
2. Adenocarcinoma
3. Adenoid cystic carcinoma
4. Mucoepidermoid carcinoma
5. Undifferentiated carcinoma
6. Transitional cell carcinoma
 Metastatic:
1. Renal cell carcinoma
2. Thyroid carcinoma
3. Prostate adenocarcinoma
4. Breast carcinoma
5. Lung carcinoma
6. Melanoma
7. Multiple myeloma and lymphoma
Miscellaneous
Cerebrospinal leaks
 Traumatic
 Spontaneous
Encephaloceles
 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451 439

Table 1
Symptoms and signs of sphenoid sinus disease
Cranial
Study Cases Pathology Headache Nerve Deficit Imaging
Wyllie et al [14] n=45 Inflammatory, 37 77% 40% Plain films
Tumor, 8
Levine et al [15] n=11 Inflammtory, 9 81% 72% Plain films
Tumor, 2
Lew et al [16] n=30 Unspecified Majority 33% CT scan
Pearlman et al [17] n=43 Inflammatory, 29 89% 24% CT scan
Tumor, 13 61% 69%
Rothfield et al [18] n=13 Inflammatory, 6 50% 16% CT scan
Tumor, 7 28% 42%
Gilain et al [19] n=12 Unspecified 66% 50% CT scan/MRI
Lawson et al [2] n=132 Inflammatory, 80 98% 12% CT scan/MRI
Malignant, 28 71% 57%
Benign, 10 90% 60%
Ruoppi et al [3] n=39 Inflammatory, 39 82% 21% CT scan
Martin et al [1] n=29 Inflammatory, 19 69% Not specified CT scan/MRI
Tumor, 7
CSF fistula, 3
Adapted from Lawson W, Reino AJ. Isolated sphenoid sinus disease: an analysis of 132
Cases. Laryngoscope 1997;107(12):1590–95; with permission.

through the trigeminothalamic pathway. Visual disturbance is the second

most frequently reported symptom. Nasal obstruction may also occur and is
often associated with the formation of nasal polyps. Smell and taste can also
be compromised as part of the symptom spectrum for chronic sinusitis.
Martin et al [1] in their review of 29 cases of isolated sphenoid sinus
disease found headache (either retro-orbital or occipital) in 69% of the
patients, facial pain in 17%, decreased vision or diplopia in 21%, purulent
rhinorrhea in 10%, and unilateral nasal obstruction in 24%. Other
miscellaneous signs and symptoms included meningitis, cerebrospinal fluid
(CSF) rhinorrhea, and endocrine abnormalities.
Lawson and Reino [2] reviewed 132 cases of isolated sphenoid sinus
disease and also found headache to be the most common symptom.
Headache occurred in 98% of the patients who had inflammatory processes
and in 76% of those who had neoplasia. The authors also distinguished
benign from the malignant neoplasia with respect to their presenting
symptoms and found that 90% of patients with benign tumors presented
with headache, versus 71% with malignant tumors. The next most frequent
symptom was visual compromise, which included blurred vision and loss of
visual acuity ranging from mild loss to partial blindness. Visual compromise
occurred in 12% of inflammatory cases, 60% of benign neoplasms, and 50%
of the malignant cases. Cranial nerve palsies were also analyzed. Diplopia
caused by involvement of the abducens nerve was the most common of these
cranial palsies, occurring in 6% of inflammatory cases and in 50% of
440 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451

neoplastic disease. Other cranial nerve palsies included oculomotor nerve

dysfunction that caused ptosis in 7.5% of the patients. All but one case
resulted from neoplastic disease, most of which were malignant lesions. The
sole inflammatory case was a mucocele. Trigeminal nerve involvement is
characterized by retro-orbital pain and midfacial pain or numbness. This
symptom occurred in only 5% of the study group. The degree of anatomic
distortion needed to cause this nerve palsy usually happens only in the more
invasive processes, such as neoplasm, aggressive fungal infections, and long-
standing mucoceles. Other less common signs and symptoms reported in this
study included CSF leak caused by clival cysts, bony dehiscence, neoplasm,
or encephalocele in six cases and epistaxis secondary to trauma, aneurysm,
or malignant tumor in five patients. Meningitis was the presenting diagnosis
in three patients. Proptosis was seen in two patients, one with metastatic
carcinoma and another with a fibro-osseous disorder. Although fibro-
osseous disorders such as fibrous dysplasia and ossifying fibroma originating
in the sphenoid sinus may cause cranial nerve palsies and manifestations of
bony deformation (proptosis), their most common manifestation in this
study was headache, seen in all four patients with fibro-osseous disorders.
Ruoppi and colleagues [3] also observed a similar pattern in the signs and
symptoms of isolated sphenoid sinus disease. In a review of 39 patients with
inflammatory sinus disease, they found that the most prevalent symptom
was headache, affecting 82% of patients. Eye symptoms, which included
diplopia, decreased visual acuity, and even blindness, were evident in 28%
of patients. Dizziness was also a common complaint, occurring in 26% of
patients. Other symptoms reported were trigeminal nerve deficit in 5%, and
oculomotor palsy in 3%. Table 1 summarizes the signs and symptoms of the
major series reviewing sphenoid sinus disease.

Acute and chronic sphenoid sinusitis

The most common abnormality encountered in the sphenoid sinus is
infection. Infection and subsequent inflammation in otherwise healthy
individuals are typically caused by insufficient clearing of mucus from the
sphenoid sinus secondary to obstruction of the ostium. Other patients at risk
include those with compromised immunity, such as diabetics, transplant
patients, and individuals receiving chemotherapy or chronic steroid therapy.
Like inflammatory disease in the other sinuses, sphenoid sinusitis most
often occurs following a viral upper respiratory infection. Contributing
factors may include pharyngitis, odontogenic infections, recurrent adeno-
tonsillitis, and a host of anatomic and mechanical obstructions (eg, foreign
bodies, nasal septal deviation, polyps, allergic hypertrophy of turbinates,
pneumatized middle turbinate, and tumors of the nasal cavity). Lifestyle and
physical agents also play a role in the pathogenesis of sphenoid sinusitis.
Pressure and hydrostatic forces can cause accumulation of secretions in the
 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451 441

sinuses. Packing of the nose to control epistaxis, changes in atmospheric

pressure, deep-sea diving, Valsalvogenic maneuvers, nose blowing, or the
presence of a polyp adjacent to the ostium all serve physically to retain the
secretions within the sinus cavity.
Acute sphenoid sinusitis is often characterized by recent onset of severe
pain, aching or dull in quality, in the retro-orbital area, the occiput, or the
vertex. There may be associated nasal congestion and a heavy feeling local-
ized to the center of the head. Purulent material can be secreted and
swallowed through the nasopharynx and can lead to complaints of a foul
taste. Loss of appetite and decreased sense of smell can also occur. Chronic
sphenoid sinusitis is usually caused by incomplete resolution of acute sinu-
sitis, which may occur following inadequate medical management of the
latter condition. The symptoms of chronic sinusitis may be more subtle and
vague than for acute sinusitis. Sometimes, mild daily headaches are the only
sign of disease. In other cases, asymptomatic disease progression is pos-
sible. Incidental finding of opacification of the sphenoid sinus on CT
imaging may be the first indication of chronic sphenoid sinusitis.
Complications of sphenoid sinusitis are rare but can have devastating
consequences. Orbital cellulitis or abscess, when it occurs, presents with
symptoms and signs that include edema of the eyelids and periorbital soft
tissues. Osteomyelitis and epidural abscess can also occur following sphenoid
sinusitis. The epidural abscess is characterized by symptoms of severe
headaches, stiff neck, and obtundation. Thrombosis of the cavernous sinus is
a classic, but rare, complication of acute sphenoid sinusitis. Typical findings
include high spiking fever, numbness along the distribution of the
ophthalmic and maxillary divisions of the trigeminal nerve, oculomotor
nerve palsy, and pulsatile proptosis. Antibiotic therapy and surgical drainage
of the involved sinuses need to be implemented to treat this often fatal
complication. Intracerebral abscess is another complication that manifests
with drowsiness, confusion, seizures, and nausea and vomiting. Drainage of
the abscess is required.

Mucocele
Mucoceles are a consequence of long-standing sinusitis and arise when
any lesion prevents the normal egress of mucus from a sinus cavity. Primary
mucoceles occur as a result of secretory duct obstruction of a single mucus
gland causing formation of a mucus retention cyst. Secondary mucoceles are
the outcome of obstruction of the natural ostium of the sinus. This latter
type is more common and arises secondary to trauma, surgery, or inflam-
mation. Accumulating secretions expand the cavity, ultimately compressing
surrounding structures [4].
Histologically, mucoceles are lined with normal respiratory mucosa, al-
though the epithelium may undergo cuboidal or stratified squamous metapla-
sia as the sinusitis evolves [5]. Fluid encountered at the time of surgery is
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commonly viscid, grayish green, brown, or yellow, and is most often sterile.
Rarely, mixed anaerobic organisms are found [6].
Sphenoid sinus mucoceles account for only 1% of all mucoceles of the
paranasal sinuses [6]. In 1970 Nugent et al [4] reviewed the world literature
and reported on 63 cases. There was an almost equal distribution between
men and women, with a mean age of 42 years.
Sphenoethmoidal mucoceles can expand in three possible directions:
anteriorly toward the nasal region, laterally toward the orbit, and superiorly
toward the anterior cranial fossa (Fig. 2). Symptoms occur when the
mucocele impinges on one or more of the critical surrounding structures:
cranial nerves II to VI, the dura mater, the anterior cranial fossa, the
pituitary gland, the cavernous sinus, the internal carotid artery, the pterygoid
canal and nerve, the sphenopalatine artery, and the sphenopalatine ganglion
[5]. Mucoceles often remain asymptomatic for years, however, and thus
diagnosis and treatment may be delayed.
Headache is the most common symptom, as was reported in the studies
mentioned previously, and is a result of the upward stretching of the dura in
the region of the planum sphenoidale and floor of the anterior cranial fossa
[4]. Referred pain is carried by the posterior ethmoid branch of the
ophthalmic division of the trigeminal nerve that supplies the sphenoid sinus
mucosa. Visual disturbance, which includes oculomotor palsies and optic
nerve atrophy leading to blindness, is seen in many patients. Pulsatile
exophthalmos and conjunctival hyperemia are also possible, but these
symptoms almost never precede the headache [7]. Nasal symptoms are much
less frequent, but 25% of patients report nasal stuffiness, rhinorrhea, or
anosmia according to Wurster and colleagues [5].

Fungal infections
Fungal infections within the sphenoid sinus are most often mycetomas,
which are fungal balls containing Aspergillus spp. A mycetoma is
a noninvasive fungal infection containing a mass of tangled hyphae of
Aspergillus fumigatus but can also contain Pseudallescheria boydii or
Alternaria spp. Patients who get mycetomas of the sphenoid sinus usually
have intact immunity. Symptoms of mycetoma are similar to those of
chronic sinusitis. The CT imaging often shows opacification, bony sclerosis,
and central calcification within the lesion (Fig. 3).
Fungal infection can also be invasive. Acute fungal sinusitis usually
occurs in immunocompromised hosts, such as diabetics and those who have
undergone organ transplantation, whereas its chronic counterpart is more
often a disease of immunocompetent patients. Acute fungal sinusitis is
caused by members of the family Mucoraceae, with Rhizopus oryzae
accounting for most cases. Chronic fungal sinusitis is caused by Aspergillus,
Mucor, Alternaria, Curvularia, Candida, or other genera. The most com-
mon symptoms of the acute form are rapidly progressive necrosis of the
 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451 443

Fig. 2. Coronal view of an MRI (T1-weighted image) showing a massive mucocele of the
sphenoid sinus. This patient presented with blurred vision and severe temporal headache.

soft tissues of the nose, sinuses, and surrounding areas, vision impairment,
and ophthalmoplegia. Aggressive management, including wide débridement,
systemic antifungal therapy, and hyperbaric oxygen is necessary to avoid
the often fatal outcome of this disease. The chronic form of the disease is
characterized by nasal congestion and polypoid mucosa. Radiographic evi-
dence of the bony destruction often lags behind the clinical progression of
the disease. Sinus aspirates or tissue biopsy may be helpful in confirming the
diagnosis.
444 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451

Fig. 3. Coronal CT image showing an Aspergillus mycetoma in the left sphenoid sinus. Note
the marked sclerosis of the surrounding bone.

Fungal mucoceles may also occur in the sphenoid sinus, and they form in
much the same way as mucoceles of bacterial origin. Fungal mucoceles are
typically noninvasive [8].

Neoplasms of the sphenoid sinus

A variety of benign and malignant tumors can be found within the
sphenoid sinuses (see Box 1).
Benign tumors of the sphenoid sinuses include inverting papilloma,
meningioma, osteoma, hemangioma, chordoma, giant-cell tumor, and many
other less common types. Similar to malignant neoplasms, they originate
from many different tissues and have varying morphologies. These tumors
are not invasive but often require surgical management because of their
mass effect.
 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451 445

Primary malignant tumors of the sphenoid sinuses include squamous cell

carcinoma, adenocarcinoma, salivary gland tumors (adenoid cystic carci-
noma), melanoma, esthesioneuroblastoma, sarcoma, lymphoma, multiple
myeloma, and others. In general, fewer than 1% of malignant paranasal
sinus tumors originate in the sphenoid sinus. Most cases present in the fifth
to seventh decades of life, and there is higher incidence in men. The
prognosis is generally poor for most histologic types, largely because of
difficulty in achieving complete excision of these lesions.
Rarely, the sphenoid sinus may be the site of metastatic lesions. The most
common metastatic lesion encountered is renal cell carcinoma, followed, in
order of decreasing frequency, by carcinoma of the thyroid, prostate, lung,
and breast [9].
Tumors of the sphenoid sinus can present in a variety of ways, but as with
all disease in this location, nonspecific symptoms predominate. Symptoms
may vary depending on extent of tumor and site of origin within or adjacent
to the sinus. Headache and visual disturbance are the most common
presenting symptoms. Cranial nerve or vessel involvement signifies spread to
the surrounding tissues (eg, cavernous sinus); this involvement is charac-
teristic of later stages of the disease and portends a poorer outcome.
CT and MRI provide important information regarding size and extent of
tumor (Fig. 4). CT provides details of bone destruction, whereas MRI is
crucial for detecting soft tissue involvement, intracranial extension, and
perineural spread. MRI is also helpful in distinguishing between fluid
collections and space-occupying lesions.

Cerebrospinal leaks, encephaloceles, and other conditions

CSF leaks most commonly occur following head trauma but may also be
spontaneous. Clear rhinorrhea is the classic symptom of a CSF leak, but
definitive confirmation may require beta-2 transferrin testing. Nasal
endoscopy and CT scanning may also be helpful in visualizing the bony
defect, particularly in cases of trauma. CSF leaks can also be the
consequence of invasive processes that disrupt normal anatomic borders
and the sphenoid sinus architecture. These processes include, but are not
limited to, mucoceles, fungal infections, and neoplastic processes. Manage-
ment of leaks induced by trauma or disease mirrors that of CSF rhinorrhea
as a postoperative complication of functional endoscopic sinus surgery. The
approach and management are addressed later.
Encephaloceles are similar to gliomas in that they represent heterotopic
glial tissue, but their CSF contents communicate with the intracranial space.
Encephaloceles involve the meninges only and do not contain actual brain
tissue. Only 20% of the encephaloceles occur around the nasal root or the
skull base; most cases are located in the occipital and parietal regions. The
intranasal and skull base cases are found medially, and they are associated
446 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451

Fig. 4. Coronal CT image showing a multiple myeloma of the right sphenoid sinus that has
eroded into bone.

with the ethmoid and sphenoid sinuses. Encephaloceles are usually soft,
blue-tinted masses that transilluminate during nasal endoscopy and are
compressible when physically manipulated. They enlarge with crying or
Valsalvogenic maneuvers (positive Furstenburg sign). Feelings of a nasal
mass, recurrent clear CSF rhinorrhea, and meningitis are the well-known
signs of encephalocele.
 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451 447

Evaluation of sphenoid sinus disease

CT remains the standard for evaluating sphenoid sinus disease. It is the
single most accurate way of diagnosing sphenoid sinus disease and its
complications [10]. MRI is an essential adjunct in the diagnosis and selection
of treatment for many lesions of the sphenoid sinus. It provides excellent
detail of soft tissues and plays an important role in the diagnosis of
intracranial lesions.
Lawson and Reino [2] proposed an algorithm for using CT and MRI in
the diagnosis of isolated sphenoid sinus disease. This elegant scheme
classifies the various radiologic findings noted on CT scan to determine
better the correct diagnosis or the need for further imaging. The purpose of
this algorithm is to optimize the diagnostic value of the imaging techniques
while minimizing unnecessary studies (Fig. 5).
The first category is the type 1 lesion, which includes acute and chronic
sinusitis, mucoceles, polyps, and retention cysts. On the CT scan, acute
sinusitis appears as an air-fluid level within the sinus cavity. Chronic sinusitis,
on the other hand, manifests as opacification representing the hypertrophic
mucosa and secretions typical of the process. Mucous retention cysts and
polyps appear as a globular opacity. For type 1 lesions, only CT scanning is
indicated. Because most isolated sphenoid sinus disease falls within this
category, CT is frequently the only imaging study needed to evaluate sinus
pathologic conditions. The only exception is when polypoid masses are seen
in the lateral sphenoid, because these can represent encephaloceles or carotid
artery aneurysms. In such cases, further evaluation by MRI is warranted.
Type 2 lesions are lesions that cause anatomic distortion of a sinus wall
on CT. This distortion may either thinning, expansion, or remodeling
secondary to a mucocele or tumor. MRI is used to differentiate between
mucoceles and benign tumors. On MRI, mucoceles give a homogenous low-
intensity signal in the T1 phase and a high-intensity signal in the T2 phase.
Tumors tend to generate an intermediate signal in both T1 and T2 phases.
Type 3 lesions of the sphenoid sinus appear on CT scan as total sinus
opacification with sclerosis of surrounding bone. This finding is associated
with fungal sinusitis and fibro-osseous lesions. MRI is needed to differen-
tiate between fungal sinusitis and fibro-osseous disorders. On MRI, fungal
sinusitis manifests as a signal void in the sinus cavity. Fibro-osseous dis-
orders, on the other hand, have a mixed signal.
Bone erosion seen on CT scan suggests a neoplastic process within the
sphenoid sinus and constitutes the type 4 lesion. Rarely, a mucocele can
have a similar presentation on CT. MRI is always used for further
evaluation of the lesion to determine the nature of the neoplastic process
and degree of extension.
Finally, the type 5 lesion shows extrasinus extension and bone erosion
around the sphenoid sinus. In these cases, MRI is indicated to determine the
extent of intracranial and bony involvement. Direct extension from tumors
 448
G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451
Fig. 5. Radiologic evaluation of sphenoid sinus disease. From Lawson W, Reino AJ. Isolated sphenoid sinus disease: an analysis of 132 Cases. Laryngoscope
1997;107(12):1590–95; with permission.
 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451 449

originating in sites adjacent to the sphenoid, such as tumors of the pituitary

gland, clivus, meninges, and nasopharynx, can also be visualized with this
imaging technique.
Angiography is extremely helpful in delineating vascular malformations
and hemangiomas as well as anatomic variations of the internal carotid
artery.
Nasal endoscopy is an important clinical tool in the diagnosis of sphenoid
sinus disease. Findings on endoscopy often complement the radiologic
findings. Nasal endoscopy allows detailed visualization of the sphenoeth-
moid recess, allowing identification of lesions that extend anteriorly to the
rostrum of the sphenoid sinus. Detection of lesion characteristics, such
pulsatility, may help define the nature of the lesion. Nasal endoscopy may
also be helpful in detecting the site of CSF leak.
Additional diagnostic tools can be used as needed in the evaluation of
isolated sphenoid sinus disease. For example, beta-2 transferrin assays can
be employed to confirm the presence of CSF in cases of suspected CSF leak.

Management of sphenoid sinus disease

Inflammatory sphenoid sinus disease
Medical therapy is often the first line of treatment for cases of acute
sphenoid sinusitis. Antimicrobial therapy is indicated to hasten the relief of
symptoms and to avoid unnecessary complications. Antibiotic coverage
should include common upper respiratory pathogens, but coverage for
staphylococcal and gram-negative organisms is also desirable [10]. Appro-
priate topical and systemic decongestants should be used to promote
drainage. Patients who present with severe symptoms may require
intravenous antibiotics. If severe symptoms, such as headache, fail to
respond to intravenous antibiotics with 48 to 72 hours, surgical drainage is
indicated. If infection has spread to the orbit or the central nervous system,
treatment with antibiotics having good penetration through the blood–brain
barrier is indicated. Chronic sinusitis is often polymicrobial in origin and
thus more difficult to treat adequately with antibiotic therapy. When chronic
sinusitis fails to resolve with medical therapy, endoscopic surgical in-
tervention is indicated to restore natural drainage and aeration of the sinuses.
Endoscopic approaches to the sphenoid sinus include the transnasal and
transethmoidal approaches. The utility of the two techniques have been
described previously [11]. The transnasal approach provides direct access to
the natural sphenoid sinus ostium through the sphenoethmoid recess and
is the most direct approach for patients with isolated sphenoid sinus disease.
The transethmoidal approach is useful when there is coexistent ethmoid
disease. Variations in anatomy, such as the presence of a sphenoethmoid
(Onodi) cell, must be considered when using this approach to avoid damage
to surrounding neural and vascular structures. Various studies have shown
450 G.A. Grillone, P. Kasznica / Otolaryngol Clin N Am 37 (2004) 435–451

endoscopic approaches to the sphenoid sinus to be safe and effective [11,12].

Partial or total surgical excision of mucoceles can be accomplished using
endoscopic or transeptal approaches. Marsupialization has been shown to be
a useful technique in the management of mucoceles [1,13]. Fungal
mycetomas are treated by surgical excision. Invasive fungal disease requires
débridement and the use of systemic antifungal agents such as amphotericin.

Neoplasms and miscellaneous conditions

A detailed discussion of the management of sphenoid sinus neoplasms is
beyond the scope of this article. Treatment is individualized for each tumor
and depends on the specific histopathology, size, site of origin, and extent of
tumor. Treatment options may include surgery, radiation therapy or other
modalities, alone or in combination. Surgical access to the sphenoid sinus
for tumor removal includes endoscopic transnasal and transethmoidal
approaches, transeptal approaches, and, for large tumors, intracranial and
anterior skull base approaches. CSF leaks can usually be managed using
endoscopic techniques.

Summary
Disease of the sphenoid sinus is often vague and nonspecific in its clinical
presentation. Therefore, the otolaryngologist must maintain a high index of
suspicion when evaluating patients who present with such nonspecific
symptoms. A thorough understanding of the radiologic characteristics of
sphenoid sinus disease is essential in the proper evaluation and management
of these patients.

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