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Special Conditions Affecting the Spine 1179

71.2.2 Ossification of the posterior longitudinal ligament (OPLL)

General information

Key concepts
● fibrosis followed by calcification and then ossification of the posterior longitudinal ligament. The
process may involve the dura
● more common in Asian population
● most patients have only mild subjective complaints
● 50% of patients have impaired glucose tolerance, respiratory compromise may result from ossifica-
tion of the costotransverse and costovertebral ligaments
● surgery is best for moderate neuro involvement (Nurick grade 3 & 4)

The age of patients with OPLL ranges from 32–81 years (mean = 53), with a slight male predomi-
nance. The prevalence increases with age. Duration of symptoms averages ≈ 13 months. It is more
prevalent in the Japanese population (2–3.5%).38,39

Pathophysiology
The pathologic basis of OPLL is unknown, but there is an increased incidence of ankylosing hyper-
ostosis which suggests a hereditary basis.
OPLL begins with hypervascular fibrosis in the PLL which is followed by focal areas of calcification,
proliferation of periosteal cartilaginous cells and finally ossification.40 The process frequently
extends into the dura. Eventually active bone marrow production may occur. The process progresses
at varying rates among patients, with an average annual growth rate of 0.67 mm in the AP direction
and 4.1 mm longitudinally.41
When hypertrophied or ossified, the posterior longitudinal ligament may cause myelopathy (due
to direct spinal cord compression or ischemia) and/or radiculopathy (by nerve root compression or 71
stretching).
Changes within the spinal cord involve the posterolateral gray matter more than white matter,
suggesting an ischemic basis for the neurologic involvement.

Distribution
Average involvement: 2.7–4 levels. Frequency of involvement:
1. cervical: 70–75% of cases of OPLL. Typically begins at C3–4 and proceeds distally, often involving
C4–5 and C5–6 but usually sparing C6–7
2. thoracic: 15–20% (usually upper, ≈ T4–6)
3. lumbar: 10–15% (also usually upper, ≈ L1–3)

Pathologic classification
See reference.42
1. segmental: confined to space behind vertebral bodies, does not cross disc spaces
2. continuous: extends from VB to VB, spanning disc space(s)
3. mixed: combines elements of both of the above with skip areas
4. other variants: includes a rare type of OPLL that is contiguous with the endplates and is confined
to the disc space (involves focal hypertrophy of the PLL with punctate calcification)

Clinical
Most patients are asymptomatic, or have only mild subjective complaints. This is probably explained
by the protective effect of the fusion resulting from OPLL and the very gradual compression.
Natural history: 17% of patients without myelopathy developed myelopathy in one study43 over
1.6 years mean follow-up. Statistically, the myelopathy-free rate in patients not initially presenting
with myelopathy was 71% after 30 years.43
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1180 Non-Traumatic Spine and Spinal Cord Conditions

Evaluation
Plain X-rays
Often fail to demonstrate OPLL. Flexion/extension views may be helpful in assessing stability.

MRI
OPLL appears as a hypointense area and is difficult to appreciate until it reaches ≈ 5 mm thickness.
On T1WI it blends in with the hypointensity of the ventral subarachnoid space; on T2WI it remains
hypointense while the CSF becomes bright. Sagittal images may be very helpful in providing an over-
view of the extent of involvement, and T2WI may demonstrate intrinsic spinal cord abnormalities,
which may be associated with a worse outcome.

Myelography/CT
Myelography with post-myelographic CT (especially with 3D reconstructions) is probably best at
demonstrating and accurately diagnosing OPLL.

Treatment
Treatment decisions
Based on clinical grade42 as follows:
1. Class I: radiographic evidence without clinical signs or symptoms. Most patients with OPLL are
asymptomatic.39 Conservative management unless severe
2. Class II: patients with myelopathy or radiculopathy. Minimal or stable deficit may be followed
expectantly. Significant deficit or evidence of progression warrants surgical intervention
3. Class IIIA: moderate to severe myelopathy. Usually requires surgical intervention
4. Class IIIB: severe to complete quadriplegia. Surgery is considered for incomplete quadriplegics
showing progressive slow worsening. Rapid deterioration or complete quadriplegia, advanced
age, or poor medical condition are all associated with worse outcome

71
In moderate grade patients (Nurick grades 3 & 4)44 (see ▶ Table 71.2), surgery provided a statistically
significant reduction in deterioration. There was no difference between surgery and conservative
treatment in mild grade (Nurick 1 or 2), and surgery was ineffective in severe grade (Nurick 5).43

Pre-op assessment
Appropriate cardiorespiratory assessment should be made knowing that:
1. respiratory compromise may result from ossification of the costotransverse and costovertebral
ligaments
2. 50% of patients have impaired glucose tolerance with the attendant risks associated with
diabetes

Technical considerations for surgery

Severe OPLL increases the risk of spinal cord injury during neck positioning for intubation, and
strong consideration should be given to awake nasotracheal intubation.
An anterior approach is generally favored, although laminectomy may be acceptable. SSEP moni-
toring has been recommended by some.40 Distraction should be avoided until the spinal cord has
been decompressed from the OPLL.

Table 71.2 Nurick grade of disability from cervical spondylosis44

Grade Description
0 signs or symptoms of root involvement without myelopathy
1 myelopathy, but no difficulty in walking
2 slight difficulty in walking, able to work
3 difficulty in walking but not needing assistance, unable to work full-time
4 able to walk only with assistance or walker
5 chairbound or bedridden
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Special Conditions Affecting the Spine 1181

Some authors advocate complete removal of bone from the dura, while others feel it is permissi-
ble to leave a thin rim of bone adherent to the dura. Care must be taken in removing bone because it
tends to blend imperceptibly with dura and the next thing one may see is bare spinal cord.
Depending on the distance of vertical involvement, vertebral corpectomy with strut grafting may
be required. Internal plate fixation is often used as an adjunct. Postoperative immobilization for at
least 3 months is employed with rigid collars for single level ACDF or 1–2 level corpectomies, or
halo-vest traction for corpectomies > 2 levels.

Results with surgery

The incidence of pseudarthrosis after vertebral corpectomy and strut graft ranges from 5–10% and
increases with the number of levels fused.
In one series there was a 10% incidence of transient worsening of neurologic function following
anterior surgery41 which may have been related to distraction.
The risk of dural tear with CSF leak following an anterior approach depends on the aggressiveness
with which bone is removed from the dura, and ranges ≈ 16–25%.
Other risks of anterior approaches, e.g. esophageal injury (p. 1120), also pertain.

71.2.3 Ossification of the anterior longitudinal ligament (OALL)

OALL of the cervical spine and/or hypertrophic anterior cervical osteophytes may produce dramatic
radiographic findings and minimal clinical symptoms. Distinct from Forestier’s disease (see below).
Cervical involvement may produce dysphagia.45

71.2.4 Diffuse idiopathic skeletal hyperostosis (DISH)

Key concepts
● usually asymptomatic, but may present with globus 71
● W/U: ✔ speech therapy consult for dysphagia evaluation (usually includes ✔ modified barium swal-
low), ✔ CT of cervical spine, ± ✔ digital video esophagoscopy

AKA “DISH,” AKA spondylitis ossificans ligamentosa, AKA ankylosing hyperostosis, among others. A
condition characterized by flowing osteophytic formation of the spine in the absence of degenera-
tive, traumatic, or post-infectious changes. Affects Caucasians and males more commonly, and usu-
ally seen in patients in their mid-60 s.
97% of cases occur in the thoracic spine, also in the lumbar spine in 90%, cervical spine in 78%, and
all three segments in 70%. Sacroiliac joints are spared, unlike ankylosing spondylitis (AS) (p. 1174).
As with AS, unfused levels may be very unstable.
Risk factors for DISH include: elevated body mass index,46 elevated serum uric acid,46 diabetes
mellitus,46 elevated growth hormone or insulin levels.47
Usually does not produce clinical symptoms. Patients may have early morning stiffness and mild
limitations of activities. Cervical involvement may present with dysphagia (p. 1181) or globus phar-
yngis (the subjective sensation of a lump in the throat, not to be confused with globus hystericus,
which refers to the sensation of a lump in the throat where there is no identifiable pathology) due to
compression of the esophagus between the osteophytes and the rigid laryngeal structures48 (part of
Forestier’s disease49).
Plain X-rays and CT scan demonstrate the bony pathology.

▶ Dysphagia due to esophageal compression by osteophytes (▶ Fig. 71.3). Evaluation should also
include speech therapy consult for dysphagia, (modified) barium swallow study to help localize the
site of obstruction, and DVE (digital video esophagoscopy) to rule out intrinsic esophageal disease.
Surgery may be considered in cases that do not respond satisfactorily to dietary modifications in
patients who are losing weight or are having recurrent episodes of choking or (aspiration) pneumo-
nia. An anterior cervical approach and utilization of a high-speed drill with careful protection of
soft-tissue structures (esophagus, carotid sheath) without need for discectomy nor spine stabiliza-
tion has been recommended.48 Patients need to be made aware that post-op they may be worse
(from manipulation of esophagus and possibly disruption of some of the autonomic innervation of
the esophagus) and that there is a significant chance they will need a (temporary) gastrostomy feed-
ing tube. Improvement sometimes takes up to 1 year to occur.