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238 V Miscellaneous
E. Treatment
1. Nonoperative
a. Breathing exercises
b. Isometric exercise of the muscles
c. Range of motion
d. Nonsteroidal antiinflammatory medications
2. Surgical management
a. Indications
(1) Flexion deformity associated with pain and neurological
compromise
(2) Loss of horizontal gaze (e.g., chin on chest deformity)
(3) Unstable spine fractures
b. Spinal deformity in ankylosing spondylitis
(1) Loss of lumbar lordosis and increased cervical and thoracic
kyphosis
(2) Primary location of disabling deformity should be assessed.
(3) Osteotomies
(a) Cervical
(i) Osteotomy at C7–T1 junction with laminectomy from
C6 to T2 is considered for primary cervical kyphosis.
(b) Thoracic
(i) In severe kyphotic thoracic deformity, multiple pos-
terior thoracic resection osteotomies can be done af-
ter anterior osteotomies.
(ii) Costotransverse osteotomies
(iii) Thoracic kyphosis can often be addressed via a lum-
bar osteotomy.
(c) Lumbar
(i) Osteotomies are performed typically between L2 and L4.
* Closing wedge osteotomy (e.g., pedicle subtraction
osteotomy)
(ii) High neurological complication rate (up to 9%)
* Closing wedge osteotomy appears to be safer than
opening wedge.
II. Reiter’s syndrome
A. Classic clinical triad
1. Urethritis
2. Conjuctivitis
3. Polyarthritis
B. Ninety percent of patients HLA-B27 positive
C. Microbes implicated in pathogenesis
1. Shigella, salmonella, yersinia, and campylobacter
D. Radiographic findings
1. Periostitis in the heel and toes
2. Sacroiliitis (unilateral)
3. Nonmarginal asymmetric syndesmophytes (large and bulky)
Chapter 22 Seronegative Spondyloarthropathies 239
III. Psoriatic arthritis
A. Seven percent of patients with psoriasis
B. Arthritis precedes skin lesions in 15% of cases.
a. Skin lesions include balanitis circinata, ulcers in the oral mucosa,
and keratoderma blennorrhagica.
C. Sacroiliitis is unilateral and asymmetric.
D. Laboratory findings
1. HLA-B27 positive in 20%
E. Treatment
1. Physical therapy
2. Medications
a. Aspirin, indomethacin, or naproxen
F. Shares many common musculoskeletal findings with Reiter syndrome
IV. Enteropathic arthritis (inflammatory bowel disease)
A. Shares many musculoskeletal features with Ankylosing Spondylitis (AS).
B. HLA B-27 positive in 5%
C. More common in Crohn’s disease than in ulcerative colitis
D. Spinal involvement independent of bowel disease
1. Peripheral arthritis following disease exacerbations
E. Marginal (starting at the end plates) symmetric syndesmophytes
F. Sacroiliitis is bilateral and symmetric.
Suggested Reading
Danisa OA, Turner D, Richardson WJ. Surgical correction of lumbar kyphotic deformity: posterior reduc-
tion “eggshell” osteotomy. J Neurosurg 2000;92:50–56
Halm H, Metz-Stavenhagen P, Zielke K. Results of surgical correction of kyphotic deformities of the spine
in ankylosing spondylitis on the basis of the modified arthritis impact measurement scales. Spine
1995;20:1612–1619
Simmons E. Kyphotic deformity of the spine in ankylosing spondylitis. Clin Orthop Relat Res
1977;128:65–68
Simmons E. The surgical correction of flexion deformity of the cervical spine of ankylosing spondylitis.
Clin Orthop Relat Res 1972;86:132–139
Simmons EH, Graziano GP, Heffner R. Muscle disease as a cause of kyphotic deformity in ankylosing
spondylitis. Spine 1991;16:S351–S360
Weatherley C, Jaffray D, Terry A. Vascular complications associated with osteotomy in ankylosing
spondylitis: a report of two cases. Spine 1988;13:43–46
I. Developmental anatomy
A. Atlas
1. Neurocentral synchondrosis forms at 6 to 24 months.
a. Fusion begins at 4 to 6 years.
b. Posterior synchondrosis fuses at 5 years.
B. Dens
1. Two primary ossification centers coalesce at 1 to 3 months of age.
2. Separated from the vertebral body by a dentocentral synchondrosis
that fuses at 6 to 8 years
C. Normal variants
1. Posterior bifid C1 arch
2. Bipartite superior articular surface of the atlas
3. Pseudonotch of the atlas
4. Absence or partial absence of the posterior arch of the atlas
5. Posteriorly displaced spinolaminar line of the axis
6. Posteriorly angulated dens
7. Pseudosubluxation of the axis (less than 10 years old)
II. Physical examination
A Limited range of motion
B. Torticollis
C. Facial asymmetry
D. Associated abnormalities
1. Scoliosis, renal, cardiac, or other head and neck anomalies
III. Radiographic evaluation (Figs. 23–1, 23–2)
A. Flexion-extension view is very important to assess stability.
B. Critical measurements on flexed lateral view
1. Atlantoaxial interval: 4.5 mm (children), compared with 3 mm (adults)
2. Space available for spinal cord (SAC): 13 mm
IV. Specific disorders
A. Basilar invagination (Fig. 23–3)
1. Deformity of the bones at the base of the skull at the margin of the
foramen magnum
a. The odontoid is migrated cephalad.
2. Types
a. Primary
(1) Congenital
(2) Associated with other findings
(a) Atlanto-occipital fusion
(b) Hypoplasia of atlas
(c) Bifid posterior arch of the atlas
(d) Odontoid abnormalities
(e) Klippel-Feil syndrome
b. Secondary
(1) Developmental condition with softening of the base of the skull
(2) Associated with other clinical disorders
(a) Osteomalacia
(b) Rickets
Figure 23–1 Lateral illustration of the normal relationship of the C1–C2 articulation demonstrat-
ing the atlantodens interval (ADI) and the space available for cord (SAC).
Figure 23–2 (A) In flexion, SAC may decrease between the posterior aspect of the dens and the
anterior aspect of the C1 posterior ring. (B) In extension, SAC may decrease between the posterior
aspect of the dens and the anterior aspect of the C2 lamina or the foramen magnum.
(c) Paget’s disease
(d) Osteogenesis imperfecta
(e) Renal osteodystrophy
(f) Rheumatoid arthritis
(g) Neurofibromatosis
(h) Ankylosing spondylitis
(i) Achondroplasia
3. Clinical findings
a. Commonly become symptomatic in second and third decades
(1) Short neck
(2) Asymmetric face
Figure 23–3 An 18-year-old man with osteogenesis imperfecta and basilar invagination present-
ing with unrelenting headache, neck pain, and myelopathy. (A) Lateral radiograph demonstrating
basilar invagination with the odontoid protruding into the foramen magnum. (B)MRI scan show-
ing the odontoid indenting the brain stem. (C) Postoperative lateral radiograph showing triple wire
technique and posterior occipital-cervical fusion.
(3) Torticollis
(4) Weakness/paresthesias
(5) Cranial nerve palsies
(6) Cerebellar signs (unsteady gait and nystagmus)
(7) Pain (head and neck)
(8) Syncope and dizziness (vertebral artery compression)
(9) Seizures/hydrocephalus (cerebrospinal obstruction)
4. Treatment
a. Posterior impingement
(1) Suboccipital craniectomy and decompression of the posterior
ring of C1 with posterior stabilization
b. Anterior impingement
(1) Mobile odontoid
(a) Occipitocervical fusion in extension is recommended
(2) If the odontoid cannot be reduced, anterior excision of odontoid
and posterior stabilization is recommended.
B. Klippel-Feil syndrome (Fig. 23–4)
1. Congenital fusion of cervical vertebrae
2. Failure of normal segmentation of cervical spine during the third to
eighth week
3. Associated anomalies
a. Genitourinary (35%)
b. Central nervous system
c. Cardiopulmonary
d. Sprengel’s deformities (40%)
e. Upper extremity anomalies
f. Scoliosis (60%)
4. Clinical findings
a. Low posterior neck line
b. Short neck
c. Limited neck motion
(1) Majority have normal appearance with mild restriction of motion.
5. Radiographic findings
a. Vertebral synostoses
b. Flattening and widening of the vertebral bodies
c. Absent disc spaces or hypoplasia
6. Treatment
a. The majority of patients are asymptomatic.
(1) Symptoms may appear later in life.
b. Conservative treatment is indicated for most patients.
(1) Anti-inflammatory medications
(2) Exercise program
c. Fusion may be indicated in select patients with instability and
spinal cord impingement.
C. Anomalies of the odontoid
1. Etiology
a. Trauma
(1) Salter I fracture with a nonunion
b. Congenital
(1) Failure to fuse (normally fuses at 3 to 6 years)
2. Clinical findings
a. Neck pain
b. Torticollis
c. Neurological symptoms
Figure 23–4 A 55-year-old man with Klippel-Feil syndrome and basilar invagination. The pa-
tient presented with severe neck pain and headache. (A) Lateral radiograph showing congenital
fusion between C3–C4 and C5–C6. (B) MRI also shows the congenitally fused vertebrae with
disk degeneration at C4–C5. Basilar invagination is noted as well. (C) Postoperative radiograph
showing an occiput-C6 fusion with plate-screw fixation.
3. Treatment
a. Conservative if stable
b. Surgical intervention
(1) Indications for surgical stabilization
(a) More than 7 to 10 mm of instability even without symptoms
(b) Atlantodens interval with flexion-extension and space
available for spinal cord is less than 13 mm
(2) Technique
(a) C1–C2 fusion with wire fixation
(i) Vertebral artery is proportionally closer to the midline
in the child
(b) Preoperative traction and reduction may be necessary.
(c) Postoperative halo vest stabilization
(d) C1–C2 fusion with C1–C2 transarticular screw fixation
(i) More rigid and avoids halo vest postoperatively
(e) Occiput–C2 fusion is necessary if C1 ring is deficient.
D. Congenital atlanto-occipital fusion
1. Failure of segmentation
2. Most commonly recognized anomaly of the craniovertebral junction
3. Prone to C1–C2 instability if associated with C2–C3 fusion or anomalies
of the odontoid (70%)
4. Associated with dwarfism, funnel chest, pes cavus, syndactyly, cleft
palate, and genitourinary anomalies
5. Clinical findings
a. Short neck, restricted neck motion, and torticollis
b. Fifty percent have relative basilar impressions secondary to dimin-
ished vertebral height of the atlas ring.
c. Neurological involvement especially if the odontoid is above the
foramen magnum level
6. Radiographic findings
a. Flexion-extension view
(1) Atlantodens interval (ADI) 3 to 4 mm
(2) SAC less than 13 mm
7. Treatment
a. Conservative treatment
(1) Cervical collar
(2) Traction
b. Surgery
(1) Occiput–C1–C2 fusion after traction and reduction if C1–C2
instability is present
(2) Posterior decompression and fusion if there is posterior com-
pression of the spinal cord
E. Torticollis (wry neck)
1. Typically discovered in the first 6 to 8 weeks of life
2. Ischemia and contracture of the sternocleidomastoid muscle may be
pathogenic.
a. Venous occlusion and fibrous replacement of tissue secondary to
intrauterine position
3. Clinical findings
a. Twenty percent associated incidence of congenital hip dislocation
b. Eighty-five percent right side involvement
c. Head is tilted toward the involved side and the chin is rotated to the
opposite side.
d. Soft, nontender enlargement beneath the skin resolves in 6 to 12 weeks.
e. Contracture of the muscle follows with decreased range of motion
of the neck.
f. Facial asymmetry and mild dorsal compensatory scoliosis
4. Differential diagnosis
a. Congenital cervical spine anomalies
b. Extraocular muscle imbalance
5. Treatment
a. Stretching exercise, positioning, and brace
(1) Eighty-five to 90% response within 1 year
b. Surgical indications
(1) After 1 year if persistent facial asymmetry
(2) Head tilting
(3) Decreased range of motion
c. Surgical options
(1) Unipolar/bipolar release
(a) Bipolar release with Z-lengthening gives the best results
(i) Careful of the posterior auricular nerve and spinal
accessory nerve
F. Atlantoaxial instabilities
1. Etiologies
a. Inflammation
(1) Pharyngeal infection (Grisel’s syndrome)
(2) Juvenile rheumatoid arthritis
b. Down’s syndrome
(1) Twenty-five percent incidence
(2) Boys older than 10 years are more at risk for myelopathy due to
rupture of transverse ligament.
c. Dysplasia
(1) Achondroplasia
(2) Diastrophic dysplasia
(3) Spondyloepiphyseal dysplasia
(4) Morquio’s syndrome
(5) Larsen’s syndrome
d. Congenital anomalies
e. Spontaneous rotatory subluxation of C1–C2
(1) Fielding classification
(a) Type I
(i) Simple shift without displacement
(b) Type II
(i) Less than 5 mm of C1–C2 displacement
(c) Type III
(i) Greater than 5 mm of C1–C2 displacement