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Paul D. Sawin and Arnold H. Menezes
✓ Osteogenesis imperfecta (OI) is a heritable disorder of bone development caused by defective collagen synthesis. Basilar invagination is an uncommon but devastating complication of this disease. The authors present a comprehensive strategy for management of craniovertebral anomalies associated with OI and related osteochondrodysplasias.
Twenty-five patients with congenital osteochondrodysplasias (18 OI, four Hajdu—Cheney syndrome, and three spondyloepiphyseal dysplasia) and basilar invagination were evaluated between 1985 and 1995. The male/female ratio in this cohort was 1:1. The mean age at presentation was 11.9 years (range 13 months–20 years). Fourteen patients (56%) presented during adolescence (11–15 years of age). Symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis (20%). Four patients (16%) were asymptomatic. Seven (28%) had undergone previous posterior fossa decompression; one had also undergone ventral decompression. Imaging findings included basilar invagination (100%), ventral brainstem compression (84%), hydrocephalus (32%), hindbrain herniation (28%), and syringomyelia/syringobulbia (16%).
Patients with hydrocephalus underwent ventricular shunt placement. Reducible basilar invagination (40%) was treated with posterior fossa decompression and occipitocervical fusion. Those with irreducible ventral compression (60%) underwent transoral—transpalatopharyngeal decompression followed by occipitocervical fusion. All patients improved initially. However, basilar invagination progressed radiographically in 80% (symptomatic in 24%) despite successful fusion. Prolonged external orthotic immobilization with the modified Minerva brace afforded symptomatic improvement and arrested progression of the deformity. The mean follow-up period was 5.9 years (range 1.1–10.5 years).
Ventral brainstem compression in OI should be treated with ventral decompression, followed by occipitocervical fusion with contoured loop instrumentation to prevent further squamooccipital infolding. Despite fusion, however, basilar invagination tends to progress. Prolonged immobilization (particularly during adolescence) may stabilize symptoms and halt further invagination. This study represents the largest series to date addressing craniovertebral anomalies in OI and related congenital bone softening disorders.
Paul M. Arnold, Robert D. Strang, and Danielle Roussel
The purpose of this study was to evaluate the usefulness and efficacy of variable-angle screws in transpedicular fixation.
Sixteen patients (10 men and six women, age range 44–73 years) underwent a posterior transpedicular fixation procedure in which variable-angle screws were placed at two or more levels. Four patients experienced spinal instability secondary to trauma, 10 patients suffered from degenerative spondylosis requiring fusion, and two patients were treated for spinal metastatic disease. All patients underwent posterior transverse process fusion in which autologous bone was used. In total, 37 vertebral levels were fixed with variable-angle screws placed at 22 levels. Postoperatively all patients were fitted with an external orthosis that was worn for 12 weeks. Serial postoperative plain x-ray films were obtained in all cases. Follow-up periods ranged from 11 to 24 months.
Based on clinical and radiographic data, solid fusion was achieved in 14 of 16 patients. There were two early disconnections of the rod/screw connectors, which were related to errors in technique. With the exception of these disconnections, there were no cases of construct failure. There were no wound infections, and no patient experienced a decline in neurological function as compared with preoperative levels. Both instrumentation systems were easy to use, and the flexibility in trajectory allowed for quicker screw placement and decreased operating room time.
Variable-angle pedicle screws are a safe, useful adjunct to transpedicular fixation, particularly in multisegment fixation and in spines with abnormal alignment.
Kavelin Rumalla, Kyle A. Smith, and Paul M. Arnold
Immunoglobulin G4–related disease (IgG4-RD) is a recently defined condition characterized by inflammatory tumefactive lesions in various organ systems. IgG4-RD is a clinical and radiological diagnosis of exclusion and requires the presence of specific histopathological criteria for diagnosis.
A 50-year-old man presented to an outside hospital with a 3-month history of progressively worsening back pain and symptoms of pleurisy, nasal crusting, and hematochezia. Radiological workup revealed an epidural-paraspinal mass with displacement of the spinal cord, destruction of the T5–6 vertebrae, and extension into the right lung. Biopsy sampling and subsequent histopathological analysis revealed dense lymphoplasmacytic infiltrate with an increased number of IgG4-positive plasma cells and a storiform pattern of fibrosis. With strong histopathological evidence of IgG4-RD, the patient was started on a regimen of prednisone. Further testing ruled out malignant neoplasm, infectious etiologies, and other autoimmune diseases. Two weeks later, the patient presented with acute-onset paraplegia due to spinal cord compression. The patient underwent decompression laminectomy of T5–6, posterior instrumented fusion of T2–8, and debulking of the epidural-paraspinal mass. After the continued administration of glucocorticosteroids, the patient improved remarkably to near-normal strength in the lower extremities and sensory function 6 months after surgery.
To the authors' knowledge, this is the first case of IgG4-related epidural inflammatory pseudotumor and spinal cord compression in the United States. This case highlights the importance of early administration of glucocorticosteroids, which were essential to preventing further progression and preventing relapse. IgG4-RD evaluation is important after other diseases in the differential diagnosis are ruled out.
