Occult spinal canal stenosis due to C-1 hypoplasia in children with Down syndrome.

OBJECT: Little has been published about subclinical spinal canal stenosis due to C-1 hypoplasia in patients with Down syndrome. In this paper the authors performed a matched comparison study with cross-sectional survey to investigate occult spinal canal stenosis due to C-1 hypoplasia in children with Down syndrome. METHODS: A total of 102 children with Down syndrome ranging in age from 10 to 15 years were matched according to age and physique with 176 normal children. In all participants, the anteroposterior (AP) diameter of C-1 and the atlas-dens interval (ADI) were measured on plain lateral x-ray images of the cervical spine. The cross-sectional area of the atlas was also measured from a cross-sectional computed tomography image of C-1. RESULTS: Eight children (6.7%) with Down syndrome developed atlantoaxial subluxation associated with myelopathy. The difference in the ADI between the patients and controls was not statistically significant. The average AP diameter of the atlas and the spinal canal area along the cross-section of the atlas were significantly smaller in children with Down syndrome than those in the control group. CONCLUSIONS: Atlantoaxial instability and occult spinal canal stenosis due to C-1 hypoplasia in patients with Down syndrome may significantly increase the risk of myelopathy.

Long-term result of combination of open reduction and femoral derotation varus osteotomy with shortening for developmental dislocation of the hip.

This study examined femoral derotation varus osteotomy with shortening performed on children with developmental dislocation of the hip. Each patient reached 14 years of age. Surgical outcomes were evaluated clinically and roentgenographically. We studied nine patients with 11 joints undergoing surgical repair. Age at the time of operation averaged 2 years and 1 month; the period of observation averaged 15 years and 7 months; and age at final investigation averaged 17 years and 8 months. Each patient underwent the study operation as an initial treatment. Salter pelvic osteotomy was reserved as a second treatment for those patients whose acetabular development proved inadequate during post-surgical observation. The evaluation method established by McKay was utilized to determine clinical results at the time of final investigation; 73% of the study group were established as having good results. Severin's evaluation method was used to assess roentgenographical results; 45% of the study group were determined to have good results. Kalamchi's evaluation method allowed six joints to be diagnosed with avascular necrosis of the femoral head; however, in all cases but one, necrosis had been present prior to surgery. Those six joints that did not have necrosis of the femoral head prior to surgery developed only one mild necrosis subsequent to surgery. Achieving a stable reduced position by femoral derotation varus osteotomy, as well as sufficient decompression of the femoral head by shortening osteotomy, are considered to have played very important roles in preventing the femoral head from developing necrosis.

Treatment of craniocervical spine lesion with osteogenesis imperfecta: a case report.

STUDY DESIGN: A case report of craniocervical spine lesions including basilar impression, atlantoaxial dislocation, and syringomyelia, with osteogenesis imperfecta is presented, and the literature is reviewed. OBJECTIVE: To discuss the problems involved in the surgical management of craniocervical spine lesion with osteogenesis imperfecta. SUMMARY OF BACKGROUND DATA: Osteogenesis imperfecta is known to have various spine lesions as complications. However, few reports have described craniocervical lesions associated with osteogenesis imperfecta. METHODS: A 14-year-old girl with osteogenesis imperfecta, Silence classification IVB, experienced difficulty walking, with marked motor disturbance and muscle weakness in the extremities. Deep tendon reflexes were exaggerated bilaterally in the upper and lower extremities, and positive Babinski reflex and ankle clonus were observed bilaterally. Basilar impression, atlantoaxial dislocation, and syringomyelia were shown by plain radiography, tomography, three-dimensional computed tomography, and magnetic resonance imaging. RESULTS: In the reported patient, posterior fossa decompression and atlantoaxial posterior fusion could not be performed because the foramen magnum and upper cervical spine invaginated to the base of the skull. Therefore, occipitocervical spine fusion using titanium loop and wires was performed at the reduced position of the atlantoaxial dislocation, resulting in improvement of neurologic deficits. CONCLUSIONS: For patients with atlantoaxial dislocation, syringomyelia, and basilar impression without clinical symptoms or signs of brain stem compression, occipitocervical spine fusion alone at the reduction of the atlantoaxial dislocation may be indicated because these procedures improve neurologic deficits and prevent postoperative development of basilar impression and enlargement of syringomyelia.