Magdy Abdel Motaal, M.D.,
S. Jay Kumar, M.D., James T. Guille, M.D.
Alfred I. duPont Institute
1600 Rockland Road
Wilmington, DE 19803
ABSTRACT: Fourteen patients with arthrogryposis multiplex congenita underwent a spinal fusion for progressive scoliosis. Scoliosis was first noticed at birth in four patients. Ten patients were non-ambulatory and used a wheelchair and four were ambulatory. Three of the four ambulatory patients used different aids for ambulation. The curve pattern was a long C-type of curve in two patients, a thoracic curve in four patients, thoraco-lumbar curve in four patients and lumbar in four patients. Pelvic obliquity exceeding 30 degrees was seen in four patients. Pelvic obliquity was not secondary to scoliosis, but due to contractures around the hips. Congenital anomalies of the spine were seen in seven of the fourteen patients. Eleven of the fourteen patients had only a posterior spinal fusion. In seven patients the posterior spinal muscles were atrophic. The intraspinous ligaments were markedly thickened. The ligamentum and the facet joints ossified. This ossified ligamentum flavum had to be excised to achieve correction. Follow-up ranged from two to seventeen years. We concluded that bracing is not effective in the treatment of these patients. Once the curve starts to increase, progression is rapid. Rigidity of the curve is mainly due to the thickened intra-spinous ligament and ossification of the ligamentum flavum and the facet joints. Once curve progression is seen the spine needs to be corrected and fused before it becomes very stiff. Fusion to the sacrum should be avoided if possible because the hips are usually stiff. If ambulatory potential is to be preserved, the lumbar lordosis should be maintained. This paper addresses the problems and results following fusion of scoliotic curves in children with arthrogryposis multiplex congenita.
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