Scoliosis in Neurofibromatosis / 대한정형외과학회잡지

ABSTRACT

Neurofibromatosis is a disease which involves both neuroectodermal and mesodermal tissue, and is characterized by cafe-au-lait spot, multiple subcutaneous neurofibromas, elephantiasis neuromatosa, a positive family history, and specific dystrophic osseous changes such as scoliosis, penciling of ribs, vertebral scalloping, a paravertebral soft tissue tumor, and congenital pseudarthrosis. The classic type of scoliosis in neurofibromatosis was known as a sharp localized short curve that is often rapidly progressive and produces severe deformity with dystrophic changes, but another form with long gentle curve is reported. Since conservative treatment is usually unsuccessful, posterior fusion with or without Harrington instrumentation is the treatment of choice even in young age when the curve is progressive. This paper was aimed to review our experience with 11 patients having neurofibromatosis and scoliosis, who were treated with posterior fusion and Harrington instrumentation from Jan. 1971 to Dec. 1980, and the results were as follows 1. The average age that spinal deformity was observed was 7.7 years old, but the average age at treatment was 14.4. 2. Cafe-au-lait spot was observed in all cases, subcutaneous nodule in 7 cases, local gigantism in 2 cases, and positive family history in 3 cases. 2 cases were combined with congenital spinal anormalies. 3. Specific pattern in spinal deformity was not significant. There were 7 short curves less than 5 vertebrae involved and 6 long curves more than 6 vertebrae involved. 4. There were 5 kyphosis which had more than 50°, and those were usually combined with severe scoliosis. 5. Preoperative average degree of scoliosis was 93.8, and the final correction was 41.2° (43.9%) with loss of correction 5.3°(5.7%) after 3.2 year follow-up in average. 6. It shouid be educated for early detection at home and school, and for the importance of early treatment for the scoliosis, to prevent rapid increase of scoliosis in neurofibromatosis.