Long-term follow-up of spinal fusion in spinal muscular atrophy.

From 1966 to 1983, 11 females and eight males with spinal muscular atrophy were treated by arthrodesis. Seventeen of 19 patients were evaluated by follow-up physical examination, roentgenograms, and questionnaires. In order to compare long-term versus short-term results, changes in management, and factors contributing to morbidity and mortality, three groups were identified for study. Group I consisted of 11 patients operated between 1966 and 1983 and alive at follow-up evaluation. Group II consisted of five patients operated between 1980 and 1983. Group III consisted of three patients, all deceased at follow-up evaluation and operated before 1980. In Group I, the preoperative curve averaged 119 degrees and at final follow-up examination, 99 degrees. The average preoperative curve in Group II was 80 degrees and at follow-up examination, 49 degrees. Long-term follow-up vital capacities were obtained in nine patients. The average preoperative vital capacity was 1033 cm3, and the average vital capacity seven years postoperation was 860 cm3. Most patients reported improvement in sitting, balance, and comfort. All patients are wheelchair-bound, as they were before surgery. Few long-term respiratory problems were reported.