SCOLIOSIS IN FAMILIAL DYSAUTONOMIA - OPERATIVE TREATMENT

The results of operative treatment of scoliosis were reviewed for twen ty-two patients (ten boys and twelve girls) who had familial dysautono mia, an autosomal recessive disorder affecting primarily Ashkenazi Jew s. The indication for operative intervention was progressive kyphoscol iosis to 45 degrees or more in a skeletally immature patient for whom bracing had failed. The mean age at the time of the operation was fift een years and five months (range, eight years and two months to ninete en years). Seventeen patients had a thoracic curve with a mean preoper ative Cobb angle of 69 degrees (range, 47 to 112 degrees), and five pa tients had a double major curve with a mean preoperative Cobb angle of 71 degrees (range, 42 to 87 degrees) for the cephalad curves and 60 d egrees (range, 45 to 72 degrees) for the caudad curves. Twenty patient s had a rigid kyphosis; in fourteen, the apex was at the seventh thora cic vertebra or more cephalad. Two patients had a lordoscoliosis, The mean preoperative kyphosis was 64 degrees (range, 12 to 110 degrees) i n the thirteen patients who had a thoracic curve and for whom informat ion regarding kyphosis was available, and it was 70 degrees (range, 54 to 84 degrees) in the five patients who had a double major curve. Pos terior spinal arthrodesis and instrumentation was performed in all pat ients. Two patients had an anterior arthrodesis as well because of the severity and rigidity of the curve. Allograft bone was used in eighte en patients, Postoperatively, all patients were managed with a body ca st or with a custom-molded thoracolumbar brace. The differences betwee n the preoperative and the most recent measurements of the scoliosis w ere significant (p < 0.05), and the differences between the preoperati ve and the most recent measurements of the kyphosis were not (p > 0.05 ). Fifteen patients had major postoperative complications, and there w ere twenty-nine reoperations due to complications. Nine patients died, between nine months and thirteen years after the operation, secondary to other causes. These nine patients were among the first eleven who were operated on before 1983; their deaths reflect difficulties in the medical management.