The Journal of Bone and Joint Surgery, Vol 77, Issue 9 1362-1369, Copyright © 1995 by Journal of Bone and Joint Surgery, Inc
Scoliosis in familial dysautonomia. Operative treatment
PT Rubery, JH Spielman, P Hester, E Axelrod, SW Burke and DB Levine
Hospital for Special Surgery, New York Hospital-Cornell University Medical College, New York City, USA.
The results of operative treatment of scoliosis were reviewed for
twenty-two patients (ten boys and twelve girls) who had familial
dysautonomia, an autosomal recessive disorder affecting primarily Ashkenazi
Jews. The indication for operative intervention was progressive
kyphoscoliosis 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
fifteen years and five months (range, eight years and two months to
nineteen years). Seventeen patients had a thoracic curve with a mean
preoperative Cobb angle of 69 degrees (range, 47 to 112 degrees), and five
patients 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 degrees
(range, 45 to 72 degrees) for the caudad curves. Twenty patients had a
rigid kyphosis; in fourteen, the apex was at the seventh thoracic vertebra
or more cephalad. Two patients had a lordoscoliosis. The mean preoperative
kyphosis was 64 degrees (range, 12 to 110 degrees) in the thirteen patients
who had a thoracic curve and for whom information regarding kyphosis was
available, and it was 70 degrees (range, 54 to 84 degrees) in the five
patients who had a double major curve. Postoperior spinal arthrodesis and
instrumentation was performed in all patients. Two patients had an anterior
arthrodesis as well because of the severity and rigidity of the curve.
Allograft bone was used in eighteen patients. Postoperatively, all patients
were managed with a body cast or with a custom-molded thoracolumbar
brace.(ABSTRACT TRUNCATED AT 250 WORDS)
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