Complications of scoliosis surgery in Prader-Willi syndrome.

STUDY DESIGN

Retrospective case series of 16 consecutive patients.

OBJECTIVE

To evaluate the results of scoliosis surgery in Prader-Willi syndrome (PWS) with special emphasis on the complications and their risk factors.

SUMMARY OF BACKGROUND DATA

PWS is a rare genetic disorder characterized by obesity, hypotonia, and frequent scoliosis. The literature dealing with scoliosis surgery in PWS consists of only few case reports. Surgical treatment was reported to be difficult with frequent and severe complications.

METHODS

Sixteen patients (3 males, 13 females) in 63 presenting scoliosis and PWS were operated on between 1974 and 2004. Preoperative, postoperative and last follow-up clinical and radiologic data were analyzed. Complications, treatment, and outcome were investigated.

RESULTS

Mean age at scoliosis diagnosis was 6.2 years (range 0.5-13.5). Mean age at surgery was 12.3 years (range 5-15). Mean follow-up was 5.4 years (range 2-18). There were 9 major complications (4 severe kyphosis above fusion, 2 deep infections, 1 transient paraplegia, 1 pseudarthrosis, 1 delayed wound healing). The 4 kyphosis required reoperation, 3 of which were complicated with permanent spinal cord injury. Minor complications affected 6 patients.

CONCLUSION

Scoliosis surgery is frequently necessary in PWS and is associated with high rate of complications. These are often related to specific features of this syndrome the surgeon should recognize and consider.