Complications of scoliosis surgery in Prader-Willi syndrome.
METHODS
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.


BACKGROUND
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.


CONCLUSIONS
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.
Accadbled
Franck
F
Department of Orthopedic Surgery, Hôpital des Enfants, Toulouse, France. faccadbled@wanadoo.fr
Odent
Thierry
T
Moine
Alexandre
A
Chau
Edouard
E
Glorion
Christophe
C
Diene
Gwenaelle
G
de Gauzy
Jérôme Sales
JS
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications; D011218:Prader-Willi Syndrome / Q000601:surgery*; D012189:Retrospective Studies; D012307:Risk Factors; D012600:Scoliosis / Q000601:surgery*; D016896:Treatment Outcome