Abstract
Background: To present the experience of three centres in the surgical management of scoliosis in patients with Prader-Willi syndrome1, and to describe the associated complications. A retrospective case series reviewing treatment type, surgical outcomes and complications with a review of the literature
Methods: There were 15 patients treated surgically in the three centres over an eleven year period. The average age at initial surgery was 9.6 (5 to 16+8) yrs. Minimum follow-up from index procedure was 28 months. 5 underwent posterior spinal fusion, 2 had anterior fusion alone and 2 had combined anterior/ posterior fusions as their index procedure. 6 patients were initially managed with growing rod constructs. Outcome measures included fusion rate, curve progression and complication rate.
Results: 11 patients achieved a solid fusion. 2 patients were still undergoing the lengthening process and were thus unfused. 1 patient had broken instrumentation with pseudarthrosis, and one patient not fit for final fusion remained without implants. No curve progression was seen in the 11 fused spines. Progressive kyphosis and scoliosis were noted in the two ongoing lengthening patients. There were 17 revision procedures not including planned lengthenings, with a total of 59 surgical procedures. 5 rod breakages occurred in the growing-rod patients. There were 11 infections (4 deep) in 7 patients. There were 6 respiratory complications, 3 requiring ventilatory support. One patient had post-operative neurological deterioration requiring re-exploration and who had grade 3 paraparesis at final follow-up. In total there were 36 complications (240%)
Conclusion: The outcome of surgical management of scoliosis in Prader-Willi syndrome is adversely affected by the obesity and respiratory difficulties of the patients and complications abound2. The use of growing constructs, whilst enabling the management of the growing child, greatly increases the risk of implant failures. Their use in Prader-Willi requires careful consideration.
The abstracts were prepared by Mr Colin E. Bruce. Correspondence should be addressed to Colin E. Bruce, Consultant Orthopaedic Surgeon, Alder Hey Children’s Hospital, Eaton Road, Liverpool, L12 2AP.
References:
1 Gurd AR, Thompson TR. Scoliosis in Prader-Willi syndrome. J Pediatr Orthop. 1981;1(3):317–20 Google Scholar
2 Rees D, Jones MW, Owen R, Dorgan JC. Scoliosis surgery in the Prader-Willi syndrome. J Bone Joint Surg Br.1989 Aug;71(4):685–8 Google Scholar
