SCOLIOSIS SURGERY IN PRADER-WILLI PATIENTS: AN UNCOMPLICATED SURGICAL PATHWAY.

Abstract

Background: Prader-Willi syndrome is associated with multiple musculoskeletal manifestations including scoliosis, joint laxity, hip dyplasia and lower limb deformity. Scoliosis is reported in almost half of patients with Prader-Willi; however, only a small proportion ever have surgery. The literature suggests that surgery in this group of patients is often difficult. Obesity and apnea cause anaesthetic concern. The surgical procedure is reported as being complicated by excessive intra-operative blood loss, and difficulty with instrumentation; possibly as a result of osteopenia, is described.

Purpose: To compare the experience of scoliosis surgery in Prader-Willi patients in our institution with that previously reported in the literature.

Methods: The notes and x-rays of 6 patients with Prader-Willi syndrome who had corrective scoliosis were reviewed.

Results: Six patients (4 female and 2male) underwent corrective scoliosis surgery. The mean age at which scoliosis was detected in the patients was 5 years (range 2–7years). The mean Cobb angle at time of initial referral was 30° (range 8°–86°). Indication for surgery in all cases was curve progression. The mean age at time of surgery was 11.8 years (range 10–13years). The mean pre-operative Cobb angle was 70° (range 40°–90°) and mean post-operative Cobb angle was 23° (range 10°–40°). Three patients had posterior surgery with instrumentation, one had anterior instrumentation only and two patients had combined anterior release with posterior instrumentation. The most proximal instrumentation levels ranged from T2–T6 and the most distal instrumentation levels ranged from T11-L5. Three patients had all pedicle screw fixation, 2 had hybrid fixation (with screws at curve apex) and one had anterior all screw fixation. The mean operative time was 335 mins (range 190–540 mins) and the mean blood loss was 29% of total blood volume (range 14–55%). Standard anaesthetic technique was used in each case and all patients were extubated immediately after surgery. Patients were discharged on average 8 days post surgery (range 6–14 days). One patient had a wound infection and a stitch granuloma requiring surgical debridement and one patient required trimming of proximal end of rod because it was prominent.

Conclusion: Scoliosis surgery in patients with Prader-Willi offers adequate deformity correction and in our experience is not associated with operative technical difficulty, major complications or a prolonged hospital stay.