Treatment of Scoliosis in patients with Prader-Willi

ScoliosisJournal report: Treatment of scoliosis in patients affected with Prader-Willi syndrome using various different techniques

Authors: Tiziana Greggi, Kostas Martikos, Francesco Lolli, Georgios Bakaloudis, Mario Di Silvestre, Alfredo Cioni, Giovanni Barbanti Brodano and Stefano Giacomini

Background
The incidence of spinal deformity in children with Prader-Willi syndrome (PWS) is high, with 86% of these patients found to have a significant structural scoliosis; however, there are very few case reports describing surgical treatment for this deformity.

Methods
The authors reviewed a case series consisting of 6 patients who underwent spine surgery for scoliosis. Children's mean age at first surgery was 12 years and 10 months (range, 10 to 15 yrs). Clinical evaluation revealed the typical phenotype of the PWS in all of the patients; 4 subjects had a karyotype confirmation of PWS. Major structural curves showed preoperative mean Cobb angles of 80.8degrees (range, 65degrees to 96degrees). Hybrid instrumentation with sublaminar wires, hooks and screws was used in the first 2 patients, while the remaining 4 were treated with titanium pedicle screw constructs.

Results
The mean clinical and radiological follow-up was 3 years and 10 months (range, 2 years to 9 years). Major complication rate was 50%. One major intraoperative complication (paraparesis) prevented spinal fusion to be obtained: it completely resolved after instrumentation removal. Solid arthrodesis and deformity correction in both coronal and sagittal plane was, however, achieved in the other 5 cases and no significant curve progression was observed at follow-up. Another major short-term complication was encountered 3 months after surgery in a patient who experienced the detachment of a distally located rod and required correction through revision surgery and caudal extension by one level. Cervico-thoracic kyphosis was seen in 1 patient who did not require revision surgery.

Conclusions
Surgery in patients with PWS is rare and highly demanding, and is best suited for posterior multilevel pedicle screw constructs. Moreover, even with modern, expert techniques, the risk of complications is still high. These new techniques, however, have shown to improve the postoperative course by allowing for immediate mobilization without any brace or cast. The use of the growing rod technique, requiring repeated surgeries, should be carefully evaluated in each single case. 

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