Abstract
BackgroundThe 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.MethodsThe authors reviewed a case series consisting of 6 patients who underwent spine surgery for scoliosis. Children's mean age at index surgery was 12 years and 10 months (range, 10 to 15 yrs). Clinical evaluation revealed the typical phenotypic features 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 80.8° (range, 65° to 96°). 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.ResultsThe mean clinical and radiological follow-up was 3 years and 10 months (range, 2 years to 9 years). Major complication rate was 50%. One patient who developed a major intraoperative complication (paraparesis) prevented spinal fusion to be obtained: the neurologic deficit resolved completely 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.ConclusionsSpine reconstructive surgery in patients with PWS is rare and highly demanding.The best method of reconstruction is posterior multilevel pedicle screw fixation. Moreover, even with modern 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 techniques, requiring repeated surgeries, should be carefully evaluated in each single case.
Highlights
Spinal diseases are considered supportive criteria with no direct impact on the diagnostic score established by Holm et al The syndrome is associated with growth hormone deficiency (GHD)
The literature dealing with scoliosis surgery in Prader-Willi syndrome (PWS) consists of only few case reports with a relatively small number of patients [9,10,11,12,13,14,15]; generally speaking, the onset of PWS, its curve pattern, the course of progression, guidelines for treatment and surgical indications have been reported to resemble those of the adolescent idiopathic scoliosis [8]
There is no definite consensus on the indications of surgical correction and instrumentation in PWS scoliosis
Summary
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; there are very few case reports describing surgical treatment for this deformity. The reported prevalence of scoliosis varies from 45% to 86%, whereas the hyperkyphotic deformity can be observed in nearly 40% of the patients [5,7,8]. The pharmacological therapy with growth hormone (GH) provides satisfactory results, its impact on the progression of spinal deformities still remains controversial [5,6]. Scoliosis is reported to require active treatment in 15% to 20% of the cases and to behave as an idiopathic scoliosis, with a high risk of progression during adolescence [1,7]
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