Use of Rib-Based Distraction in the Treatment of Early-Onset Scoliosis Associated With Neurofibromatosis Type 1 in the Young Child

Abstract

Study DesignThis study was an institutional review board–approved retrospective review of a prospectively collected multicenter database for rib-based distraction systems used in the treatment of young children with early-onset scoliosis associated with neurofibromatosis type 1 (NF-1). ObjectiveTo evaluate the effectiveness of rib-based distraction and associated complications in managing scoliosis in the growing child with NF-1. Summary of Background DataPatients with NF-1 commonly have scoliosis with curves that can be dysplastic and progressive and respond poorly to bracing. Rib-based distraction systems have recently been described in the management of these complex patients. The efficacy and complication rate using these systems have not previously been reported. MethodsTwelve children with NF-1 and scoliosis who were treated with rib-based distraction systems were identified from a prospectively collected multicenter registry. Preoperative and postoperative Cobb angle and T1–S1 spine height were measured from posteroanterior radiographs. The number of lengthenings, age at implantation, years of follow-up, and complications were also acquired from the registry or patient charts. ResultsMean age at implantation of the Vertical Expandable Prosthetic Titanium Rib device was 6.34 years. Mean preoperative Cobb angle was 66.3°. Average follow-up was 5.2 years. Mean postoperative Cobb angle was 60.8° after an average of 7.75 lengthening procedures (range, 2–16 procedures). T1–S1 height increased in all patients. There were 17 complications in 8 patients (device migration in 6, wound dehiscence in 3, rod breakage in 2, medical issues in 5, and 1 revision for progression of curve). Of the 17 complications, 10 were grade I, 1 was grade II, and 6 were grade IIA; there were no grade III complications. ConclusionsThe use of rib-based distraction is an effective and relatively safe method of stabilizing curve progression through growth in severe dysplastic scoliosis associated with NF-1.