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

Spine Deform. 2015 May;3(3):239-245. doi: 10.1016/j.jspd.2014.10.003. Epub 2015 Apr 23.


Study design: This 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).

Objective: To evaluate the effectiveness of rib-based distraction and associated complications in managing scoliosis in the growing child with NF-1.

Summary of background data: Patients 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.

Methods: Twelve 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.

Results: Mean 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.

Conclusions: The 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.

Keywords: Complications; Neurofibromatosis; Scoliosis; VEPTR.