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

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

Author information

1
University of Utah, Department of Orthopedics, Primary Children's Hospital, 100 N. Mario Capecchi Dr., Suite 4550, Salt Lake City, Utah, 84113, USA. Electronic address: johnaheflin@gmail.com.
2
William Beaumont Army Medical Center, Orthopedic Surgery Department, 5005 N. Piedras St. Bldg. 7777, El Paso, TX, 79920, USA.
3
University of Utah, Department of Orthopedics, Primary Children's Hospital, 100 N. Mario Capecchi Dr., Suite 4550, Salt Lake City, Utah, 84113, USA.

Abstract

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

PMID:
27927465
DOI:
10.1016/j.jspd.2014.10.003

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