Complications of growth-friendly techniques in early onset scoliosis of neurofibromatosis type 1: a case series.

PURPOSE

Neurofibromatosis type 1 (NF-1) is a genetic disease with many clinical manifestations, including early onset scoliosis (EOS). There has been increasing interest in the use of growing rod constructs in these patients. Our study seeks to understand the complication rates of these procedures in patients with NF-1.

METHODS

In this multicenter retrospective study, demographic data, radiologic measurements, type of surgical construct, complication information, and deformity outcomes were obtained. This information was then summarized using descriptive statistics in order to evaluate complication rates in the total cohort, between different types of curves, and between different surgical constructs.

RESULTS

Thirteen patients (8 males and 5 females) were included in the study, eight with dystrophic and five with non-dystrophic scoliosis. The mean preoperative major Cobb angle was 67.8° (median 75°, interquartile range [IQR] 53-83°). Four types of growth-friendly constructs were employed: seven vertical expandable prosthetic titanium rib (VEPTR), four traditional growing rod (TGR), one magnetically controlled growing rod (MCGR), and one Shilla. Major complications occurred in 11/13 patients (85%). Three patients (23%) had surgical site infections, nine (69%) had instrumentation complications, four (31%) had an unplanned surgical intervention other than for fusion, two (15%) had neurological complications, three (23%) had other complications, and seven (54%) required early surgical fusion. Two patients (15%) had additional complications after definitive fusion. The rate of overall complication rates was similar for dystrophic versus non-dystrophic curves (88% versus 80%).

DISCUSSION

There is a high rate of complications associated with growth-friendly constructs in patients with NF-1 dystrophic and non-dystrophic scoliosis. These complications must be weighed against the advantages of these procedures when deciding on the optimal procedure for patients with NF-1 EOS.