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Does the Presence of Dystrophic Features in Patients With Type 1 Neurofibromatosis and Spinal Deformities Increase the Risk of Surgery?

Lykissas, Marios G. MD, PhD*; Schorry, Elizabeth K. MD; Crawford, Alvin H. MD*; Gaines, Sean DO*; Rieley, Margaret MD; Jain, Viral V. MD*

doi: 10.1097/BRS.0b013e31829a7779
Surgery

Study Design. Retrospective chart and radiographical review.

Objective. To present the demographics of patients with scoliosis and neurofibromatosis type 1 (NF-1), to record the incidence of dystrophic features, and to determine whether the presence of dystrophic features increase the risk of surgery in patients with NF-1 and associated spinal pathology.

Summary of Background Data. The most common of the osseous complications of NF-1 is spinal deformity, occurring in 10% to 30% of individuals with NF-1. Many of these patients will eventually require surgery for curve progression, which makes study of demographics and identification of features predicting the need for surgery essential in this patient population.

Methods. A retrospective review was performed in patients with NF-1 and spinal deformities, followed in a multidisciplinary neurofibromatosis center. A subset of 56 patients with complete radiographical evaluation was reviewed for identification of risk factors for spine surgery.

Results. One hundred thirty-one patients from a population of 694 patients with NF-1 (19%) had scoliosis. Mean age at diagnosis of scoliosis was 9 years (range; 1–17 yr). Scoliosis and need for surgery were equally distributed between males and females. In the group of 56 patients, 63% had 3 or more dystrophic features. The presence of 3 or more dystrophic features was the strongest predictor of the need for surgery (odds ratio = 14.34; P < 0.001). Individual features most predictive of need for surgery were the presence of vertebral scalloping (odds ratio = 13.19; P < 0.001) followed by the presence of dural ectasia (odds ratio = 6.38; P = 0.005). Patients with no dystrophic features were unlikely to progress to need for surgery.

Conclusion. Scoliosis and need for surgery were equally distributed between males and females. The presence of 3 or more dystrophic features was highly predictive of the need for surgery, with the most significant individual predictors being vertebral scalloping and dural ectasia. A combination of radiographical and MRI features can be used to predict need for spinal surgery.

Level of Evidence: 3

A retrospective review was performed in patients with neurofibromatosis type 1 and spinal deformities. Scoliosis and need for surgery were equally distributed between males and females. The presence of 3 or more dystrophic features was highly predictive of the need for surgery, with the most significant individual predictors being vertebral scalloping and dural ectasia.

*Divisions of Orthopaedic Surgery and

Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

Address correspondence and reprint requests to Alvin H. Crawford, MD, Cincinnati Children's Hospital Medical Center, Division of Orthopaedic Surgery, 3333 Burnet Ave, MLC 2017, Cincinnati, OH 45229; E-mail: Alvin.Crawford@cchmc.org

Acknowledgment date: November 26, 2012. First revision date: January 18, 2013. Second revision date: February 18, 2013. Third revision date: March 12, 2013. Fourth revision date: April 25, 2013. Acceptance date: April 29, 2013.

The manuscript submitted does not contain information about medical device(s)/drug(s).

No funds were received in support of this work.

Relevant financial activities outside the submitted work: grants.

© 2013 by Lippincott Williams & Wilkins