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

• Published in: Spine (Philadelphia, Pa. 1976) Vol. 38; no. 18; pp. 1595 - 1601
• Main Authors: Lykissas, Marios G., Schorry, Elizabeth K., Crawford, Alvin H., Gaines, Sean, Rieley, Margaret, Jain, Viral V.
• Format: Journal Article
• Language: English
• Published: United States by Lippincott Williams & Wilkins 15-08-2013
• Subjects: Adolescent; Child; Child, Preschool; Female; Humans; Infant; Male; Neurofibromatosis 1 - diagnostic imaging; Neurofibromatosis 1 - epidemiology; Neurofibromatosis 1 - surgery; Radiography; Retrospective Studies; Risk Factors; Scoliosis - diagnostic imaging; Scoliosis - epidemiology; Scoliosis - surgery; Treatment Outcome
• ISSN: 0362-2436; 1528-1159
• DOI: 10.1097/BRS.0b013e31829a7779

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 Evidence3