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High-grade spondyloretrolisthesis in a 12-year-old girl with neurofibromatosis type 1: a case report and literature review

Martín-Fuentes, Ana María; Pretell-Mazzini, Juan; Curto de la Mano, Angel; Viña-Fernández, Rafael

Journal of Pediatric Orthopaedics B: March 2013 - Volume 22 - Issue 2 - p 110–116
doi: 10.1097/BPB.0b013e328357eac2

Neurofibromatosis (NF) type 1 is characterized by several skin, endocrine, central nervous system and musculoskeletal manifestations, spine deformities being the most common, affecting up to 64% of patients. Thoracic kyphoscoliosis is the most common deformity observed; however, high-grade spondylolisthesis and dural defects such as dural ectasia can also be found. The aim of this study is to describe a case of high-grade spondyloretrolisthesis in an NF-1 patient, associated with dural ectasia and extensive lumbar laminectomies, and to discuss our management and review the current literature on this controversial topic. A 12-year-old girl with NF-1 who had undergone extensive lumbar laminectomies in an outside facility presented to our emergency department complaining of back pain and lower limbs upper motor neuron symptoms. Image studies showed a high-grade lumbar spondyloretrolisthesis associated with dural ectasia. The first step of treatment was spine immobilization using a Boston brace. An anterior approach was used, and an L2 corporectomy was performed, using a Moss type cage between L1 and L3 with an instrumented arthrodesis and autologous bone graft for stabilization purposes. The second step planned was a posterior approach for arthrodesis and instrumentation, but after an extensive discussion with the parents and the patient, the parents did not agree to the procedure planned for the patient. A brace was used for 1 year while rehabilitation was performed. At the 1-year follow-up, there was a 70° kyphosis at the thoracolumbar junction but it was clinically stable, with an acceptable sagittal balance. Dural ectasia is not a common finding in children with NF-1; however, it should be identified as its presence may predispose to spine instability and as a consequence the development of a high-grade spondyloretrolisthesis. Even though a few cases have been reported, we believe that it is important to consider the option of a double approach to achieve a better correction in both planes and a good outcome. If an extensive laminectomy is involved, it is mandatory to perform a posterior fusion and instrumentation.

Pediatric Orthopaedic Division, Spine Unit, Doce de Octubre Hospital, Avda. De Córdoba s/n, Madrid, Spain

This work was presented at Pediatric Orthopaedic Division, Doce de Octubre Hospital.

Correspondence to Juan Pretell-Mazzini, MD, Pediatric Orthopaedic Division, Doce de Octubre Hospital, Avda. De Córdoba s/n, Madrid 28041, Spain Tel: +34 670 426 915; fax: +34 913 908 081; e-mail:

© 2013 Lippincott Williams & Wilkins, Inc.