The association of spinal deformity with dural ectasia in neurofibromatosis type 1

Br J Neurosurg. 2019 Dec;33(6):620-623. doi: 10.1080/02688697.2019.1673313. Epub 2019 Oct 4.

Abstract

Objective: To establish the prevalence of dural ectasia in patients with complex neurofibromatosis type-1 (NF-1) and its association with spinal deformities that manifest alongside.Methods: Analysis of the neuroradiological notes from 24 months of multidisciplinary team meetings for patients with complex NF-1 (equating to 378 patients). Review of the MRI scans of all patients with dural ectasia with each scan graded using a novel grading system.Results: A total of 38 of the 378 patients were found to have dural ectasia (10.05%). The median age of these 38 patients was 36 years ranging from 18 to 64. The male: female ratio was 16:22. In all, 90.9% of patients with a 'major' form of dural ectasia had a concurrent spinal deformity present compared to 18.18% of patients with a minor form.Conclusions: The more severe the dural ectasia, the greater the likelihood a concurrent deformity with NF-1. The vertebral bodies and pedicles are more commonly involved than the posterior elements.

Keywords: Neurofibromatosis; dural ectasia; neurofibromatosis type 1; scoliosis; spinal deformity.

MeSH terms

  • Adolescent
  • Adult
  • Dilatation, Pathologic
  • Dura Mater / pathology*
  • Female
  • Humans
  • Incidence
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neurofibromatosis 1 / complications*
  • Neurofibromatosis 1 / diagnostic imaging
  • Neurofibromatosis 1 / pathology*
  • Spine / abnormalities*
  • Spine / diagnostic imaging
  • Spine / pathology*
  • Young Adult