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

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
• neurofibromatosis type 1
• dural ectasia
• scoliosis
• spinal deformity

Acknowledgements

The authors would like to thank Dr Calvin Soh for providing the neuroradiological notes from the MDT meetings and Dr Alexandru Cotici for assistance with MRI analysis. The authors would also like to thank Mr. Ryan Siabi and Miss Rima Shah for their assistance with conducting statistical analyses.

Disclosure statement

The authors report no conflicts of interest. This work has not been published elsewhere. We declare the following presentation: Poster presentation at the Joint Global Neurofibromatosis Conference 2018, Paris.

Author contributions

All authors met ICJME recommendations for authorship:

• SS collected data, performed data analysis and wrote the manuscript.

• KJG aided with the study design and method, helped in devising the grading systems and provided comments on manuscript drafts.

• AC helped in MRI analysis using devised grading system.

• CS provided neuroradiological notes from MDT meetings.