Dural ectasia: a manifestation of type 1 neurofibromatosis

Abstract

Dural ectasia denotes circumferential expansion or dilatation of the dural sac, and has been frequently reported in association with type 1 neurofibromatosis (NF1). The pathogenesis has not been defined, but its correlation with NF1 infers a congenital malformative hypothesis. The neural elements in the dilated sleeve typically are not enlarged or abnormal, nevertheless the enlarged area contain an increased amount of cerebrospinal fluid. The dura in the area of ectasia is extremely thin and fragile, and erodes the surrounding bony structures destabilising the spine and permitting spectacular spinal deformities. We present a case: a 40-year-old woman suffering from neurofibromatosis type 1 who developed a thoracic dural ectasia and anterior meningocele.

Keywords

• dural ectasia
• neurofibromatosis type 1
• anterior meningocele