Multidisciplinary approach for repair of a large ventral thoracic meningocele in neurofibromatosis-1; a systematic review of the literature and case report

Abstract

Thoracic meningoceles and dural ectasia are less commonly recognized manifestations of neurofibromatosis 1 (NF1). Rarely, large thoracic meningoceles may become compressive and lead to respiratory compromise secondary to lung compression. Surgical goals aim to increase lung aeration through decreasing the size of the meningocele through shunting, excision or repair of the meningocele, and varying degrees of dural tube reconstruction. There is no agreement on the best approach for large, symptomatic meningoceles. Here, we discuss the case of a 41-year-old woman with NF1 who presented with dyspnea and enlargement of a large, 19 cm thoracic meningocele. A multidisciplinary team of thoracic, plastic, and neurological surgery participated in the operation to excise the meningocele and reconstruct the dural tube without the need for subsequent shunting of spinal fluid. We also systematically review the literature on thoracic meningoceles in NF1 to understand the optimal treatment of this pathology.