Rare Case of C4 Perineurinoma with Lambdatic Parieto-occipital Neuroma in Von Recklinghausen Disease in a 5 Years Old Child: A Case Report

Abstract

Neuro fibromatosis groups together distinct diseases that most often share only certain cutaneous signs. Neurofibromatosis (NF) is a neurocutaneous syndrome characterized by the development of tumors of the central or peripheral nervous system including the brain, spinal cord, organs, skin, and bones. Prineurinoma is a relatively rare tumour that has been shown to be a proliferation of perineural cells with an incomplete basal lamina and no onion bulb appearance. Neuroma also known as traumatic neuroma or amputation neuroma or stump neuroma, is a focal non-neoplastic area of proliferative hyperplastic reaction secondary to peripheral nerve damage that commonly occurs after a focal trauma (acute or chronic) or surgery, such as amputation or partial transection; We report an extremely rare clinical case of a 5 year old child with neurfibromatosis congenita type 1 with asyntomatic medullary comrepssion and incidental discovery of a left intraductal C4 perineurinoma during the exploration of an occipital-parietal neuroma associated with a dehiscence of the lambda suture. He was operated on, with the postoperative course marked by paresis of the cervical muscles, which was in the process of recovery by physiotherapy.