DR. SHUBHANGI DORKAR

DR. PRAJAKTA SALUNKHE

Abstract

Introduction

Neurofibromatosis type 1 is a rare autosomal dominant genetic disorder. Plexiform neurofibroma is an uncommon variant (30% ) of NF1. It arise from multiple nerves as bulging and deforming masses involving connective tissue.

Material and Methods

We report a 61 year old male patient who came for facial and lid growth.

He had multiple plexiform neurofibromas on his both upper lid ,entire face, ear lobe and scalp region which has distorted his appearance. The patient was councelled for a reconstructive facial surgery but he was not willing for same. Histopathology of biopsy samples showed overgrowth of peripheral nerve component. CT scan brain showed multiple soft tissue lesions in the scalp.

Results – Plexiform neurofibromas an uncommon variant of NF 1 is a rare disfiguring condition.Early identification and surgical intervention needed to prevent gross facial disfigurement. Screening for malignancy should be done and regular follow ups needed.