Open AccessVon Recklinghausen’s Disease Presenting as Otolaryngologic Emergency: A Rare Occurrence
Author(s) -
Debajyoti Ghosh,
Jayanta Saha,
Shaswati Sengupta,
Ajay Manickam,
Sumit Basu
Publication year - 2017
Publication title -
indian journal of otolaryngology and head and neck surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.229
H-Index - 22
eISSN - 2231-3796
pISSN - 0973-7707
DOI - 10.1007/s12070-017-1092-7
Subject(s) - medicine , neurofibromatosis , otorhinolaryngology , plexiform neurofibroma , lesion , neurofibroma , disease , chin , neurofibromatoses , dermatology , head and neck surgery , surgery , pathology , anatomy
Type 1 Neurofibromatosis (NF1) is a rare autosomal dominant genetic disorder. There are seven clinical features of which two are necessary to diagnose it. Another important feature is plexiform neurofibroma which commonly presents as painful expansile lesion. Here we present a case of NF1 with huge swelling of left hemiface and chin following trauma over pre-existing swelling and presented as life threatening emergency and managed surgically.