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Von Recklinghausen's Disease Presenting as Otolaryngologic Emergency: A Rare Occurrence
Debangshu Ghosh1,2, Jayanta Saha3, Shaswati Sengupta1
11Department of ENT, R.G.Kar Medical College, Kolkata, West Bengal India.
Abstract:
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.
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