Neurofibromatosis type 1 of the head and neck: dilemmas in management

The Journal of Laryngology and Otology
F RapadoM Small

Abstract

Neurofibromatosis type 1 (NF 1) or Von Recklinghausen's disease is an autosomal dominant condition characterized by multiple skin café-au-lait lesions and multiple neurofibromas. In the head and neck neurofibromas have a predilection to arise in th deep planes of the neck. Surgical management is nearly always required for functional or cosmetic reasons or to exclude the possibility of malignant transformation. We present four cases of neurofibroma of the head and neck and illustrate the difficulties inherent in managing this condition, particularly the age range in which it may occur, the risk of malignant transformation, the possibility of abnormal bleeding and the morbidity that may be associated with the surgical resection. The role of magnetic resonance (MR) scan in the assessment and monitoring of these lesions is highlighted. We also report a unique association of a pharyngeal pouch with NF 1 in a young (25-year-old) patient.

Citations

Jun 20, 2007·Aesthetic Plastic Surgery·Selma Sönmez ErgünHusamettin Yasar
Sep 27, 2006·The Kaohsiung Journal of Medical Sciences·Hsun-Mo WangWen-Rei Kuo
Nov 28, 2008·Journal of Plastic, Reconstructive & Aesthetic Surgery : JPRAS·Milan StojicicDanijela Stojmirovic
Jun 5, 2002·Plastic and Reconstructive Surgery·Sebnem Hanioglu KargiBulent Erdogan
Jun 1, 2007·The Journal of Craniofacial Surgery·Maria Teresa FaddaStefano S Calvieri
Mar 9, 2010·Journal of Neurologic Physical Therapy : JNPT·Kristin M Helmers, Kent E Irwin

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