R Mahdoufi*, I Barhmi, N Tazi, R Sami, R Abada, M Roubal and M Mahtar
ENT-Neck and Face Surgery, Hospital August 20, 1953, University Hospital IBN ROCHD, Casablanca, Morocco
Received: 24 June, 2016; Accepted: 01 July, 2016; Published: 04 July, 2016
R Mahdoufi, ENT-Neck and Face Surgery, Hospital August 20, 1953, University Hospital IBN ROCHD, Casablanca, App. H1, Résidence Mawlid IX, Rue Brahim Nakhaii, Maarif, Casablanca, Morocco, Tel: +212663466994; +212617173539; E-mail:
Mahdoufi R, Barhmi I, Tazi N, Sami R, Abada R, et al. (2016) Plexiform Neurofibroma of the Larynx in Children: About an Unusual Case Report. Arch Otolaryngol Rhinol 2(1): 035-037. DOI: 10.17352/2455-1759.000020
© 2015 Mahdoufi R, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Larynx; Plexiforme; Neurofibroma; Child
NF: Neurofibroma; CT: Computed Tomography; MRI: Magnetic Resonance Imaging
Introduction: The plexiform neurofibroma is a rare benign tumor which is often associated to type 1 neurofibromatosis or Von Recklinghausen’s disease. The laryngeal involvement in neurofibromatosis is extremely rare.
The objective of our report is the consideration of laryngeal neurofibromatosis in the differential diagnosis of dyspnea in infants and children.
Summary of the clinical case: We report the case of a 4 year-old child who presented with inspiratory dyspnea. Laryngoscopy revealed a mass obstructing completely the laryngeal vestibule. Pathological examination of the resected tissue revealed a plexiform neurofibroma after immunohistochemical examination. The patient had regular follow up and she remained symptom free.
Discussion: There is an association between neurofibromatosis type 1 and plexiform neurofibromas of the larynx. Analysis of the margins of neurofibromas of the larynx associated with neurofibromatosis type 1 may help predict clinical behavior
The plexiform neurofibroma is a benign tumor of the peripheral nerves, made at the expense of the connective cells perineurium, often considered pathognomonic of neurofibromatosis type 1.
The patient can develop tumors of neural origin at any age and at any location. However, laryngeal involvement in neurofibromatosis remains rare, and is usually manifested by obstructive airway symptoms. Because its rarity and our inexperience, it is often misdiagnosed.
In this review we discuss the pertinent clinical findings of this rare lesion and review the literature relative to laryngeal neurofibroma.
A 4 year-old-girl was admitted to our department with obstructive respiratory symptoms. She had a history of laryngeal stridor since birth, more intense during physical activity and screams, associated with progressive dyspnea and nocturnal snoring; there was no history of previous intubation or foreign body ingestion.
The physical exam showed a substernal draw with flaring nose. And found many café-au-lait spots over the trunk and limbs Figure 1.