Pediatr Allergy Respir Dis.  2011 Jun;21(2):131-136.

A Case of Laryngeal Neurofibroma with Sleep Obstructive Apnea

Affiliations
  • 1Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. sjhong@amc.seoul.kr
  • 2Childhood Asthma Atopy Center, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Abstract

Most patients with neurofibromas suffer from neurofibromatosis type 1 (von Recklinghausen's disease), which is characterized by cafe-au-lait spots and cutaneous neurofibromas. Neurofibromas in the laryngeal area are extremely rare. Most patients with a laryngeal neurofibroma present with dyspnea, dysphagia, stridor, or hoarseness, depending on the location and size of the tumor. We present a case of a laryngeal neurofibroma in a boy with neurofibromatosis type 1. A 30-month-old boy with neurofibromatosis presented to our hospital with respiratory difficulty and obstructive sleep apnea. Magnetic resonance imaging demonstrated a round mass over the left carotid space, and its location made surgical resection impossible. Hence, tracheostomy was performed to maintain airway patency. The patient's symptoms improved after tracheostomy. Long-term follow-up is essential owing to the possibility of recurrence. The patient has thus far shown no obstructive airway symptoms for one year after closure of the tracheostomy.

Keyword

Neurofibroma; Neurofibromatosis; Larynx; Obstructive airway symptoms
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