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

A Case of Laryngeal Neurofibroma with Sleep Obstructive Apnea

• Kim JH
• Ko H
• Seo JH
• Kim HY
• Yu JH
• Hong SJ

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

MeSH Terms

Apnea
Cafe-au-Lait Spots
Deglutition Disorders
Dyspnea
Hoarseness
Humans
Larynx
Magnetic Resonance Imaging
Neurofibroma
Neurofibromatoses
Neurofibromatosis 1
Preschool Child
Recurrence
Respiratory Sounds
Sleep Apnea, Obstructive
Tracheostomy

Figure

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  Fig. 1. Chest radiograph showed (A) cardiomegaly with pulmonary congestion before tracheostomy and (B) improved cardiomegaly after tracheostomy.

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  Fig. 2. Neck computed tomography scan showed asymmetric obliteration of nasopharynx and larynx secondary to ill-defined hypodense mass-like lesion.

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  Fig. 3. An axial magnetic resonance imaging revealed about 4 cm sized, hyperintense, minimally enhancing, multinodular and infiltrative mass involving left parapharyngeal and left carotid space.

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  Fig. 4. Bronchoscopic examination showed omega shape edema in the supraglottic area adjacent to the epiglottis.

Reference

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