폐쇄성 수면 무호흡으로 발현한 후두 신경섬유종 1례
A Case of Laryngeal Neurofibroma with Sleep Obstructive Apnea
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초록
후두의 신경섬유종은 매우 드문 것으로 알려져 있으며, 대부분 상기도 폐쇄 증상으로 발현된다. 저자들은 수면 시 무호흡과 호흡곤란을 주소로 내원한 30개월 환자에서 후두 폐색을 유발하는 종괴를 확인하였으며 다수의 담갈색 반점과 신경섬유종증의 가족력을 근거로 신경섬유종으로 진단하였다. 병변의 위치상 수술적 절제술은 시행하지 않고 기관절개술을 통해 증상이 호전된 증례를 경험하였기에 보고하는 바이다.
Abstract
Most patients with neurofibromas suffer from neurofibromatosis type 1 (von Recklinghausen’s disease), which is characterized by café-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.
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