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[儿童呼吸困难的罕见原因。喉丛状神经纤维瘤]

[An uncommon cause of dyspnea in children. Plexiform neurofibroma of the larynx].

作者信息

Moussali N, Belmoukari S, Elmahfoudi H, Elbenna N, Abdelouafi A, Gharbi A

机构信息

Service de radiologie, hôpital 20-Août, CHU Ibn-Rochd, Casablanca, Maroc.

出版信息

Arch Pediatr. 2013 Jun;20(6):629-32. doi: 10.1016/j.arcped.2013.03.011. Epub 2013 Apr 28.

Abstract

Plexiform neurofibroma is a rare benign tumor developed from cells of the sheath of Schwann peripheral nervous system often associated with neurofibromatosis type 1 or Von Recklinghausen disease. Laryngeal involvement in neurofibromatosis is rare and usually presents with obstructive respiratory symptoms. Imaging including CT and MRI provides the diagnosis as well as the staging of this lesion, whose treatment is primarily surgical. We report the case of a plexiform neurofibroma of the larynx found in a 4-year-old child revealing a mass completely obstructing the laryngeal vestibule.

摘要

丛状神经纤维瘤是一种罕见的良性肿瘤,由施万外周神经系统鞘细胞发展而来,常与1型神经纤维瘤病或冯·雷克林豪森病相关。神经纤维瘤病累及喉部较为罕见,通常表现为阻塞性呼吸道症状。包括CT和MRI在内的影像学检查可用于该病变的诊断和分期,其治疗主要为手术治疗。我们报告一例在一名4岁儿童中发现的喉部丛状神经纤维瘤病例,该肿瘤显示为一个完全阻塞喉前庭的肿块。

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