Matt B H
Department of Otolaryngology, Head and Neck Surgery, Indiana University School of Medicine, Riley Hospital, Indianapolis 46202-5230, USA.
Int J Pediatr Otorhinolaryngol. 1996 Oct;37(2):163-72. doi: 10.1016/0165-5876(96)82359-7.
To alert practitioners to the risk of sudden airway obstruction and death in patients with Wegener's granulomatosis.
Case report and literature review.
University and Community hospitals.
A 17-year-old white female.
(1) evaluation and treatment for mental status changes over 2 months. (2) Evaluation and surgical biopsy of nasal septal perforation under general anesthesia at a university children's hospital. (3) Evaluation and observation at a community hospital 2 days later. (4) autopsy.
Serology performed 3 days ante-mortem revealed (at 2 days post-mortem) cytoplasmic anti-neutrophil cytoplasmic antibody positive at 1:128. Autopsy was significant for microscopic fibrosis and granulomas in the kidneys and essentially total obstruction of the subglottis and upper trachea by a 3.5 x 1 x 1 cm mass of fibrosis and granulomas overlying circumferentially necrotic mucosa. This mass was centered on the crico-tracheal junction.
Wegener's granulomatosis can lead to proliferative tissue growth with acute airway obstruction in the larynx and trachea, and death. Any patient with WG under age 20, as well as patients with WG and laryngotracheal symptoms (e.g. strider, hoarseness, wheezing) would benefit from evaluation of the airway.
提醒从业者注意韦格纳肉芽肿患者发生气道突然阻塞和死亡的风险。
病例报告及文献综述。
大学医院和社区医院。
一名17岁白人女性。
（1）对2个月来精神状态变化进行评估和治疗。（2）在一家大学儿童医院全身麻醉下对鼻中隔穿孔进行评估和手术活检。（3）2天后在一家社区医院进行评估和观察。（4）尸检。
死前3天进行的血清学检查（死后2天）显示，胞浆抗中性粒细胞胞浆抗体呈阳性，滴度为1:128。尸检发现，肾脏有微观纤维化和肉芽肿，声门下和气管上部基本上完全被一个3.5×1×1厘米的纤维化和肉芽肿肿块阻塞，该肿块覆盖着周向坏死的黏膜，以环状软骨气管交界处为中心。
韦格纳肉芽肿可导致喉部和气管出现增生性组织生长并伴有急性气道阻塞，进而导致死亡。任何20岁以下的韦格纳肉芽肿患者，以及有韦格纳肉芽肿且伴有喉气管症状（如喘鸣、声音嘶哑、喘息）的患者，都将受益于气道评估。
