Infantile inflammatory pseudotumor of the facial nerve as a complication of epidermal nevus syndrome with cholesteatoma
Infantile inflammatory pseudotumor of the facial nerve as a complication of epidermal nevus syndrome with cholesteatoma
复制标题
婴儿面神经炎性假瘤是表皮痣综合征合并胆脂瘤的并发症
DOI：
10.1016/j.anl.2012.11.016
复制
发表时间：
2013
影响因子：
1.7
通讯作者：
Tauchi H.
中科院分区：
文献类型：
--
作者：
Hato N;Tsujimura M;Takagi T;Okada M;Gyo K;Tohyama M;Tauchi H.
本文报告了首例因面神经炎性假瘤(IPT)引起的面瘫病例，这是表皮痣综合征(ENS)的并发症。一名10个月大的女性患者在3个月大时被诊断为ENS。她是因为中度左面神经麻痹而转诊给我们的。她左耳廓的表皮痣深入到外耳道。耳镜检查显示左耳充满息肉样痣和胆脂瘤碎片。计算机断层扫描显示，包括中耳在内的耳道内充满了一个软性肿块，以及一个巨大的面神经。手术探查发现许多息肉样痣、外耳胆脂瘤和面神经肿胀。中耳听小骨完全消失。减压术后面瘫明显改善，5个月后复发。第一次手术后10个月进行了第二次手术。面神经减压术从膝状神经节至茎乳孔附近完成。面神经肿瘤的组织学诊断为IPT，可能是由表皮痣引起的慢性外耳炎所致。第二次手术后5年，面瘫逐渐改善至House-Blackmann III级。
The first reported case of facial paralysis due to an inflammatory pseudotumor (IPT) of the facial nerve as a complication of epidermal nevus syndrome (ENS) is herein presented. A 10-month-old female patient was diagnosed with ENS at 3 months of age. She was referred to us because of moderate left facial paralysis. Epidermal nevi of her left auricle extended deep into the external ear canal. Otoscopy revealed polypous nevi and cholesteatoma debris filling the left ear. Computed tomography showed a soft mass filling the ear canal, including the middle ear, and an enormously enlarged facial nerve. Surgical exploration revealed numerous polypous nevi, external ear cholesteatoma, and tumorous swelling of the facial nerve. The middle ear ossicles were completely lost. The facial paralysis was improved after decompression surgery, but recurred 5 months later. A second operation was conducted 10 months after the first. During this operation, facial nerve decompression was completed from the geniculate ganglion to near the stylomastoid foramen. Histological diagnosis of the facial nerve tumor was IPT probably caused by chronic external ear inflammation induced by epidermal nevi. The facial paralysis gradually improved to House–Blackmann grade III 5 years after the second operation.