title: Infantile inflammatory pseudotumor of the facial nerve as a complication of epidermal nevus syndrome with cholesteatoma.
abstract: 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.
authors: Department of Otolaryngology, Ehime University School of Medicine, Ehime, Japan. nhato@m.ehime-u.ac.jp
authors: Naohito
authors: N
authors: Hato
authors: Mika
authors: M
authors: Tsujimura
authors: Taro
authors: T
authors: Takagi
authors: Masahiro
authors: M
authors: Okada
authors: Kiyofumi
authors: K
authors: Gyo
authors: Mikiko
authors: M
authors: Tohyama
authors: Hisamichi
authors: H
authors: Tauchi
mesh_terms: D002781:Cholesteatoma / Q000150:complications*; D019299:Decompression, Surgical; D005155:Facial Nerve Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D005260:Female; D006104:Granuloma, Plasma Cell / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D006801:Humans; D007223:Infant; D009506:Nevus / Q000150:complications; D013577:Syndrome; D014057:Tomography, X-Ray Computed
keywords: Cholesteatoma; Epidermal nevus syndrome; Facial nerve; Inflammatory pseudotumor