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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.
AuthorsNaohito Hato, Mika Tsujimura, Taro Takagi, Masahiro Okada, Kiyofumi Gyo, Mikiko Tohyama, Hisamichi Tauchi
JournalAuris, nasus, larynx (Auris Nasus Larynx) Vol. 40 Issue 6 Pg. 569-72 (Dec 2013) ISSN: 1879-1476 [Electronic] Netherlands
PMID23433476 (Publication Type: Case Reports, Journal Article)
CopyrightCopyright © 2013 Elsevier Ireland Ltd. All rights reserved.
Topics
  • Cholesteatoma (complications)
  • Decompression, Surgical
  • Facial Nerve Diseases (diagnostic imaging, etiology, surgery)
  • Female
  • Granuloma, Plasma Cell (diagnostic imaging, etiology, surgery)
  • Humans
  • Infant
  • Nevus (complications)
  • Syndrome
  • Tomography, X-Ray Computed

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