Congenital intracranial frontotemporal dermoid cyst presenting as a cutaneous fistula.

BACKGROUND: Intracranial extension and a cutaneous sinus tract are rarely seen with craniofacial dermoid cysts, with few cases reported in the literature.

METHODS: We report a case of a 1-year-old girl who was initially seen with a cutaneous fistula of the frontotemporal region, which revealed an intracranial dermoid cyst.

RESULTS: The patient underwent a right lateral orbitotomy by a bicoronal approach. The cyst was seated within the lateral orbital wall, with intracranial extension through the temporal and sphenoidal bones to the dura of the temporal lobe. Histopathologic analysis confirmed the diagnosis of a dermoid cyst.

CONCLUSIONS: Craniofacial dermoid cysts may be associated with a cutaneous sinus tract and/or intracranial extension. Failure to recognize and promptly treat these lesions may lead to a progressive skeletal distortion and/or recurrent infection with a potential for meningitis or cerebral abscess. Therefore, detailed CT and MRI scans are mandatory before surgical treatment of any cutaneous fistula in the head and neck region.