Background:

Epidermoid cyst is a benign lesion that represents about 1 % of intracranial neoplasms, of embryonal origin and frequent in the media line.

Objective:

To present a rare case of an epidermoid cyst within the fourth ventricle in a patient who had hydrocephalus in the fifth decade of life.

Clinical report:

49 years old, white male patient, who presented headache, dizziness and gait instability. He was conscious when neurologic physical examination, with appearances of a cerebellar vermis syndrome. Computed axial tomography also simple and contrasted nuclear magnetic resonance imaging studies showed a homogeneous, rounded lesion with low contrast acquisition, within the fourth ventricle, accompanied by triventricular hydrocephalus. The surgical strategy was first oriented to place a ventricle-peritoneal shunt, then direct surgical treatment of the lesion through a posterior fossa medial craniotomy. After dural surgery, a pearly lesion was observed, approximately 2 cm diameter, encapsulated, which allowed its complete resection and the reestablishment of cerebrospinal fluid circulation. The patient evolved positively with total recovery of his clinical manifestations and without any sequela.

Conclusions:

Epidermoid cysts, although predominant in the media line are very rare within the ventricular system. Magnetic resonance imaging is the choice study, the positive histopathological diagnosis and complete surgical resection allowed the patient to be cured.

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