Chiari type I malformation related to hydrocephalus

Abstract

Chiari malformation is characterized by a caudal displacement of the cerebellar tonsils that penetrate into the spinal canal through the foramen magnum, managing to reach the atlas or axis, there is no descent of the trunk and neither of the fourth ventricle. Being of rare epidemiology with a generally variable clinical presentation. It was decided to present the case of a 21-year-old female patient, with no personal pathological history, who referred headache. The physical examination revealed the presence of holocranial headache, absence of cranial nerve alterations, absence of signs of neurological focalization and a 15 points Glasgow coma scale. Bilateral papilledema (Frisen grade 4) is observed in the fundus. The imaging tests showing herniation of the cerebellar tonsils greater than 5 mm below the Mac-Rae line, herniation of the lower portion of the medulla oblongata and triventricular obstructive hydrocephalus without associated syringomyelia. The diagnosis of Chiari malformation type I is confirmed. A right ventriculoperitoneal shunt was performed to correct the hydrocephalus. After six months of follow-up, post-shunt univentricular hydrocephalus was confirmed. Endoscopic septostomy was performed without complications and with a positive result. Chiari malformation is an entity with a low epidemiology, whose presentation may be asymptomatic, generally associated to hydrocephalus. Its surgical treatment is usually aimed at reestablishing the circulation of the cerebrospinal fluid and decompression of the posterior fossa.