Visual acuity loss and sixth nerve palsy as the only manifestations of slit ventricle syndrome

Abstract

The case is presented of a girl diagnosed with obstructive hydrocephalus due to pilomyxoid astrocytoma, which required a ventriculoperitoneal shunt (VPS) at the age of 5 years and 10 months. Two months later, magnetic resonance imaging of the brain did not show ventriculomegaly or other signs of increased intracranial pressure. At the age of 6 years and 2 months, a rapid onset of bilateral visual acuity loss developed and she was diagnosed with slit ventricle syndrome. Despite valve revisions of the VPS, she developed an abrupt decline of visual acuity to hand motion at 10cm. Fundus examination revealed bilateral optic atrophy. She did not report any other systemic symptoms suggesting increased intracranial pressure, such as headache, nausea, vomiting, lethargy, irritability, or altered levels of consciousness.

Keywords: Astrocitoma pilomixoide; Atrofia óptica; Derivación ventrículo-peritoneal; Hidrocefalia obstructiva; Hipertensión intracraneal; Intracranial hypertension; Obstructive hydrocephalus; Optic atrophy; Paresia de VI nervio craneal; Pilomyxoid astrocytoma; Sixth nerve palsy; Slit ventricle syndrome; Síndrome de colapso ventricular; Ventriculomegalia; Ventriculomegaly; Ventriculoperitoneal shunt.