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

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 10 cm. 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.

Pérdida de agudeza visual y parálisis de VI nervio craneal como únicas manifestaciones de un síndrome de colapso ventricular / Visual acuity loss and sixth nerve palsy as the only manifestations of slit ventricle syndrome

Se describe una niña con hidrocefalia obstructiva por un astrocitoma pilomixoide, que requirió implantar una derivación ventrículo-peritoneal (DVP) a los 5 años y 10 meses de edad. Dos meses después, la resonancia magnética cerebral no mostró ventriculomegalia ni otros signos de aumento de la presión intracraneal. A la edad de 6 años y 2 meses desarrolló una rápida disminución de la agudeza visual bilateral siendo diagnosticada de síndrome de colapso ventricular. A pesar de las revisiones valvulares de la DVP, se produjo una disminución abrupta de la agudeza visual a movimientos de mano a 10cm. El examen del fondo de ojo reveló atrofia óptica bilateral. No refirió otros síntomas sistémicos que sugirieran un aumento de la presión intracraneal, como dolor de cabeza, náuseas, vómitos, letargia, irritabilidad o niveles alterados de conciencia.

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.

Visual acuity loss and sixth nerve palsy as the only manifestations of slit ventricle syndrome. / Pérdida de agudeza visual y parálisis de VI nervio craneal como únicas manifestaciones de un síndrome de colapso ventricular.

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.

Fluorescence guided resection with 5-aminolevulinic acid of a pilomyxoid astrocytoma of the third ventricle.

Fluorescence-guided resection with 5-aminolevulinic acid has been shown to be useful in the resection of certain brain tumors other than high grade gliomas, facilitating the intraoperative differentiation of neoplastic tissue. The technique enables the surgeon to ensure that no tumor fragments remain, thereby achieving higher rates of complete resection. Tihan first described pilomyxoid astrocytomas in 1999. They are currently classified as grade II astrocytoma according to the WHO classification system and, because of their tendency to recur and their dissemination through the cerebrospinal fluid pathways, they are considered to be more aggressive than pilocytic astrocytoma. As a result, management of these tumors must be more aggressive, always aiming for complete macroscopic resection whenever possible. In this article, we present a case of pilomyxoid astrocytoma of the third ventricle in which the use of fluorescence-guided resection with 5-ALA facilitated complete resection. Imaging tests performed after five years revealed no signs of recurrence and no adjuvant radiotherapy or chemotherapy was required. This article also comprises a review of the literature concerning the characteristics and management of this tumor, which was recently considered to be a different histopathological entity.