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Cranial disjunction and visual failure in a slit ventricle syndrome with patent shunt
P Van Bogaert1, C Raftopoulos, M Cordonnier
1Department of Neurology (Pediatric Neurology), Hôpital Erasme, Université Libre de Bruxelles, Belgium.
Insights
A shunt complication in a child with congenital toxoplasmosis led to blindness due to rapid intracranial pressure drop. This highlights a risk during intracranial hypertension management.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Ophthalmology
Background:
- Congenital toxoplasmosis can cause triventricular hydrocephalus requiring shunt placement.
- Intracranial hypertension with papilledema is a serious complication in pediatric hydrocephalus.
Observation:
- A 6.5-year-old child with a shunt for hydrocephalus developed intracranial hypertension and papilledema.
- Imaging revealed slit ventricles, and the shunt was patent.
- Spontaneous suture widening resolved headaches, but vision loss occurred.
Findings:
- Rapid intracranial pressure drop is suspected as the cause of irreversible vision loss.
- Optic nerve sheath fenestration did not restore vision.
Implications:
- Clinicians should consider rapid intracranial pressure changes as a cause of blindness in shunt patients.
- This complication is relevant when planning interventions like calvarial expansion in similar cases.
Abstract:
A 6.5-year-old child who received a shunt at 3 weeks of age for triventricular hydrocephalus related to his congenital toxoplasmosis developed symptoms of intracranial hypertension and papilloedema. Computed tomographic scan demonstrated slit ventricles. The shunt device was shown to be patent on isotope transit study. Spontaneously the cranial sutures widened and headaches disappeared, but loss of vision occurred and did not reverse despite optic nerve sheath fenestration. We suspect that a rapid drop in intracranial pressure played a role in the pathogenesis of our patient's blindness. This possible complication should be taken into account when calvarial expansion is planned in a patient with an intracranial hypertension syndrome with papilloedema in the presence of slit ventricles and a patent shunt.