Valve Pressure Upgrade May Produce Progressive Deterioration of Vision in Children with Slit Ventricle Syndrome

Recently, valve upgrade and/or endoscopic third ventriculostomy, which have the merit of no additional shunting, were introduced for the treatment of slit ventricle syndrome, because lumboperitoneal shunting entails various complications including development of Chiari malformation, shunt malfunction, and infection. However, the safety of valve upgrading is not confirmed, especially in a child with slit ventricle syndrome developed as a result of pseudotumor cerebri. A 5-year-old boy with pseudotumor cerebri presented with headache, intermittent vomiting, and sudden deterioration of visual acuity. His cerebrospinal pressure during lumbar puncture was 69 cm H 2 O and his magnetic resonance imaging revealed only small ventricles. He underwent a ventriculoperitoneal shunt resulting in dramatic improvement. Four months later, he returned with recurrent spontaneous valve malfunction with recurrent severe headache and visual deterioration. After shunt revision with a programmable valve, his intermittent valve malfunction was improved by upgrading the valve opening pressure. However, his visual acuity became progressively aggravated. He underwent a lumboperitoneal shunt with low-pressure valve, which resulted in the disappearance of intermittent headaches and a deterioration of visual acuity. We suggest that valve pressure upgrade in children with slit ventricle syndrome after ventriculoperitoneal shunt for pseudotumor cerebri may produce acute deterioration of vision that had already been compromised, even within the normal intracranial pressure range and with improvement of associated symptoms.