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.