Visual disturbance following shunt malfunction in a patient with congenital hydrocephalus

A 25-year-old woman presented with complaints of nausea and headache. She had been treated with a ventriculoperitoneal shunt for hydrocephalus when she was 7 months old. Her bilateral optic discs showed moderate atrophy. Right visual acuity allowed only perception of hand movement and left visual acuity was 0.02 (1.2). Computed tomography (CT) showed mild ventricular dilation but no periventricular lucency. Intracranial pressure (ICP) was not high when the shunt valve was punctured. Her visual acuity deteriorated 5 days after the consultation. She was referred again 8 days after the first consultation. The bilateral optic discs were completely pale. Both pupils were dilated on admission, and the bilateral direct light reflexes were absent. The patient could slightly detect only green light stimulus. CT showed moderate enlargement of the ventricle. ICP was 47 cmH(2)O when the shunt valve was punctured. Shuntgraphy showed obstruction of the shunt at the distal end of peritoneal catheter. Emergent total shunt revision was performed. She could detect dark stimulus and the still-dilated left pupil had recovered direct light reflex on the next day. The visual acuity was 0.01 (0.7) on the left 6 months after the operation, although she was blind in the right eye and the bilateral optic discs were completely pale. Visual loss associated with shunt failure remains a major morbidity in shunted congenital hydrocephalus. Early diagnosis and shunt revision may allow visual recovery.