Ophthalmic complications of slit-ventricle syndrome in children.

OBJECTIVE

To present the ophthalmic features and visual prognosis of patients with slit-ventricle syndrome (SVS).

DESIGN

Observational case series.

PARTICIPANTS

Six patients diagnosed and treated with SVS at the Montreal Children's Hospital between 1985 and 1999.

METHODS

Patients were included in this study if they had an appropriate ophthalmologic follow-up and if they fulfilled the criteria for the diagnosis of SVS based on intracranial pressure monitoring and neuroimaging studies.

MAIN OUTCOME MEASURES: Features studied included patients' baseline ophthalmologic evaluation, visual outcome, and neurosurgical characteristics.

RESULTS

The follow-up ranged from 1 to 14 years. The average number of shunting procedures was 3.7. Ocular examination performed on the initial visit revealed a normal visual acuity in four patients, esotropia in three of six patients, and nystagmus in two of six patients. Cycloplegic refraction was normal in all the subjects. Initial funduscopic evaluation revealed optic atrophy in a 4-month-old infant, whereas two children developed optic atrophy later in the course of the disease. One child developed severe visual field defects. The two children with optic atrophy had moderate to severe loss of visual acuity associated with SVS.

CONCLUSIONS

A prompt recognition of patients with SVS is crucial, because these individuals are at an increased risk for significant visual loss.