Endoscopic third ventriculostomy for hydrocephalus in osteopetrosis: a case report and review of the literature.

INTRODUCTION

There are very few reports in the literature associating in hydrocephalus in osteopetrosis. As a complication of shunt procedure, there are two reports on shunt malfunction due to osseous overgrowth at the burr hole in patients with osteopetrosis. We herein report a case of osteopetrosis with hydrocephalus that was successfully treated with endoscopic third ventriculostomy (ETV).

CASE REPORT: At 5 months of age, a male patient presented with developmental delay. Head computed tomography (CT) demonstrated triventricular hydrocephalus with a cerebellar tonsillar herniation. At 7 months of age, he underwent suboccipital decompression with decompression of the foramen magnum. The hydrocephalus did not improve postoperatively, and the patient was transferred to our hospital. At 12 months of age, the hydrocephalus was successfully treated with ETV. The postoperative period was uneventful. Postoperative CT demonstrated an improvement in the ventricle size.

CONCLUSIONS

The etiology of hydrocephalus in osteopetrosis is not completely understood; however, there have been several reports in which ETV was effective. ETV should be considered the treatment of choice for hydrocephalus in osteopetrosis, as it avoids the characteristic shunt complications that can occur in patients with osteopetrosis.