Bone growth causing ventriculoperitoneal shunt malfunction in a patient with osteopetrosis. Case report.

Abstract

Osteopetrosis is an inherited skeletal condition of defective osteoclastic resorption of bone resulting in increased bone density. Osseous changes occur most severely at the base of the skull. Important clinical symptoms include cranial nerve palsies due to uni- or bilateral obliteration of cranial nerve foramina including deafness, facial paralysis, and optic nerve compression. Thickening of the skull and progressive diminution of the cranial capacity may lead to elevation of intracranial pressure, papilledema, and hydrocephalus. The authors present an unusual case of a patient with osteopetrosis in whom a ventriculoperitoneal shunt became obstructed at its exit from the skull by the developing bone mass. To the best of their knowledge, this has not been described.