Patients with autosomal-recessive osteopetrosis presenting with hydrocephalus and hindbrain posterior fossa crowding
Yahia Z Al-Tamimi1, Atul K Tyagi, Paul D Chumas
1Neurosurgery Department, Leeds General Infirmary, Leeds, United Kingdom. yahia27@hotmail.com
Abstract:
Osteopetrosis is a heterogeneous group of disorders characterized by abnormal bone sclerosis. As a result, patients often require input regarding various neurological complications. Although autosomal-recessive osteopetrosis has been associated with hydrocephalus, it has not been linked to hindbrain abnormalities. The authors present 3 cases of auto-somal-recessive osteopetrosis in patients who presented with hydrocephalus. In each of these patients, cerebrospinal fluid diversion procedures were required and hindbrain compression developed. To date, only 1 patient has needed craniocervical decompression due to symptomatic brainstem compression.
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