Sclerosteosis: neurosurgical experience with 14 cases.

Abstract

Fourteen cases of sclerosteosis seen during a 14-year period have been reviewed. Sclerosteosis is a craniotubular bone modeling disorder and presents with cranial nerve palsies and raised intracranial pressure; sudden death may occur without neurosurgical intervention. Experience gained from the management of these patients suggests that bifrontal decompressive craniotomy followed by a posterior fossa decompression should be performed in all cases, preferably in the second decade of life. Cerebrospinal fluid diversion procedures to reduce elevated intracranial pressure are unsatisfactory. Patients should be followed closely since the regrowth of bone may necessitate repeated decompressive procedures. Technical problems related to the surgical management are discussed.