Chiari I malformation associated with Gorham's disease of the skull base

Abstract

Gorham’s disease, or ‘vanishing bone disease’, is a rare condiion characterised by lymphangiomatosis of bone which manifests s osteolysis. It may affect any bone and patients may present with a yriad of symptoms depending on the affected region, but typically ain, swelling or a pathological fracture in childhood is characterstic [1]. The condition is likely under recognised or misdiagnosed. rimary involvement of the skull base is atypical but the insidious nset of tinnitus, deafness and otorrhoea may all result from the ondition. Here we report a case of adult Gorham’s disease with nvolvement of the skull base presenting clinically with a Chiari I alformation.