Anterior Total Hip Arthroplasty With Bulk Femoral Head Autograft in a Patient With Camurati-Engelmann Disease.

ABSTRACT

Camurati-Engelmann disease (CED) is an extremely rare, sclerosing bone disorder of intramedullary ossification with only 300 reported cases worldwide. The pathogenesis is related to activating mutations in transforming growth factor beta 1, which results in bilateral, symmetric hyperostosis affecting primarily the diaphysis of long bones. Despite effective pharmacological treatment options, the diagnosis of CED is problematic owning to its rarity and variability of clinical presentation. We present a patient with known CED with advanced early hip osteoarthritis, secondary to underlying hip dysplasia, for which she underwent a successful total hip arthroplasty via a direct anterior approach with the use of bulk femoral head autograft to reconstruct her native acetabulum.