Osteopetrosis - a challenge for the orthopaedic surgeon

Osteopetrosis (OP) is a rare heterogeneous group ofinherited skeletal dysplasias characterised by osteoclastdysfunction, impaired bone resorption and poorbone remodelling. Three groups can be categorised onthe basis of clinical findings. These include neurologicalsymptoms, haematological abnormalities and renaltubular acidosis in the first group. Increased bone density,osteomyelitis and frequent fractures are the clinicalfindings in the second group, and the third group havenormal life expectancy but may develop cranial nervecompression and osteomyelitis. Fractures in patientswith OP are common and require appropriate pre-,peri- and postoperative management. The long bonesare most frequently affected, fractures of the femoralneck and proximal (upper third) shaft being particularlycommon. This case report proposes possible operativefracture treatment in a patient with OP and highlightsthe potential perioperative pitfalls in this rare surgicalpopulation.