Fibrous dysplasia, shepherd's crook deformity and an intra-capsular femoral neck fracture

Abstract

Fibrous dysplasia (FD) is a rare bone disorder in which normal medullary bone is replaced by fibro-osseous tissue. It typically presents in childhood with pain, skeletal deformities, gait abnormalities and occasionally, fatigue fractures. The management of FD remains a challenge. Surgical procedures have been developed to provide symptom relief, correct skeletal deformity and offer mechanical support in cases at risk of fracture. However, there is a paucity of data on the management of acute femoral neck fractures in the adult population with FD. We report the case of a 23-year-old man with a shepherd's crook deformity secondary to FD, who sustained an intra-capsular femoral neck fracture whilst playing football. The patient initially underwent closed reduction and internal fixation with cannulated screws. However, during the procedure, a guide wire broke whilst inside the femoral head. The patient was referred to the senior author, who undertook a second operation to remove the metalwork and correct the varus deformity using a closing-wedge femoral osteotomy, whilst achieving osteosynthesis at the fracture site. At 1-year follow-up, the patient is pain-free and demonstrates a full range of movement. These cases can be technically demanding and carry a greater risk of complication. It is important that preoperative planning is undertaken and surgery performed by individuals with experience in managing FD and complex femoral neck fractures. Correction of the skeletal deformity whilst fixing the fracture will help restore the mechanical axis and reduce the risk of a recurrent fracture. © 2013 The Author(s).