Shepherd's crook deformity: How to set it straight. A five-step surgical guide.

PURPOSE: Fibrous dysplasia (FD) of the proximal femur can result in severe deformity and disability. The results of surgical management in such situations have been reported to be poor. We present a novel, five-step surgical treatment to correct proximal femoral deformity in FD.

MATERIAL AND

METHODS

This study is a review of prospectively collected data of cases of proximal femur polyostotic FD managed at our institute from 2012 onward. We managed three patients with FD involving four femora (one patient had bilateral disease). Mean age at presentation was 16 years with an average follow-up of 46 months. All underwent five steps, that is, (1) curettage/excision of the lesion, (2) allograft in intramedullary region, (3) lateral closing wedge valgus osteotomy, (4) fixation with extramedullary implant, and (5) augmentation of osteotomy site with autograft. Functional outcome was assessed using Harris Hip Score preoperatively and at the final follow-up.

RESULTS

The neck shaft angle was corrected from an average of 91.7° to 152.1°, while the Harris Hip Score improved from an average of 59 to 95. There was no clinical or radiological evidence of recurrence of disease or deformity in any patient till the last follow-up.

CONCLUSION

This five-step technique ensures good functional and radiological outcomes in the management of proximal femur FD.