Harshavardhan J K Giriraj, Uppalapati Bharghava Ram, Menon P Gopinath
Department of Orthopaedics, Sri Ramachandra Medical University, Chennai, Tamil Nadu, India.
J Orthop Case Rep. 2018 May-Jun;8(3):33-37. doi: 10.13107/jocr.2250-0685.1096.
Fibrous dysplasia is a rare benign disorder of the skeletal system characterized by fibro-osseous proliferation with intervening areas of normal or immature bone in the intramedullary region. It can either be a monostotic (involves one bone) or a polyostotic (involves more than one bone) presentation and usually occurs equally in males and females. Deformities such as scoliosis and shepherd's crook deformity are frequently encountered in the polyostotic form. We report the management of a rare managed case of bilateral shepherd's crook deformity of the proximal femur with impending neck of femur fracture.
A 22-year-old male known case of polyostotic fibrous dysplasia presented with bilateral shepherd's crook deformity of the proximal femur. Preoperatively, neck-shaft angle was 15° on the right side and 55° on the left side. The patient had severe pain on the right side due to an impending neck of femur fracture. On the right side, we first did a valgus osteotomy in the subtrochanteric region and fixed with dynamic hip screw, and after 3 months, the left proximal femur valgus osteotomy with dynamic hip screw fixation was done. Postoperatively, we achieved a neck-shaft angle of 140° on the right side and 135° on the left side. 1-year follow-up imaging showed union at both the osteotomy sites. At present, at 12-month post-operative, the patient is walking full weight-bearing without support, no apparent shortening.
Valgus osteotomy is an easy and effective method to correct the shepherd's crook deformity and achieve correct mechanical alignment. Valgization of the shepherd crook deformity will prevent the development of a pathological neck of femur fracture.
骨纤维异常增殖症是一种罕见的骨骼系统良性疾病，其特征是骨髓腔内存在纤维-骨组织增生，其间夹杂正常或未成熟骨区域。它可以是单骨型（累及一块骨）或多骨型（累及一块以上骨）表现，通常在男性和女性中发病率相同。脊柱侧弯和牧羊拐畸形等畸形在多骨型中较为常见。我们报告了一例罕见的近端股骨双侧牧羊拐畸形且有股骨颈骨折倾向病例的治疗情况。
一名22岁的多骨型骨纤维异常增殖症男性患者，出现近端股骨双侧牧羊拐畸形。术前，右侧颈干角为15°，左侧为55°。由于存在股骨颈骨折倾向，患者右侧疼痛严重。右侧，我们首先在转子下区域进行外翻截骨，并用动力髋螺钉固定，3个月后，对左侧近端股骨进行外翻截骨并采用动力髋螺钉固定。术后，右侧颈干角达到140°，左侧为135°。1年的随访影像学检查显示截骨部位均愈合。目前，术后12个月时，患者可在无支撑下完全负重行走，无明显短缩。
外翻截骨是纠正牧羊拐畸形并实现正确力学对线的一种简单有效的方法。牧羊拐畸形的外翻可预防病理性股骨颈骨折的发生。
