Crook Deformity Research Articles

Shepherd’s crook right coronary artery: a multidetector computed tomography coronary angiography study

Shepherd's crook deformity of the right coronary artery (RCA) is considered a haemodynamically non-significant course anomaly. It may be challenging for cardiologists during percutaneous transluminal coronary angioplasty. To investigate the radiological anatomy of the high riding course of the proximal segment of the RCA, especially focused on the Shepherd's crook RCA (SCRCA), on multidetector computed tomography (MDCT) coronary angiography. A total of 1,118 MDCT coronary angiography examinations were reviewed retrospectively (349 [31.2%] male; 769 [68.8%] female, age range from 18 to 88 years [54.4 ± 14.7 years, mean ± standard deviation]). We classified the segments that had a higher course than RCA ostium (high riding RCA) into two types: type 1--courses turning inferior with an angle equal to or more than 90 degrees, type 2 (SCRCA)--courses turning inferior with an angle less than 90 degrees. The origin of the sinoatrial node artery (SANA) and conus artery were also noted. High riding RCA was detected in 146 (13%) patients. Of them, 105 (71%) had type 1 and 41 (29%) had type 2. Atherosclerotic plaque formations, to varying degrees, were presented in 10 (24.3%) of the patients with SCRCA. Approximately half of SANA originated from the SCRCA segment of the RCA in SCRCA cases. This is the first study about SCRCA that has been performed with MDCT coronary angiography. This study brings important anatomical knowledge to cardiologists. To have knowledge about the origin of the SANA prior to PTCA procedures is important to prevent possible arrhythmias and infarcts.

Two-Stage Surgical Treatment of Complex Femoral Deformities with Severe Coxa Vara in Polyostotic Fibrous Dysplasia

In patients with polyostotic fibrous dysplasia with extensive femoral involvement, severe coxa vara may cause complex femoral deformities that are difficult to treat with a single-stage surgical procedure. We evaluated the results of treatment of such patients with a two-stage procedure. Eleven patients with polyostotic fibrous dysplasia and severe coxa vara (including one who required bilateral treatment and one who required repeat treatment) were treated surgically at a mean age of fourteen years and four months. A two-stage surgical procedure was planned to correct the deformity. The first stage involved correction of the coxa vara and fixation with a hip plate. The second stage involved correction of a shepherd's crook deformity if present and definitive fixation with an interlocking cervicodiaphyseal nail. All patients were evaluated clinically and radiographically at a mean of four years and seven months after the second-stage procedure. The femoral neck-shaft angle averaged 83° before surgery and was corrected to a mean of 130° after the first-stage procedure. In two patients, cutout of the hip screw-plate caused acute postoperative loss of correction (by 40° in one patient and 20° in the other). After the second-stage procedure, the neck-shaft angle was either fully corrected or improved and the shepherd's crook deformity was fully corrected in all patients. At the time of the latest follow-up, the mean neck-shaft angle was 124°, with a mean loss of correction of 5° relative to the angle measured at the end of the second-stage procedure. The mean estimated blood loss was 625 mL in the first stage and 979 mL in the second. The two-stage surgical procedure designed to treat patients with polyostotic fibrous dysplasia with complex femoral deformity and severe coxa vara restored a nearly normal femoral alignment that was maintained at a mean of four years and seven months of follow-up. The clinical benefits were pain relief in all of the patients and gait normalization or improvement in most. The estimated blood loss was substantial in both stages. Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

9. A case report of mazabraud's syndrome and literature review

Background Mazabraud's syndrome is rare and has characteristic radiological and histopathological findings. Within radiological circles the appearance is such a common radiology registrar trap that it is considered a ‘spotter’ amongst practicing radiologists. The pathological appearance of fibrous dysplasia and intramuscular myxomas are individually characteristic however the differing natural history and management means that the connection between both entities may not be apparent on histopathology alone. Clinicoradiological findings A 43 year old man presented with a 2 month history of a tender mass below the inguinal ligament. An ultrasound of the right thigh found a 4.4 cm solid intramuscular tumour which was subsequently imaged with X-ray and MRI and showed typical features of polyostotic fibrous dysplasia involving the pelvis and proximal femur with a Shepherd's crook deformity and multiple soft tissue masses with features of intramuscular myxomas and the diagnosis of Mazabraud's syndrome was given. Six years after the initial diagnosis the patient presented after a fall with a pathological subtrochanteric femoral fracture. A total hip replacement was performed and one intramuscular lesion was sampled. Pathological findings The femoral neck was deformed and the medulla was cystically expanded by blood and fragments of pale grey fibrous tissue which had ‘Chinese character’ shaped metaplastic trabeculae on morphology. The myxoid lesion was surrounded by a thin rim of incomplete skeletal muscle and had a gelatinous grey surface which was hypocellular and hypovascular microscopically confirming the presence of fibrous dysplasia and intramuscular myxoma and thus the diagnosis of Mazabraud's syndrome. Conclusion The combination of fibrous dysplasia and intramuscular myxoma is characteristic of Mazabraud's syndrome.

Fibrous dysplasia of the proximal femur: surgical management options and outcomes.

PurposeThe management of proximal femoral deformity in fibrous dysplasia (FD) is a challenge to the orthopaedic surgeon. The purpose of this study was to analyze the various presentations of FD of proximal femur and the results of the various treatment modalities for the same.MethodsThis is a retrospective cohort study of 23 patients (24 femora) with FD who underwent surgery for the proximal femur. The study sample included 14 males, nine females. Ten patients had a monostotic disease, eight patients had polyostotic disease, and five patients had McCune-Albright syndrome.ResultsGroup 1: shepherd crook deformity—included five patients who underwent femoral neck osteotomy. Four patients had intramedullary (IM) nailing with neck cross-pinning and all patients showed union. One patient was stabilized with external fixation, which failed. Group 2: nine patients (ten femora) presented with frank pathological fracture. Nine underwent fixation with IM nailing, one with locking plate and screws. Three patients had to undergo more than one procedure and all fractures showed good union. Group 3: nine patients who presented with bone cyst and pain. All patients underwent biopsy; four of them had curettage with bone graft.ConclusionShepherd crook deformity can be treated by a well-planned osteotomy and fixation with intramedullary implants with neck cross-pinning. Frank pathological fractures fixation with an intramedullary nail has excellent results even if not accompanied by resolution of the fibrodysplastic lesion. More than one procedure may be required. External fixation is not an optimal choice for fixation of femoral osteotomies in FD.

Shepherd’s crook deformity の観血的治療経験

Shepherd’s crook deformity の観血的治療経験

Radiographic Classification of Coronal Plane Femoral Deformities in Polyostotic Fibrous Dysplasia

Fibrous dysplasia of bone is a skeletal dysplasia with a propensity to affect the femur in its polyostotic form, leading to deformity, fracture, and pain. The proximal femur is most commonly involved with a tendency to distal progression, thereby producing the typical shepherd's crook deformity. However, there are few data on the spectrum and progression of femoral deformities in polyostotic fibrous dysplasia. The purposes of this study were (1) to develop a radiographic classification for polyostotic fibrous dysplasia; (2) to test this classification's intra- and interobserver reliability; and (3) to characterize the radiographic progression of polyostotic fibrous dysplasia in a population of patients with the condition who were treated with a variety of approaches at several centers. We retrospectively reviewed radiographs of 127 femurs from 84 adult patients affected by polyostotic fibrous dysplasia. Fifty-nine femurs had undergone one or more operations. The radiographs were evaluated in the coronal plane for neck-shaft angle and angular deformities along the whole femoral shaft down to the distal epiphysis. Four observers evaluated each film two times at intervals; intra- and interobserver reliability testing was performed using the kappa statistic. Eighty-nine femurs (70%) were available for followup to evaluate for progression at a mean of 10 years (range, 6-20 years). Six reproducible patterns of deformity were identified in both untreated and operated femurs: type 1 (24%), normal neck-shaft angle with altered shape of the proximal femur; type 2 (6%), isolated coxa valga with neck-shaft angle > 140°; type 3 (7%), isolated coxa vara with neck-shaft angle < 120°; type 4 (20%), lateral bowing of the proximal half of the femur associated with normal neck-shaft angle; type 5 (14%), like in type 4 but associated with coxa valga; and type 6 (29%), like in type 4 but associated with coxa vara. Interobserver and intraoberver kappa values were excellent, ranging from 0.83 to 0.87. In 46 of the 89 femurs (52%) for which longitudinal radiographic documentation was available, there was progressive worsening of the original deformity, although the pattern remained the same; types 1 and 2 tended not to progress, whereas types 3 to 6 did. A reproducible radiographic classification of polyostotic fibrous dysplasia-associated femoral deformities is proposed, which can serve as a tool for assessing and treating these deformities. After reviewing the radiographs of 127 femurs, we identified six reproducible patterns of femoral deformities. Level III, diagnostic study. See Guidelines for Authors for a complete description of levels of evidence.

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

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.

Skeletal and Soft Tissue Involvement in Mazabraud Syndrome

Mazabraud syndrome is characterized by fibrous dysplasia and muscular myxomas (1). It is frequently associated with the McCune-Albright syndrome, which includes polyostotic fibrous dysplasia, cafe-au-lait spots, and endocrine abnormalities as a result of activating mutations of the GNAS1 gene (2). A 45-year-old woman presented with the McCune-Albright syndrome and difficulties with walking. She had precocious puberty, short stature, a history of over 20 fractures, cafe-au-lait spots, and femoral deformities. On x-ray, femoral shaft bowing (shepherd’s crook deformity) and bony lesions with smooth sclerotic margins and hazy matrix, consistent with fibrous dysplasia, were noted, but no fractures (Figure 1A). Four weeks later, the patient presented with acute left hip pain and was unable to walk. Femoral neck fracture was diagnosed and treated with placement of a dynamic hip screw (Figure 1B). In addition, a jaw cyst was removed in 2007, and bilateral mastectomy was performed because of hemorrhagic nipple discharge, which revealed ductal papilloma. A sc forearm lesion removed in 2011 revealed a myxoma (Figure 1C). This case of Mazabraud syndrome consists of the McCune-Albright syndrome and myxoma, combined with ductal papillomas and jaw cysts. Although bisphosphonate therapy was considered, given the history of multiple fractures, extremely low bone mineral density (Z-score at the spine, 6.1), and high bone turnover, we refrained from it because of the patient’s history of jaw bone cysts (3). The patient is currently treated with calcium/vitamin D supplementation and estrogen replacement therapy. On close follow-ups, she has sustained no further fractures.

Surgical treatment of fibrous dysplasia in the proximal femur

The aim of this study was to summarize oncological and functional results and to investigate surgical treatment methods and efficacies by conducting a retrospective study of patients with fibrous dysplasia (FD) in the proximal femur. A total of 15 patients with FD in the proximal femur were selected. Among them, 12 cases were monostotic and 3 cases were polyostotic. In addition, 2 cases were accompanied by shepherd’s crook deformity. All cases received internal fixation following focus curettage and impaction grafting. Among them, valgus osteotomy was conducted for 2 cases of shepherd’s crook deformity. All patients were followed up for 12–32 months. For 2 patients with shepherd’s crook deformity in the proximal femur, the collodiaphysial angle was recovered after an orthopedic procedure following osteotomy. In addition, no patient presented with postoperative recurrent lesions. At 3 months following surgery, local bone resorption was visible in the bone grafting area. Between 8 and 12 months after surgery, the bones in the bone grafting area had healed, pain had disappeared and gaits were nearly normal. An effective internal fixation following thorough focus curettage and allograft bone transplantation is an effective method of treating FD in the proximal femur. For patients with shepherd’s crook deformity, it is also necessary to perform valgus osteotomy to recover hip joint function.

Valgus osteotomy in combination with dynamic hip screw fixation for fibrous dysplasia with shepherd’s crook deformity

The treatment of fibrous dysplasia with shepherd's crook deformity is a big challenge. The purpose of this study was to investigate the clinical effect of valgus osteotomy in combination with dynamic hip screw (DHS) fixation to treat fibrous dysplasia with shepherd's crook deformity. Twenty-one clinical cases of femoral fibrous dysplasia with shepherd's crook deformity treated between April 2001 and May 2010 were retrospectively analyzed. The valgus osteotomy and internal fixation were performed for these patients. Six patients underwent DHS and trochanter stabilizing plate internal fixation, and the other 15 cases were stabilized by DHS fixation. Patients were followed for 19-128 months. The neck-shaft angle was corrected from 89° (range 65°-107°) preoperatively to 129° (range 119°-140°) postoperatively. Limb-length discrepancy was corrected from 3.0 (range 1.8-4.5) cm preoperatively to 0.7 (range 0-1.9) cm postoperatively. All osteotomies had healed at the final follow-up examination. The clinical scores, which were evaluated by the modified criteria of Guille, improved from an average of 2.9 (range 1-7) to 8.5 (range 6-10). Our results demonstrate that valgus osteotomy in combination with DHS internal fixation is an easy and effective method for the treatment of fibrous dysplasia with shepherd's crook deformity. It can restore the neck-shaft angle and re-establish the mechanical alignment of the femur to improve function.

One-Stage Osteotomy and Fixation Using a Long Proximal Femoral Nail and Fibular Graft to Correct a Severe Shepherd's Crook Deformity in a Patient with Fibrous Dysplasia: A Case Report

We report a case where a one-stage osteotomy and fixation, using a long proximal femoral nail and fibular graft, was performed to correct a severe shepherd's crook deformity (70-degree varus and 50-degree retroversion) of the femoral neck with a pathological stress fracture in a patient with fibrous dysplasia. The neck shaft angle was corrected to 125 degrees. At the 57-month follow-up, the patient was free of pain and had no limp or evidence of recurrence.

Valgus osteotomy combined with intramedullary nail for Shepherd’s crook deformity in fibrous dysplasia: 14 femurs with a minimum of 4 years follow-up

To investigate the effectiveness of valgus osteotomy combined with intramedullary nail in treatment of Shepherd's crook deformity of fibrous dysplasia. A retrospective study was performed in 13 patients (14 femora) of fibrous dysplasia who were treated in our hospital between August 2000 and February 2005. All patients had Shepherd's crook deformity. Six patients had monostotic disease, and seven had polyostotic disease. There were seven males and six females. The four-step procedure was performed orderly as valgus osteotomy, curettage lesion, massive impaction allograft, and insert intramedullary nail with neck cross pinning. All patients were followed up from 4 to 7 years with an average duration of 75.3 months. The average neck-shaft angle was corrected from preoperative 75 degrees (range 55 degrees -100 degrees ) to postoperative 120 degrees (range 95 degrees -130 degrees ). The average extremity lengthening was 3.4 cm (range 2.0-4.5 cm). 19 location of osteotomy showed good union. There were no infection and recurrent fracture and progression of deformity. The valgus osteotomy can correct Shepherd's crook deformity, prevent recurrent fracture, and restore alignment, thus improve functioning of limb. The intramedullary nail with neck cross pinning should be the first consideration of internal fixation. Massive impaction allograft is the key technique to improve full incorporation of allograft and to prevent pathological fracture.

Ilizarov Technique for Correction of the Shepherd's Crook Deformity: A Report of Two Cases

We report 2 patients with fibrous dysplasia who underwent correction, using the Ilizarov technique, of Shepherd's crook deformities and pathological fractures of the left femurs. A 12-year-old boy underwent an opening wedge osteotomy and a 48-degree gradual correction, whereas a 43-year-old woman underwent a 34-degree acute correction without osteotomy at the fracture site. Both patients could initiate early weight bearing. Final leg function was excellent and alignment was maintained. No complications were encountered. Both patients had no difficulty sleeping and no major complaints about the Ilizarov technique. It is more important to achieve accurate alignment than resection of the lesion. The Ilizarov technique is effective for treating the Shepherd's crook deformity in patients with fibrous dysplasia.

Multiple osteotomies and intramedullary nailing with neck cross-pinning for shepherd's crook deformity in polyostotic fibrous dysplasia: 7 femurs with a minimum of 2 years follow-up

Background In polyostotic fibrous dysplasia, particularly in lesions of the proximal femur, pathological fracture and coxa vara deformity (including shepherd's crook deformity) are likely to develop and progress.Patients and methods In 7 femurs with shepherd's crook deformity (5 patients), we performed intramedullary nailing by using multiple osteotomies and two screws crossing the femoral neck.Results Restoration of the neck shaft angle of the femur was obtained from an average of 92° prior to surgery to 129° after surgery. During the minimum 2-year follow up, no loss of neck shaft angle of the femur and no refracture occurred. All patients were able to return to normal activities of daily living.Interpretation In polyostotic fibrous dysplasia, multiple osteotomies and intramedullary nailing with neck cross-pinning can be used to correct developed or progressing shepherd's crook deformity, and to prevent recurrence and refracture.

Shepherd's Crook Deformity of Polyostotic Fibrous Dysplasia Treated with Corrective Osteotomy and Dynamic Hip Screw

Fibrous dysplasia, a condition in which the skeleton fails to develop normally, is characterized by fibroblastic stroma and immature bone. Bowing of the long bones occurs frequently in the polyostotic form, and stress fractures often result. Shepherd's crook deformity is a characteristic feature of fibrous dysplasia. The goal of its treatment is to obtain normal walking ability and relieve pain due to pathologic fracture secondary to the deformity; however, correction of the deformity is a surgical challenge. We present 2 cases of shepherd's crook deformity treated with corrective osteotomy and a dynamic hip screw. Both cases showed good bone healing and no recurrent deformity. The gross deformities were corrected, and both patients were pain-free after operation.

A Two- stage surgery for severe femoral neck deformity due to fibrous dysplasia: A case report

Various kinds of surgical treatments have been reported for varus deformity of the proximal femur due to fibrous dysplasia. We report a case of two-stage corrective osteotomy for severe varus-retroversion deformity of the femoral neck due to monostotic fibrous dysplasia. The patient was an 18 year-old man. On initial examination, the spina malleollar distance was 88 cm on the right side and 83 cm on the left.Plain radiography showed prominent varus deformity of his left proximal femur. The morphology was 130 degrees on the right side and 85 degrees on the left. Computed tomography revealed 60 degree retroversion of the femoral neck. A two-stage surgery was performed, consisting of curettage and bone grafting followed by corrective osteotomy 16 months later. A 55 degree valgus osteotomy was performed in the subtrochanteric region. After osteotomy and 40 degree internal rotation of the shaft, a 130 degree angle plate was used for osteosynthesis.Postoperative radiological examination showed a morphology of 140 degrees and computed tomography revealed a 20 degree retroversion of the femoral neck. No recurrence or varus deformity was seen at four years after surgery. Although the leg length discrepancy was 2.5 cm, the patient had no difficulty in one foot standing and no restriction of ADL (activity of daily living).The well-known progressive varus Shepherd's crook deformity in the polyostotic form of fibrous dysphasia is associated with limb shortening, limping, and occasionally chronic fatigue fractures with disabling pain (1). Various kinds of surgical treatments have been reported for this type of varus deformity (1–5). Curettage and bone grafting is one of the most common and simple treatments. However, this method often gives bad results as the grafted bones are absorbed and that the progress of varus deformity van not be prevented (6). We report a case of two-stage corrective osteotomy for severe varus-retroversion deformity of the femoral neck due to monostotic fibrous dysplasia.

Fibrous dysplasia of the proximal part of the femur. Long-term results of curettage and bone-grafting and mechanical realignment.

We reviewed the long-term outcomes of treatment of fibrous dysplasia of the proximal part of the femur in twenty-two patients (twenty-seven femora). There were fifteen male patients and seven female patients. Patients who had monostotic disease had no involvement of the calcar femorale, fewer microfractures, less deformity, and stronger bone that could support internal fixation. Patients who had polyostotic disease had frequent involvement of the calcar femorale; more microfractures; severe deformity, including shepherd's crook deformity; and, in many instances, bone that could not support internal fixation. Twenty-two of the twenty-seven femora had a microfracture at the time of the initial presentation. At least one osteotomy was performed in four femora that had monostotic disease and in nine femora that had polyostotic disease. Curettage and cancellous or cortical bone-grafting did not appear to have any advantage compared with osteotomy alone in the treatment of symptomatic lesions, as all grafts resorbed with persistence of the lesion. At the time of the latest follow-up evaluation, no lesion had been eradicated or had decreased in size. A satisfactory clinical result was achieved in twenty patients (twenty-four femora): nine who had monostotic disease and eleven who had polyostotic disease. Two patients who had polyostotic disease and an endocrinopathy (one of whom had bilateral involvement) had an unsatisfactory result. All three femora in these two patients had a neck-shaft angle of less than 90 degrees at the time of the most recent follow-up evaluation. Varus deformity of the proximal part of the femur is best treated with valgus osteotomy and internal fixation early in the course of the disease. If the calcar of the femoral neck is involved or if the quality of the bone is such that internal fixation is not possible, a medial displacement valgus osteotomy can provide a more mechanically favorable position for healing of the microfracture.

Shepherd??s Crook Deformity With an Intracapsular Femoral Neck Fracture in Fibrous Dysplasia

A shepherd's crook deformity with an intracapsular femoral neck fracture in the fibrous dysplasia of a 19-year-old woman is reported. The deformity and fracture were treated by bone grafting using a single fibula through the femoral neck to its head, and corrective valgus osteotomy. Blood flow measurement using electrochemically generated hydrogen clearance was useful in evaluating avascular necrosis of the femoral head affected by tumorous tissue. Osteosynthesis combined with a fibular graft, and corrective valgus osteotomy after proper evaluation of the blood flow in the femoral head, is a reliable procedure for the treatment of shepherd's crook deformity associated with an intracapsular femoral neck fracture.

Fibrous dysplasia associated with Shepherd's Crook deformity of the humerus.

* Professor and Chairman, Division of Orthopedic Surgery, Martland Hospital, 65 Bergen Street, Newark, N. J. 07107 **Assistant Professor, College of Medicine and Dentistry