Crook Deformity Research Articles

Monostotic Fibrous Dysplasia of the Hip Treated with Autologus Non-Vascularized Fibular GraftMonostotic Fibrous Dysplasia of the Hip Treated with Autologus Non-Vascularized Fibular Graft

Fibrous dysplasia represents a dysplastic disorder of bone characterized by solitary or multifocal polyostotic intramedullary lesions composed of proliferations of fibroblast-like spindle cells with a characteristic whorled pattern in which trabeculae of immature woven bone. A 20 years old female with pain and limping on his left hip has been suffered for about 1 month. The pain was localized on the left hip with dull characteristics, and worsen when she moves and walks. The pain intensity also decreased with rest. Then she came to hospital and performed X-ray with shepherd crook deformity on her left hip and performed ipsilateral non-vascularized fibular strut graft on her left hip. Right after post-operative wound has been healed the chief complain was marked decrease. For about 2 years after the surgery follow up by X-ray and also MRI examination with contrast the condition of the fibular strut graft was viable, the complaint was gone and the progression of the disease has been stopped.

Surgical correction of valgus deformities of the knee in Polyostotic Fibrous Dysplasia.

Coxa vara and shepherd's crook deformity represent the most common femoral deformities in patients affected by polyostotic fibrous dysplasia (PFD) and McCune Albright syndrome (MAS). The tibia is also commonly affected, with antero-medial bowing and valgus deformity. Surgical treatment of these deformities are technically demanding and, in most cases, intramedullary nails are the preferred method to stabilize corrective osteotomies. Genu valgus may also be present, as a residual defect after the surgical correction of the aforementioned deformities. The aim of our study was to report the outcomes obtained in five patients with PFD or MAS operated on for complex deformities of the lower limbs with an associated residual genu valgum greater than 15°. It was surgically treated by distal femoral or proximal tibial osteotomy stabilized with a screw plate. The osteotomy was performed distal to the intramedullary nail in femurs, while it was performed after removing the intramedullary nail in the proximal metaphysis of the tibia. In latter cases, the intramedullary device was reinserted at least 6 months after surgery. At follow-up, the femoro-tibial angle was corrected in all cases and the patients were satisfied with the final result. Two patients showed mild limping, unrelated to the alignment of the knee. In our opinion, residual valgus deformities of the lower limb greater than 15 degrees should be corrected to improve function and cosmetic appearance of these patients. The osteotomy of the distal femur or of the proximal tibia stabilized by a screw-plate represent the treatment of choice for the correction of these deformities.

Shepherd's crook deformity: Do you recognize this sign?

Shepherd's crook deformity: Do you recognize this sign?

Pathological fracture in fibrous dysplasia: a case report

Fibrous dysplasia (FD) is described as a growth disorder characterized by the progressive replacement of normal bone elements by fibrous-osseus tissue. Bones affected by FD is presented with bone weakening and prone to pathological fracture. We reported a case of FD in an 8-years-old boy, who came to the hospital with pain in the upper right thigh after falling with bumps in his head and foot. An X-ray revealed a deformity as a diasteal scalloping with radiolucency lesions on one-third proximal of the right upper femur, greater trochanter, and lesser trochanter, with complete transverse discontinuity in the distal deformity. No similar lesion was found in the other bones. Bone structure and trabeculation in the deformity area has decreased and the cortex was thinning. On magnetic resonance imaging (MRI), bone size was enlarged, hypointense on T1WI and inhomogeneous hyperintense on T2WI. Fibrous dysplasia with a pathological fracture is a rare case. The appearance on the X-ray was diasteal scalloping with a ground-glass radiolucency represented with Shepherd's crook deformity. This lesion was accompanied by a pathological fracture (fragility fracture) on distal lesion. Fibrous dysplasia which characterized by bone developmental anomaly gave an inhomogeneous hypointense on T1W1 and hyperintense on T2WI.

Surgical treatment of femoral deformities in polyostotic fibrous dysplasia and McCune-Albright syndrome: A literature review.

BACKGROUNDSurgical correction of femoral deformities in polyostotic fibrous dysplasia (PFD) or McCune-Albright syndrome (MAS), such as coxa vara or shepherd’s crook deformity, is a challenge. AIMTo evaluate the treatment of patients with femoral deformities caused by PDF or MAS treated by osteotomies and stabilized with different methods, by analyzing the most relevant studies on the topic. METHODSA literature search was performed in Medline database (PubMed). Articles were screened for patients affected by PFD or MAS surgically managed by osteotomies and stabilized with different methods. RESULTSThe initial search produced 184 studies, with 15 fulfilling the eligibility criteria of our study. Selected articles (1987-2019) included 111 patients overall (136 femurs). CONCLUSIONBased on our results, the preferred method to stabilize corrective osteotomies is intramedullary nailing with neck cross pinning. When the deformity is limited to the proximal part of the femur, a screw or blade plate may be used, although there is a high risk of fracture below the plate. When the femur is entirely involved, a two-stage procedure may be considered.

Management of Shepherd’s Crook Deformity by Proximal Femoral Osteotomy without Bone Graft - A Case Report

Management of Shepherd’s Crook Deformity by Proximal Femoral Osteotomy without Bone Graft - A Case Report

Shepherd' Crook right coronary artery in an octagenerian

Right coronary artery after origin from right coronary sinus traverses horizontally to run in right atrioventricular groove till the crux of the heart where it divides into posterior descending artery and posterior left ventricular branch. When the right coronary artery after its origin takes an acute high take off from the ostium and then abruptly descends down making a hairpin loop to run in the right atrioventricular groove, it is known as Shepherd's crook right coronary artery which is extremely rare to encounter in routine clinical practice. This interesting coronary artery deformity has great significance per se so far as the coronary physiology and intervention is concerned. We present a case of Shepherd's crook right coronary artery in the most elderly patient of 84 years of age in world literature and detailed review of this peculiar anatomy. Review literature about Shepherds Crook deformity is yet not there in the literature except some case reports and this review article will be providing the interventional cardiologists a detailed insight into the right coronary ischemia secondary to this interesting anomaly.

Shepherd’s Crook Deformity with Pathological Femur Neck Fracture in a Patient with Fibrous Dysplasia Managed with Proximal Femoral Nailing and Valgus Osteotomy

Fibrous dysplasia is a rare condition in which bone is replaced by fibroosseous tissue. It is a benign condition, divided into monostotic and polyostotic fibrous dysplasia, which accounts for about 5-7% of all benign bone tumours. Here, authors reported a case of a 38-year-old male that presented with left hip pain since 15 days and had a history of trauma. X-ray pelvis and Magnetic Resonance Imaging (MRI) showed shepherd’s crook deformity in the left proximal femur with pathological femur neck fracture. It was managed with valgus osteotomy for deformity correction and fracture fixation using long proximal femur nail and cannulated cancellous screws. At one and half year follow-up, the patient showed good hip and knee range of motion with no complications.

Increasing serum alkaline phosphatase is associated with bone deformity progression for patients with polyostotic fibrous dysplasia

BackgroundFibrous dysplasia (FD) is a rare bone disorder in which normal intramedullary bone is replaced by fibro-osseous tissue, which is complicated by the progression of Shepherd’s crook deformity. How to predict the progression of Shepherd’s crook deformity is still a challenging for the orthopedic surgeon.MethodsA total of 159 cases were reviewed in the retrospective study between January 2000 and September 2016. Clinical and monitoring data were collected. We analyzed the correlationship between the bone turnover markers and other parameters (age, gender, FD type, deformity, BMI, and lesion location).ResultsAge, gender, lesion location, lesion type, and shepherd’s crook deformity had a close relationship with preoperative ALP level in the univariate analysis, and the multivariate analysis showed age, gender, lesion type, and shepherd’s crook deformity had the significant relationship with the preoperative serum ALP level. The surgery could remove the bone lesion and suppressed the abnormal bone metabolism. Furthermore, the preoperative ALP level of FD patients with the shepherd’s crook deformity was obviously higher than that without deformity, and the preoperative calcium and phosphorus levels of FD patients with deformity were significantly lower than that without deformity. Notably, for some patients with progression of the shepherd’s crook deformity during the follow-up, ALP increased to the high level and at that time X-ray showed the shepherd’s crook deformity severely progressing.ConclusionsPFD with higher serum ALP level has obvious tendency to progress severely, and risk factors of progression to the deformity are the condition of bony metabolism and FD type. The deformity of PFD may be related to high speed of bone turnover, which is exactly reflected by the levels of serum ALP and calcium. Evaluation of patients with FD should include a thorough evaluation of calcium/phosphate metabolism and bone turnover.

Lower limb lengthening and deformity correction in polyostotic fibrous dysplasia using external fixation and flexible intramedullary nailing

The study describes preliminary experience of the use of external fixators for limb lengthening and deformity correction in combination with flexible intramedullary nailing in management of polyostotic fibrous dysplasia. Patients and methodsThe retrospective study included 8 patients (mean age 11.6 ± 3.38 years; range 7–17 years) with polyostotic fibrous dysplasia operated on using external circular frame and flexible intramedullary nailing. Mean follow-up was 2.6 years. Surgical technique consisted of percutaneous osteotomy of a segment and application of circular external frame. The intramedullary nailing was done using two bent nails. Hydroxyapatite-coated nails were applied in three patients; five patients had titanium nails. Amount of lengthening (cm and %), amount of deformity correction, duration of external fixator use, index of external fixation, “nail/medullary canal at narrowest site” ratio, “nail-medullary canal at osteotomy site” ratio were analyzed. Results and complications were assessed according to Lascombes's classification. ResultsThe mean amount of lengthening was 4.5 cm (or 13.7 ± 6.0% per segment). This gave a mean external fixation index of 32.5 ± 13.97 days/cm. The mean ratio of IM nail diameter/medullary canal diameter at the narrowest site was 0.22 ± 0.07 (range, 0.125–0.3 mm). No migration of IM nails into medullary canal were noticed. But in one case there was external migration of Ti-nail. In a year after frame removal, the results of treatment were classified as grade I in 7 cases and IIb in one case.At the latest follow-up control, mechanical axis deviation was found within normal limits in six patients. Two patients had excessive MAD of 11 and 28 mm. In the first case a partial varus deformity recurrence occurred at middle shaft site where a large dysplastic zone was presented. In the second case, a specific shepherd's crook deformity developed and caused excessive MAD. Mean lower limb length discrepancy varied from 1 to 15 mm. ConclusionThere are advantages of using elastic intramedullary nailing and external fixation in the treatment of limb length discrepancy and deformity of long bones in patients with PFD. This strategy ensures reduced external fixation time and high accuracy of alignment. Intramedullary nails left in situ, especially nails with HA-coating, seem to prevent deformity recurrence and stimulate remodeling in dysplastic fibrous zones.

A Novel Technique for Single-stage Correction of Severe Shepherd Crook Deformity of a Femur With the Large Cystic Lesion and Pathologic Fracture

Fibrous dysplasia with severe proximal femur deformity and multiple large cystic lesions associated with a pathologic fracture is an orthopedic challenge. Correction is often impossible with conventional fixation methods. We report a technique to address the deformity and fracture simultaneously in these scenarios. A 32-year-old man, a case of polyostotic fibrous dysplasia with severe shepherd crook deformity and the pathologic fracture was managed by curettage, multilevel osteotomy, tibial strut allograft, extramedullary fixed-angle implant, and augmentation with cortico-cancellous bone grafting in a single stage. The mechanical axis of the femur was corrected, with an increase in the neck-shaft angle from 70.6 to 121 degrees. Union was achieved with good outcomes in cosmetic and functional status without recurrence at 18 months follow-up. In proximal femur fibrous dysplasia, critical analysis of the bone stock for implant purchase especially in the proximal femur with large cystic lesions is essential. Augmentation of the head, neck, and calcar region with cortical strut allograft provide stable fixation and decreases the deformity recurrence. This technique is particularly useful in severe forms of the disease; wherein there would be an impaired implant purchase in the weak pathologic bone, deficient lateral wall, and a wide medullary canal.

Management of Fibrous Dysplasia of Proximal Femur by Internal Fixation Without Grafting: A Retrospective Study of 19 Patients.

Introduction:This paper assesses whether treating patients with fibrous dysplasia of proximal femur by internal fixation with correction of the deformity, if present, without grafting is enough or not.Methods:This study is a retrospective study using patient's medical records, including analysis of 19 patients with fibrous dysplasia of proximal femur treated by internal fixation only and followed up between 2000 and 2017 for at least 2 years. Epidemiologic data, clinical manifestations, radiologic and histologic investigations, surgery, functional outcomes, and complications in these patients were analyzed.Results:The study included 12 male patients and 7 female patients, with a mean age of 20.74 years. Of these, two patients presented with pathologic fractures, two presented with shepherd's crook deformity, and the remaining presented with hip pain and limping. Implants used for internal fixation were intramedullary nail in four patients, dynamic hip screw in eight patients, cannulated screws in four patients, broad dynamic compression plate in two patients, and narrow dynamic compression plate in one patient. The mean follow-up period was 53.58 months. Four patients had postoperative complications. The mean Musculoskeletal Tumor Society score was 27.63 points.Discussion:The optimal management option for fibrous dysplasia of proximal femur is debatable. We suggest that internal fixation without grafting has a good local control and satisfactory functional long-term outcome.

Surgical treatment for shepherd's crook deformity in fibrous dysplasia: THERE IS NO BEST, ONLY BETTER.

The optimal strategy for shepherd's crook deformity correction remains technically challenging. In particular, it is difficult to performan accurate osteotomy based on the pre-operative correction plan. Moreover, the choice of ideal hardware remains unclear. In addition, when combined with the deformity of knee joint, the sequence of deformity correction is another overlooked factor when making a correction strategy. From February 2012 to March 2014, we retrospectively examined a cases series in our department involving the creation of three-dimensional (3D) printing osteotomy templates and inner fixation for shepherd's crook deformity in fibrous dysplasia. A total of ten patients of shepherd's crook deformity were enrolled in this study. The neck shaft angle was corrected from a mean value of 88.1° (range, 73-105°) pre-operatively to a mean value of 128.5° (range, 120-135°) post-operatively; no marked loss in the value was observed (mean, 123.7°; range, 115-130°) at the final follow-up. In addition, compared with patients using dynamic hip screw (DHS), longer operation time and additional blood loss were recorded in patients using intramedullary nail (IN). Moreover, after correction of shepherd's crook deformity, two patients were observed more predominant on their pre-existing valgus knee deformity. 3D printing osteotomy templates facilitate the correction of shepherd's crook deformity. Dynamic hip screw (DHS), combined with polymethylmethacrylate (PMMA) augmentation, yields excellent outcomes and ensures easy placement and non-intramedullary manipulation, lower bleeding volume, and reduced operation time. Prior to the correction of shepherd's crook deformity, the mechanical axis of the lower limb should be carefully examined, and any evidence of valgus knee deformity should be addressed in advance.

A Modified Technique of Fixation for Proximal Femoral Valgus Osteotomy in Abnormal Bone: A Report of Two Cases.

The ideal size of intramedullary device to fix corrective osteotomy of proximal femur in abnormal bone in children and small patients may not be easily available. We report the successful use of Rush rod in combination with multiple Kirschner wires to fix the corrective osteotomy of coxa vara and shepherd crook deformity in two patients with osteogenesis imperfecta and fibrous dysplasia. The union was achieved on time, neck shaft angle and rotation were maintained.

The Successful Treatment of Shepherd’s Crook Deformity in Fibrous Dysplasia by Ilizarov Technique

The Successful Treatment of Shepherd’s Crook Deformity in Fibrous Dysplasia by Ilizarov Technique

Treatment of shepherd's crook deformity in patients with polyostotic fibrous dysplasia using a new type of custom made retrograde intramedullary nail: a technical note.

AimsThe severe form of coxa vara, the ‘shepherd’s crook deformity’, is always a consequence of a locally extensive form of polyostotic fibrous dysplasia (or McCune-Albright syndrome). Treatment of this deformity is a challenge. The soft bone does not tolerate any implant that depends on the stability of the cortical bone (like plates or external fixators). Intramedullary nails are the most appropriate implants for stabilisation, but if they are inserted from the greater trochanter, they cannot correct the varus deformity enough.Patients and MethodsWe have developed a special intramedullary nail that can be inserted from the osteotomy site and can be driven retrograde into the femoral neck in an appropriate valgus position. We have operated 15 legs in 13 patients. The average age at surgery was 14 years and 5 months (6 to 28.9). In all, 11 femora had been operated before (unsuccessfully) with various implants.ResultsThe average follow-up was 54.2 months (7 to 132). The average correction of the neck/(distal) shaft angle was 57.5° (10° to 80°) ( = 72.8%). While pre-operatively none of the patients was able to walk without aid, at follow-up only one patient was unable to walk, three used the aid of crutches because of tibial lesions and one patient had an increased external rotation of the leg. At follow-up, most patients were free of pain. One implant broke and had to be replaced.ConclusionThis new operative method offers the possibility of efficient correction and stabilisation of this severe and difficult deformation.

Changing Pattern of Femoral Deformity During Growth in Polyostotic Fibrous Dysplasia of the Bone: An Analysis of 46 Cases.

In polyostotic fibrous dysplasia (PFD) affecting the femur, 6 types of stable femoral deformities have been identified, ranging from the mild type 1 to the most severe type 6. The purpose of our study was to identify the type of deformity present at diagnosis in children with PFD affecting the femur, and to investigate possible changes in the original type with growth. Twenty-seven children affected by either PFD or McCune-Albright syndrome, with 46 affected femurs, were consecutively observed from 1994 to 2010. Radiographs of the pelvis and femurs were taken at diagnosis, and afterward at regular intervals of 6 to 12 months depending on the clinical evolution of each case. Radiographs of the femurs taken at 3 and 7 years after diagnosis were selected to assess the evolution of the femoral deformity. Deformities were classified according to the femoral shape, neck-shaft angle value, and the presence of shepherd's crook deformity evaluated on AP radiographs. The average age of the patients at diagnosis was 5 years (range, 2 to 11 y). At diagnosis, 30 femurs were type 1; 12, type 2; 1, type 3; 1, type 4; and 2, type 5. Three years later, 39% of the femurs had maintained the original deformity present at diagnosis, whereas only 13% did so 7 years after diagnosis.Predictors of change to a more severe type were: younger age at diagnosis, McCune-Albright syndrome, rapid spreading of fibrodysplastic tissue from the trochanteric area to the proximal femoral shaft, cortical thinning of the proximal femur, calcar osteolysis, and presence of an intertrochanteric cystic-like lesion eroding the calcar. In children with PFD affecting the femur, 61% of the femoral deformity present at diagnosis switched to a more severe type of deformity within 3 years from diagnosis, whereas 87% either shifted to a more severe type of deformity or already had corrective surgery within 7 years after diagnosis. Level IV.

CORR Insights ® : What Is the Role of Allogeneic Cortical Strut Grafts in the Treatment of Fibrous Dysplasia of the Proximal Femur?

B one deformities in patients with fibrous dysplasia can be potentially disabling and difficult to treat. When involving the proximal femur, fibrous dysplasia is often progressive; resulting in pain, varus angulation of the hip (shepherd’s crook deformity) and potentially, pathological fractures [6, 7]. The disease process is caused by a postzygotic (somatic) mutation of the GNAS gene, manifesting in poorly functioning osteocytes, which produce an inferior quality fibro-osseous bone [9]. The extent of the disease, as well as its extraskeletal manifestations (such as McCune–Albright Syndrome), depends upon the eventual location of the migrating, abnormal embryonic cells [2, 10]. Traditional treatment with curettage and grafting has been associated with resorption of the graft and replacement with newly formed, dysplastic bone produced by the persistent population of abnormal osteoblasts [7]. Enneking and colleagues [3] have suggested that cortical allografts may provide superior outcomes when treating lesions of the femoral neck. The suggestion was that incorporation (or replacement) of the cortical bone would be limited in comparison to cancellous grafting thus maintaining structural support the femoral neck. Evaluation of retrieved bulk allografts, previously implanted for tumor reconstructions confirmed (at least adjacent to normal bone) that allografts demonstrated delayed and incomplete incorporation [4]. Such findings suggest that the premise might also be valid in fibrous dysplasia. Shortly after the Majoor and colleagues paper was accepted for publication, a similar article was published—also from a national referral center (NIH)—maintaining a longitudinal database for patients with fibrous dysplasia [5]. Leet and colleagues reviewed surgical management of long bone lesions in their patients, comparing various grafting techniques including cortical allografts. They identified no differences in outcome comparing patients treated with the cortical grafts versus other techniques, concluding that both allografting and autograft are of ‘‘limited value in ablating lesions of fibrous dysplasia’’ [5]. This CORR Insights is a commentary on the article ‘‘What Is the Role of Allogeneic Cortical Strut Grafts in the Treatment of Fibrous Dysplasia of the Proximal Femur?’’ by Majoor and colleagues available at: DOI: 10.1007/s11999-016-4806-3. The author certifies that he, or a member of his immediate family, has no funding or commercial associations (eg, consultancies, stock ownership, equity interest, patent/ licensing arrangements, etc.) that might pose a conflict of interest in connection with the submitted article. All ICMJE Conflict of Interest Forms for authors and Clinical Orthopaedics and Related Research editors and board members are on file with the publication and can be viewed on request. The opinions expressed are those of the writers, and do not reflect the opinion or policy of CORR or The Association of Bone and Joint Surgeons. This CORR Insights comment refers to the article available at DOI: 10.1007/s11999-0164806-3.

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

For complex femoral deformities in patients affected by polyostotic fibrous dysplasia and coxa vara associated with shepherd's crook deformity of the femoral shaft, the correct treatment is stabilization with an interlocking cervicodiaphyseal nail after correction of all of the femoral deformities. Evaluate the patient radiographically and select the hardware. Position the patient on the traction table. Expose the lateral aspect of the proximal part of the femur and mark the position of both the blade-plate and the osteotomy. Prepare the slot for the blade, perform the osteotomy, and correct the valgus alignment of the proximal part of the femur. Check postoperative bleeding, provide blood transfusions if needed, and have the patient resume walking. Study the patient radiographically, simulate the osteotomies, perform another assessment of lower-limb length, and select the hardware. Position the patient on the traction table and expose the lateral aspect of the femur from the tip of the greater trochanter to the level of the most distal planned osteotomy. All of the osteotomies are performed, and a medullary canal is drilled in each femoral fragment. Introduce the nail into the osteotomy fragments and insert the spiral blade and distal locking screws. Check postoperative bleeding, which is usually marked; administer blood transfusions as needed; and have the patient resume walking. We performed this surgery on 13 femora in 11 patients.

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