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Shaheed

Suhrawardy Medical College Hospital. Dhaka-1207, Bangladesh, Department of Orthopedic & Traumatology.
A case report on Treatment of Shapherd crooks deformity in

Firous dysplasia
(Assist. Prof), Dr.Subir Hussain 3 (Assist. Registrar), Dr. M d. Nazrul Islam 4 (Resident Surgeon) , Department of Orthpedic & Traumatology. Shaheed Suhrawardy M esdical College Hospital. Dhaka-1207, Bangladesh. Dr. Paritosh Chandra Debnath 1 (Associate Prof,), Dr.Kazi Shamimuzzaman 2

Summery

Fibrous dysplasia the lesion of bone consisting of irregularly arranged bone trabeculae in bland fibrous tissue. It is the cystic lesion of bone that can weaken and lead to pathological fracture. It can involve a single bone (monostatic fibrous dysplasia), but in other it may involves many bones (polyostotic fibrous dysplasia). The severity of the disorder ranges from isolated small lesion that remains asymptomatic to lesions that cause repeated pathological fracture & deformity of the bone. A young male patient about 20 years old came to us with huge cystic lesion in the proximal femur with pathological fracture, typically known as Shepherds Crook deformity. The object of this case report is to highlight the huge deformity with pathological fracture & emphasis the surgical procedure where correction of the bony deformity by osteotomy, bone strengthening and bone union by mixed bone graft has been achieved satisfactorily in a single surgical procedure.
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Introduction
Most authors believe fibrous dysplasia is a developmental anomaly of bone formation that may exist in a monostatic or polyostatic form. In fibrous dysplasis trabecular bone is replaced by cellular fibrous tissue. If the lesions are large the bone is considerably weakened and the pathological fracture with progressive deformity may occur. Small single lesions are asymptomatic. But patients with polyostatic disease present in childhood or adolescent with pain, limp, bony enlargement, deformity & pathological fracture. Fibrous dysplasia can occur in the epiphysis, metaphysic or diaphysis that affects the ribs, tibia, femur, maxilla, skull, humerus, pelvis, radius and rarely spine. Number of bone involvement in polyostatic fibrous dysplasia varies from 2-12, the most common involvement in monostatic variety is femur. It appears in childhood and may progressively enlarge. They are less active or inactive in skeletal maturity but rarely can they grow in adult. Fibrous dysplasia may be associated with precocious puberty and darkly pigmented skin lesions called Albright syndrome. X-Ray of the lesion shows radio-lucent cystic areas in the metaphysic or shaft which contains fibrous tissue with diffuse spots of immature bone, the lucent patches typically have a slightly hazy or ground glass appearance. The weight bearing bone may bend in massive osteolytic lesion and one of the classic feature is Shepherds crook deformity of proximal femur. Malignant transformation to fibrosarcoma occurs in 5-10% of patients with polyostatic lesions but only rarely in monostastic lesions.

Case report
A 20 years old boy came to us with difficulty in walking for 7 months. He could not walk without support. He had a history of mild trauma to the right thigh 7months back He noticed mild aching pain in right upper thigh with gradual shortening and bending in right thigh. His right lower limb was shortened by 5 cm. of his femoral component and huge hard swelling was seen in the right upper thigh. Abnormal mobility of the upper femur with moderate tenderness was found.

Investigations
1. X-Ray of the right upper femur shows a huge osteolytic lesion involving right upper femur, right trochanter, with loss of cortical bone and lucent patches typically hazy looks like grown glass appearance with pathological fracture at the subtrochantaric region. Blood for TC,DC,ESR,Hb%--Normal CXR-P/A-- Normal MT-- Normal RBS - Normal S. creatinine- normal Blood urea- normal S. calcium -Normal Alkaline phosphatase- Normal

2. 3. 4. 5. 6. 7. 8. 9.

Operation note
Through lateral approach upper end of the femur was exposed. Outer part of the proximal femur was so thin that it needs little effort to curette the cystic areas carefully. After curettage vulgus wedge osteotomy was done at subtrochantaric region to correct deformity, massive irradiated allograft with fibular autograft was applied to enhance healing and incorporation of the cystic bony lesion and fragments were fixed with proximal femoral nail (PFN). Wound was closed in layers by keeping a drain inside which was removed after48 hours. Stiches were removed after 10th post operative day and the healing was satisfactory. Abduction bar was applied after completion of the operation to protect the weakened bone for 2 months. After removal of the abduction bar at 2 months clinically and radiologically bone was stable and uniting satisfactorily. He was advised to use crutch for non weight bearing of his right lower limb upto 3 months.

Histopathological report
Curetted material was sent for hitopathological study and report shows fibrous dysplasia.

Pre-operative Radiological Picture


Plane X-ray Rt. Upper femur (Preoperative)

Post Operative radiograph.

Post operative period with derotation Bar Follow-up visit

Discussion
Fibrous dysplasia is a developmental disorder in which trabecular bone are replaced by cellular fibrous tissue affects the single bone (monostatic) or many bones (polystatic). Small single lesions are asymptomatic. Large monostatic lesion causes pain & may develop pathological fractures. The biopsy may require for establishing diagnosis. Some lesions may resolves spontaneously & very large deformed cystic lesions with pathological fracture require special surgical measure for correction of deformity, fixation of bone by nailing, strengthening of bone by bone grafting. A mixture of cortical & cancellous bone may provide added strength even this lesion is not fully eradicated. In this unusual deformed case we carefully planned to curette the cystic areas of bone, lateral wedging for correction of the varus deformity, proximal femoral nailing was done for maintaining reduction & stability, and erradiated cancellous bone mixed with fibular cortical bone were introduced in the lesion to enhance incorporation, strengthening & union of the bone. This is a rare & difficult case to treat. After applying this procedure in Shaheed Surhawardy Medical college Hospital, union was achieved in usual time, bone graft was nicely incorporated & maximum deformity has been corrected & shortening was also overcome following operation.

Reference
1. Apleys System of Orthopaedics and fractures.8th edition .2001 . 174175 2. Campbells Operative Orthopaedics. 10th edition. 2003. 794 3. Tureks Orthopaedics, Principle & their application. 5th edition. 1994. 299-300. 4. Adams J.C. Outline of Orthopaedic. 12th edition, Churchill Livingston. 1995. 53-54