Abstract

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Fibro cartilagenous dysplasia is a variant of fibrous dysplasia in which extensive cartilage differntiation is seen.Fibrous dysplasia is an anomaly in skeletal development accounting approximately 5 to 7 percent of all benign bone tumors,resulting in defective bone forming mesenchyma leading to defective osteoblastic differentiation and maturation.It exists in two forms ,monoostotic and poly ostotic fibrous dyspalsias whereas fibrocartilagenous dysplasia occurs commonly in polyostotic form.It commonly occurs in proximal femur at metaphyseal areas i.e intertrochentric regions ,sub trochantric region and neck.Due to repeated micro fractures at these sites various deformities like shepherd crook deformity can occur.It usually doesnot involve the epiphyseal region of head of femur.we are presenting a case of fibrocartilagenous dysplasia of bilateral proximal femora involving the neck and extending upto the articular surface of head of femur ,which is an unusual site and bilateral total hip arthroplasty was performed in this case.

Introduction

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INTRODUCTION: we are presenting a case report of polyostitic fibrocartilagenous dysplasia involving left humerus and bilateral femora involving the neck and articular marging of both femoral heads which is an unusual site for fibrocartilagenous dysplasia,so the management of this case is different from the usual cases where the head is not involved and we only need to take care of neck and intertrochanteric regions by doing curatagge ,bone grafting with or without prophylactic internal fixations.In some cases where the neck is highly deformed and various osteotomies may play some role.So we had to perform bilateral total hip arthroplaties in this case indicated to improve pain and function .To our knowledge this unusual presentation has not been reported in literature.There are also very few published reports regading the arthroplasties in fibrous dysplasias of proximal femur and the indications of performing arthroplasties were also different

Case Report

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A 20 years old female patient came to us with history of progressive, continuous, dull aching pain in bilateral hip joints for last five years in right limb and for four years in left limb.The pain used to become severe after walking few steps and used to aggrevate on prolonged standing or walking few steps.The relieving factors were taking medications and rest.The pain was associated with limp.The limp started two to three years after the start of pain in right hip.The patient was able to walk upto approximately fifty metres beyond which pain becomes so severe to restrict her from walking further.There was also history of pain in left shoulder for last two years.There was no history of precoucious puberty or any menstrual dysfunction or any significant trauma. On examination there was global decrease in the range of motion in bilateral hip joints with limb length inequality of 3 cms. Plain radiograph of right hip showed typical shephard crook deformity with typical ground glass appearance involving neck and head of femur.The joint space was obliterated with loss of sphericity of head of right femur and there was acetabular dysplasia because of uncontainment of head of femur.Radiograph of left hip joint also showing ground glass appearance with loss of sphericity of head with decrease joint space .The head of left humerus also showed ground glass appearance.The radiograph of right knee showing genu valgum deformity of around 25 degrees which may be because of coxa vara of femur. NCCT bilateral hip joint was done suggested either fibrous dysplasia or metabolic bone disease.Tc 99 MDP scan was done revealing hot margins at femoral and left humoral head with central cold spots.MRI bilateral hip joint suggested gross deformity and expansion b/l femora with associated hypo-intense region suggestive of fibrous dysplasia. Core biopsy was taken from bilateral neck and head regions which was also suggestive of fibrous dysplasia. All routine blood investigations were done .Addisional investigations were done to rule out mc cune Albright syndrome like thyroid and parathyroid profile all serum hormone levels urine for free cortisole,ct and mri head to rule out pituitary adenoma . So the diagonosis of bilateral fibrous dysplasia was made after the report of excisional biopsy revealing bony trabeculae without osteoblastic rimming with interspersed fibrous stroma ,numerous mature hyaline cartilage nest area which is compatible with fibro cartilaginous dysplasia. The patient was having pain,limp and limb length inequality of 3 cms so uncemented bilateral hip arthroplasty was performed,first in right hip followed by left after an interval of 6 months. Three weeks post operatively patient started walking with the aid if stick .There was minimal pain withgood range of motion and with no limb length inequality.one year follow up has been done and the patient is is wlking witout support and has no pain or limp.For fibrous dysplasia of right shoulder,we have kept the patient in routine follow up and she may require some surgical intervention like curratage with bone grafting later on.

Discussion

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DISCUSSION: The common site of involvement at proximal femora in case of fibrocartilagenous dysplasia is intertrochantric region,subtronchantric region and neck of femur.The involvement of head of femur is very unusual that’s why total hip arthroplasty is very rarely done in a case of fibrous dysplasia of proximal femur.The mainstay of treatment is curratage ,bone grafting ,internal fixations and osteotomies in most of the cases.In our case the head of femur was involved upto articular margins ,the patient was having pain ,decrease in range of motion and limp and the patient was young so we planned to perform total hip arthroplasty which was indicated to improve pain and function. Total hip artroplasty in fibrocartilagenous dysplasia is very challenging and various complications have been reported in literature.In case of fibrous dysplasia of proximal femur cemented total arthroplasty have preferred over non cemented total hip arthroplasty in literature because of high rate of stem loosening,but in our case the patient was only twenty years old that’s why we perfomed non cemented total hip arthroplasty .the main difficulity we encountered in this case was the huge size of head and dysplastic acetabulum.peroperatively the capsule was very large so we had to remove the capsule,we created the acetabulum by reaming and fixed with screws to increase the stability of the acetabulum.we checked the limb length and stability preoperatively,which seemed to be good. It is very difficult to say that after how many years, the revision surgery will be reqired but at present the patient is pain free with good functions. Thus we concluded that the fibrocartilagenous dysplasia of proximal femur can involve any part from diaphysis to head of femur and if the head of femur is involved especially at weight bearing area,the functional disability and pain are inevitable .Thus the total hip arthroplasty is an excellent option to give the patient a better functional outcome and pain free hip joints.

References

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Source(s) of Funding

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funding was done by esic hospital,basaidarapur ,new delhi,india.

Competing Interests

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none