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Dr N K Sinha & Dr Rajaram Pai [Manipal campus], Melaka-Manipal Medical College Malaysia.

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Presentation on theme: "Dr N K Sinha & Dr Rajaram Pai [Manipal campus], Melaka-Manipal Medical College Malaysia."— Presentation transcript:

1 Dr N K Sinha & Dr Rajaram Pai [Manipal campus], Melaka-Manipal Medical College Malaysia

2  A 25 year old lady sustained a trivial fall at home  Unable to walk after the fall  There was acute pain,swelling, deformity and abnormal mobility in upper part of her right thigh

3 When asked further, the patient gave a history of shorter right lower limb and restriction of right hip abduction since long

4 Xray AP pelvis with both hips : Pathological fracture right hip with coxa vara

5  The femur was bent laterally  Cortex was thinned and expanded  Neck shaft angle was reduced  Proximal femur was having a ground- glass appearance Traction AP view both hips showed

6 Fracture sideNormal side

7 Skeletal survey of other bones did not reveal affection of other bones Blood investigations were within normal limits Workup

8 Monostotic fibrous dysplasia with pathological subtrochantric fracture right femur

9  Laterally based wedge was resected from distal fragment  Medullary canal was reamed  Internal fixation was done with 135º dynamic hip screw  Concellous bone graft was harvested from contralateral iliac crest - packed at fracture site and medullary canal to promote union

10 1 2 3 Operative steps 1.Lateral based bone wedge excision 2.Sent for biopsy 3.Wedge closure by abduction

11 Post op day 1 Bone graft 135º Post operative xray

12 14 week post-op

13 23 weeks post op : Union & leg length restoration

14  Fibrous dysplasia is a developmental disorder  There is replacement of bony structure by relatively avascular fibrous tissue within which thin trabacular bone is scattered

15 Skeletal affection  70 % monostotic  20% polyostotic  2-3% have associated endocrinopathy that manifests as pricocious sexual development, cafe- au-lait spots (Albright’s syndrome)

16  Monostotic fibrous dysplasia affects rib, femur, tibia gnathic bone, calvarium, humerus  Polyostotic fibrous dysplasia affects skull, facial bones, pelvis, spine and shoulder girdle  Polyostotic fibrous dysplasia maybe unilateral/ bilateral  Does not affect the epiphysis  Diaphysis and metaphysis are affected

17 Gross pathology  Affected bone is irregular and bent  Cortex is thin and expanded  The fibrous tissue appears reddish gray/gray  Feels like a fine sand paper  Pathological fracture might occur but does not displace because of fibrous tissue.  The femur may have outward bowing and varus deformity at neck that produces shephard’s crook deformity

18 Microscopy  Microscopic areas of woven bone scattered in cellualar fibrous tissue  Giants cells may be seen at the areas of hemosiderin deposits  Cartilage is seen at the areas of cystic degeneration

19  Small single lesion may be asymptomatic  Disease starts in childhood (before 10 years) and may progress till puberty  Incidentally discovered on x-ray  Bending deformity may develop in weight bearing bone followed by pathological fracture  Affection of skull may cause asymmetry, cranial nerve involvement

20  Blood investigations are normal in most of the cases  X-ray shows a radiolucent ‘cystic’ area in the metaphysis or shaft giving a hazy or ground glass appearance  Sometimes there are cystic multilocular lesions causing scalloped endosteal erosion and osseous expansion Investigation

21  It can progress rapidly or slowly causing deformity, pain or fractures  Malignant changes are 5-10 % in polyostotic lesion  Majority of pathological fractures unite with treatment Natural history

22  It has a strong tendency to recur  Large cystic lesion may bleed profusely during surgery Points to consider during surgery

23 Apley’s System of Orthopaedics and Fractures,8 th edition,Oxford university press inc.,New York

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