fibrous dysplasia with pathological fracture of proximal femur in a 25 year old lady
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Fibrous dysplasia with pathological fracture of proximal femur in a 25 year old lady. Dr N K Sinha & Dr Rajaram Pai [ Manipal campus], Melaka- Manipal Medical College Malaysia. Case history & clinical examination. A 25 year old lady sustained a trivial fall at home - PowerPoint PPT PresentationTRANSCRIPT
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Fibrous dysplasia with pathological fracture of
proximal femur in a 25 year old lady
Dr N K Sinha &
Dr Rajaram Pai [Manipal campus],Melaka-Manipal Medical College
Malaysia
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Case history & clinical examination
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
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When asked further, the patient gave a history of shorter right lower limb and restriction of right hip abduction since long
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Xray AP pelvis with both hips : Pathological fracture right hip with coxa vara
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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
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Fracture side Normal side
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Skeletal survey of other bones did not reveal affection of other
bones
Blood investigations were within normal limits
Workup
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Provisional diagnosis
Monostotic fibrous dysplasia with pathological subtrochantric fracture right femur
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Operative procedure
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
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1
2
3 Operative steps1. Lateral based bone
wedge excision2. Sent for biopsy3. Wedge closure by
abduction
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Post op day 1
Bone graft
135º
Post operative xray
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14 week post-op
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23 weeks post op :Union & leg length restoration
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Discussion Fibrous dysplasia is a
developmental disorder There is replacement of bony
structure by relatively avascular fibrous tissue within which thin trabacular bone is scattered
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Skeletal affection 70 % monostotic 20% polyostotic 2-3% have associated
endocrinopathy that manifests as pricocious sexual development, cafe-au-lait spots (Albright’s syndrome)
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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
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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
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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
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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
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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
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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
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It has a strong tendency to recur
Large cystic lesion may bleed profusely during surgery
Points to consider during surgery
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Reference
Apley’s System of Orthopaedics and Fractures,8th edition,Oxford university press inc.,New York