a case of fibrous dysplasia

1. A case of FIBROUS DYSPLASIA
PRESENTER
Dr.Karna Venkateswara Reddy
2nd yr PG
MODERATOR
DR.V. Nageswara Rao DNB(ortho),FJR
Associate professor
Dept of orthopaedics
G.S.L Medical college &hospital

2. • NAME : Marisetty Annavaram
• AGE: 36 Years
• GENDER: male
• PLACE : Kanumagraharam
• OCCUPATION: Labourer
• MARITAL STATUS: Married
• DATE OF ADMISSION: 15.06.16

3. CHIEF COMPLAINTS
• C/O Pain in left groin since 1 day
• Difficulty in walking since 1 day

4. HISTORY OF PRESENT ILLNESS
 Alleged to have sustained injury due to accidental
fall from bike on 14-6-16 around 9 am
 Since then patient c/o pain in left groin which was
sudden in onset , gradually progressive in nature
and aggravated by movements & not relieved by
rest or medications.
 Unable to bear weight on left lower limb

5. • No h/o loss of consciousness
• No h/o head injury
• Bowel n bladder –normal

6. PAST HISTORY
1996 - patient had h/o fall from tree
?? Fracture proximal 1/3 left femur.
ORIF with Plates and screws
Started walking with the help of support after 1 ½mt
After 3 months started his regular work

7. 2002 - implant removal done (plates n screws)
Started walking after 1 wk of surgery
5 months later patient started c/o pain at left hip
and he continued to walk for next 1 year with help of
support ( stick)
Patient noticed deformity at left proximal thigh and
shortening of left lower limb

8. 2003 - patient consulted a private practitioner for
pain and deformity at left proximal thigh
Re-fracture at ?? sub trochanteric level (proximal
thigh)
ORIF with plates and screws
Patient started walking after 6 wks
2006- Implant removal done – started walking
after 1 wk and regular activity

9. 2010 - h/o trivial trauma
Fracture shaft femur ? Middle 1/3rd left
ORIF with plates and screws
Started walking after 6 wks with the help of walker
-Since then he is unable to do regular work
-Limited to house hold work only

10. 14- MAY – 2016
H/o fall from bike
GSL GENERAL HOSPITAL

11. • Not a known case of Diabetes / Hypertension
• No other significant medical history in past

12. • Personal history - Mixed diet
Non- smoker
Non –alcoholic
• Family history – Nothing significant

13. General examination
• Appearance: moderately built and
nourished
• Pallor : present
• Icterus : Absent
• Cyanosis: Absent
• edema : Absent
• Clubbing: Absent
• Lymph nodes: Not palpable
• Skin pigmentation: Absent

14. Vitals @ time of admission
• Pulse: 76 bts/min
• Blood pressure: 110/70 mm of Hg
• Respiratory rate: 20 /min
• Temperature: normal

15. LOCAL EXAMINATION: (lt UPPER THIGH)
• INSPECTION:
• An ill defined deformity
occupying at the superio-lateral
aspect of the upper left thigh
with convexity antero-laterally.
• Multiple healed surgical scars
present over deformed area
which was healed by secondary
intention

16.  Varus deformity of hip with shortening of the
lower limb.
 Wasting of the thigh, & gluteal muscles
 No engorged veins.

17. PALPATION
 There is an irregular, expanded bony deformity
with convexity antero-laterally extending from the
hip to subtrochanteric area.
 No local rise of temperature
 Tenderness present at subtrochanteric area
 Abnormal mobility and crepitus felt at U/3 rd femur
 Distal neurovascular status normal
 Regional lymph nodes not enlarged.

18. MOVEMENT
• Left Hip (ROM)– painful.
• Knee & ankle –ROM – normal

19. Measurements
• Shortening of limb – 3 cm.
(femoral component)
• Muscle wasting-
Thigh – 4 cm.
Leg – 2 cm

20. OTHER SYSTEMS
• Respiratory system examination - NAD
• Cardiovascular system examination - NAD
• P/A Abdomen - NAD
• CNS examination- No focal neurological deficit

22. Investigations
• X-Ray pelvis with both hips A/P view

23. • Shows SHEPHERD’S
CROOK DEFORMITY with
multiple osteolytic lesions
involving part of the
trochanteric & subtrochanteric
area, with thinning of cortical
bone & lucent patches typically
hazy, looks like ground-glass
appearance with pathological
fracture at the subtrochanteric
region with implant in situ.

24. Blood investigations
• Hb% - 9. 4 gm / dl
TC of WBC – 6,900 / cu mm
• DC of WBC
• N - 64% B -0%
• L - 32% M -01%
• E – 03%
• ESR - 30 mm in 1st hr
• Urine RME - Normal study
• CXR- P/A view -Normal
• RBS – 113 mg / dl

25.  S. creatinine -0.8 mg/ dl
 Blood urea -24 mg/ dl
 S. calcium -9 mgm / dl
 S. alkaline phosphates – 110 IU/ L
 S. sodium- 142meq/l
 S.potassium - 3.8 meq/l

26. • CT and MRI SCAN couldn't be done due
to presence of implant which may lead
metal artifacts

27. PROBABLE DIAGNOSIS
• PATHOLOGICAL Sub trochanteric Fracture of
left femur with osteolytic lesions at both
trochanteric and sub trochanteric region

28. DIFFERENTIAL DIAGNOSIS
• Fibrous Dysplasia
• Pagets disease
• Nonossifying fibromas
• Aneurysmal Bone Cyst

29. • In order to confirm our diagnosis we have
done open biopsy under spinal anesthesia
on 20 -6-2016
Bone bits taken from trochanteric
and sub trochanteric region
HISTO PATHOLOGY EXAMINATION

30. PATHOLOGY

31. • Name: X
• Age & Sex:36/M
• Date:20.06.16
• Biopsy No.:4802/16
• Clinical diagnosis:?Pagets disease/ Fibrous
dysplasia
• Specimen: sample taken from fracture site(dark
red in color)
• Gross features: Received multiple reddish brown
haemorrhagic tissue bits consisting of bony
particles together measuring
4.0x3.0x2.0cm(G/S)

32. Microscopic features
X 100H &E
Curvilinear trabeculae (Chinese
letters) of metaplastic woven bone
devoid of osteoblastic rimming
Normal osteoblastic rimming

33. X100 H&E
Sheets of benign spindle cells Foamy macrophages
Microscopic features
X 400 H&E

34. • OPINION –
Features consistent with FIBROUS DYSPLASIA – left femur

35. CONFIRMATORY DIAGNOSIS
“Monostotic fibrous dysplasia with Shepherd’s
Crook deformity in left femur with
pathological fracture at subtrochanteric region”

36. TREATMENT
• This patient was posted for surgery on 08-07-16
• Procedure:
• Under combined spinal and epidural anaesthesia

37. • Supine position
• Fracture table

38. Through lateral approach upper end of the femur was exposed

39. • Implant removal (plates and screws) done
• Lateral cortex of femur was very thin hence we
curetted the cortex easily and carefully
• After curettage valgus wedge osteotomy was
done at sub trochanteric region to correct
deformity.
• Bone grafting was done

41. • Fragments were fixed with proximal femoral
locking compression plate (PFLCP).

42. • Wound was closed in layers with drain insitu

43. POST OP EVENTS
• 1st POD - drain -250ml
HB- 6.3 gm/dl
1 unit whole blood was transfused
• 2nd POD- drain 130 ml
HB- 7.6 gm /dl
1 unit whole blood was transfused
• Dressing and post op x ray done

44. Post op x ray on 2nd P O D

45. • 3rd POD- drain -50ml and
• drain removal done
• 5th POD- wound inspection done
- healthy
HB- 9.1 gm/dl

46. Post operative management
& follow up
• Initial 5 days patient was on IV ANTIBIOTICS and ORAL
ANTIBIOTICS for next 10 days
(inj cefoperazone+ sulbactam)
(tab. amoxicillin clavulanate)
• sutures were removed after 15th POD
• bisphosphonates started
• He was advised to take calcium & iron supplements regularly.
• Knee bending & quadriceps exercise advised.

47. Last follow up (1 month after surgery)
• Clinical
– Pain & Deformity markedly reduced
– wasting improving
– Now limb length discrepancy – 0.5cm
• Radiological
– Deformity is almost corrected
– well incorporation of the grafted bone.
– Union process is satisfactory

48. 1 Month

49. Discussion

50. FIBROUS DYSPLASIA
Definition:
“ A non-neoplastic primary disorder of bone in
which normal medullary bone is replaced by a
variable amount of structurally weak fibrous and
osseous tissue ”

51. Demographics
• Age: < 50 = 80 %
• Sex- M : F = 1:1
SITES : Most Common in jaw bones,
long bones &
ribs & skull are favored sites

52. Idiopathic
Non hereditary
Caused by mutation in GNAS1 gene
ETIOLOGY & PATHOGENESIS

53. Types of fibrous dysplasia
Fibrous
dysplasia
Monostotic
fibrous
dysplasia
70%
Polystotic
fibrous
dysplasia
30%

54. GROSS FEATURES
 The bone is often expanded
 tan grey colour
 firm-to-gritty consistency.
 There my be cysts, which
may contain some yellow-
tinged fluid

55. Microscopy
 Well cirumscribed
 Composed of fibrous & osseus component
 The fibrous component is composed of bland spindle cell with
low mitotic rate
 The osseus component is comprised of irregular curvilinear
trabeculae of woven bone (rarely lamellar bone)
 Secondary changes : foam cells, multinucleated giant cells,
aneurysmal bone cyst or myxoid changes

56. Curvilinear trabeculae often describing as resembling
‘Chinese characters’

57. COMPLICATIONS OF FIBROUS DYSPLASIA
1. Pathological fracture.
2. Bone deformity.
3. Massive cartilage hyperplasia.
4. Accelerated bone growth.
5. Sarcomatous degeneration.

58. PATHOLOGICAL FRACTURE

59. SHEPHERD CROCK DEFORMITY

60. SARCOMATOUS DEGENERATION
• is very rare, it may occur
– spontaneously or
– following radiation therapy.
• Radiological Criteria:
– Cortical destruction.
– Extra osseous soft tissue
component.

61. Special forms of fibrous dysplasia
• Leontiasis ossea.
• Cherubism.
• Mc Cune Albright syndrome.
• Mazabraud syndrome
is the rare combination of fibrous
dysplasia and soft-tissue myxomas

62. LEONTIASIS OSSEA
• A special form of polystotic fibrous dysplasia that
affects the skull & facial bones.

63. CHERUBISM
• Familial fibrous dysplasia of the jaws.

64. Mc Cune Albright
syndrome
• exclusively affect FEMALES.
Polystotic fibrous
dysplasia
• Precocious puberty.
• Hyperthyroidism.
• Gigantism or acromegally.
• Cushing syndrome.
Endocrine
disorder
• Café au lait patches that never cross
the midline & have irregular border
(coast of Main borders).
Skin
pigmentation

65. Mc Cune Albright syndrome Neurofibromatosis
Never cross the midline and Cross the midline.
Irregular borders
(coast of Maine)
Smooth borders
(coast of California)

66. THANK YOU