FIBROUS-DYSPLASIA- CASE-PRESENTATION-At-Shaheed-Suhrawardy-Medical-College-Hospital-Dhaka-Bangladesh (1).pptx is queued for conversion. Meanwhile you can add details and save.

1. A case Report
By-
Dr. Md Nazrul
Islam
MBBS, M.sc. (Bio-medical
Engineering).

2. Particulars of the
patient
• Name: Rabiul Islam
• Age: 20 years
• Gender: Male
• Address: Fulbaria, Bogra
• Occupation: Labour
• Marital status: Married
• Religion: Muslim
• Date of admission:17.09.09
• Date of examination:17.09.09

3. Chief complaints
Pain & deformity at the right upper thigh for 7
months following a trauma.
Gradual shortening of the right lower limb with
difficulty in walking for 6 months.

4. History of present
illness
According to the statement of the patient, he was
reasonably well 7 months back, then suddenly he felt
down on the ground by accidental trauma.
He could walk following trauma without support, after
which he noticed mild, fixed aching pain in the right
upper thigh which was not associated with
fever, non-radiating & aggravated during walking &
incompletely relived by taking some pain killers.

5. History of present
illness…cont
He also noticed a deformity in supero-lateral aspect of
right thigh which was gradually increasing in
size, associated with bending of the affected part &
shortening of the lower limb. For which his walking
became difficult & was possible only with a
support, for the last 6 months.

6. History of present
illness…cont
He has neither complain of pain & deformity in the
other parts of the body nor H/O weight loss or
loss of appetite .
With these complaints he got admitted at
ShaheedSuhrawardy Medical college Hospital
for better management.

7. History of past illness
He had no history of tuberculosis.
He is non Diabetic

8. Family history
None of his family member suffered
from such illness.
Personal history
He is not smoker

9. Socio-economic
Lower middle class family
Immunization history
Immunized against tuberculosis
& tetanus
Drug history
H/O taking NSAIDs to relieve pain

10. General examination
 Appearance: Ill looking
 Body built: Average
 Co-operation: Co-operrative
 Decubitus: On choice
 Anaemia: Absent
 Jaundice: Absent
 Cyanosis: Absent
 Oedema : Absent
 Temperature: normal

11. General examination…..cont.
 Pulse: 76 bts/min
 Blood pressure: 110/70 mm of Hg
 Respiratory rate: 16 /min
 Dehydration: No sign
 Koilonychia: Absent
 Leukonychia: Absent
 Clubbing: Absent
 Neck vein: Not engorged
 JVP: Not raised
 Lymph nodes: Not palpable
 Thyroid gland: Not palpable
 Skin pigmentation: Absent

12. Local examination: (Right Upper
thigh)
Look:
An ill defined deformity occupying at the
supero-lateral aspect of the upper right thigh
with convexity antero-laterally.
Skin over the deformed area is normal
Varus deformity of hip with shortening of the
lower limb.
Unable to walk without support.
Wasting of the thigh, &gluteal muscles
No engorged vein.

13. Local examination: (Right Upper thigh)
Feel:
There is an irregular, expanded bony deformity
with convexity antero-laterally extending from
the hip to subtrochanteric area. local
temperature normal, mild tenderness
present, over lying skin is free.
Shortening of limb - 9 cm.
Muscle wasting-
Gluteal - 4 cm.
Thigh – 4 cm.
Leg – 3 cm
Distal neurovascular status normal
Regional lymph nodes not enlarged.

14. Local examination: (Right Upper thigh)
Movement:
 walk with support.
 Trendelen Burg’s test positive
 Right Hip (ROM)–
Flexion 0-1000 [normal 0-1200]
Extension 0-50 [normal 0-200]
Abduction 0-50 [normal 0-400]
Adduction 0-150 [normal 0-250]
Internal rotation at 900 flexion 0-200[0-450]
External rotation at 900 flexion 0-100 [0-450]
Internal rotation in extension – 0-200 [0-350]
External rotation in extension – 0-150 [0-450]
 Rt. Knee & ankle: normal range of movement

15. Systemic examination:
Locomotorsystem
Gait:Can walk with support
Inspection:Varusdeformity - right hip
Palpation:Tenderness – affected area
Spine:Normal

16. Nervous system examination
 Higher psychic function: Normal
 Cranial nerve examination: Normal
 Motor function:
Inspection: Gross Muscle wasting in right
hip, thigh & leg

17. Nervous system
examination…cont
Palpation:
Bulk of muscle: Wasting Hip-4cm. thigh:
4cm, Leg 3cm
Tone of muscle:muscle tone is normal

18. Nervous system
examination…cont.
Power: [MRC scale]
Hip (rt.):
extensor- 2 internal rotator- 4
flexor- 4 external rotator- 3
adductor- 4
abductor- 3
Knee (rt.):
extensor- 3
flexor- 3

19. Nervous system
examination…cont.
 Deep tendon reflex:
All jerks are present & normal
 Sensory function test:
All the sensory functions are normal

20. Alimentary system
examination
Inspection: nothing abnormality detected
Palpation: soft, non tender
Percussion: tympanic
Auscultation: bowel sound present
Per-rectal examination: normal findings

21. Respiratory system
examination
Inspection: Normal in size & shape of the chest
Respiratory rate: 16 /min
Palpation: Trachea centrally placed, normal
chest expansibility
Percussion: Resonant
Auscultation: Bronchial breathing sound
with no added sound

22. Cardiovascular system examination
Pulse: 76 bts/ min
B.P. 110 mm of Hg
JVP: Not raised
Inspection: NAD
Palpation: Apex beat in Lt 5thintercostal space, NAD
Percussion: superficial cardiac dullness present over
the precordium
Auscultation: s1& s2 is audible
Geneto - Urinary system examination
Reveals no abnormality

23. Salient feature
Mr. Rabiul Islam, a 20 years old man, coming
from Fulbaria, Bagura admitted in
ShaheedSuhrawardy Medical College Hospital
with the complaints of pain & deformity at the rt.
Upper thigh following a mild accidental trauma 7
months back & gradual shortening of rt. Lower
limb with difficulty in walking for 6 months.

24. Salient feature….cont.
The pain was mild , fixed, non radiating, aching in
nature which was not associated with
fever, aggravated during walking & incompletely
relived by taking NSAIDs.
He also noticed a bending deformity in supero-
lateral aspect of right thigh which was gradually
increasing in size causing shortening of the
affected limb

25. Salient feature….cont.
Other parts of the body were normal with no history
of weight loss or anorexia. none of his family
member suffered from such illness.
On general examination, the patient is ill-
looking, not anaemic, non
icteric, normothermic, normotensive& skin
pigmentation is absent.

26. Salient feature….cont.
On local examination, an ill defined, mildly painful
bowing deformity was seen occupying at the supero-
lateral aspect of the right thigh with convexity antero-
laterally extending from the hip to subtrochanteric
area with CoxaVara. Overlying skin & local
temperature was normal.

27. Salient feature….cont.
Shortening of the limb was found 9 cm than the left. He
was unable to walk without support.
There was gross muscle wasting in rt. Lower
limb, measuring  gluteal- 4 cm, thigh- 4cm, leg- 3
cm. with loss of muscle power at hip & knee. Muscle
tone was normal.

28. Salient feature….cont.
Distal neurovascular status was normal & Regional
lymph nodes were not enlarged. Trendelen
Burg’s test was positive with reduced Range of
movement (ROM) in hip in all direction. ROM of
knee & ankle was normal. The spine was
normal. Other systemic examination reveals no
abnormality.

29. Provisional diagnosis
Fibrous dysplasia –
upper third of the right femur

30. Differential diagnosis
Giant cell tumor
Enchondroma
Aneurysmal Bone Cyst
Brown tumor

31. Investigations
1. X-Ray right thigh with hip A/P & lateral view:
Shows Shephard’s crook deformity (neck-shaft angle:
900) with multiple osteolytic lesions involving part
of the neck, trochanteric&subtrochanteric
area, with thinning of cortical bone & lucent
patches typically hazy, looks like ground-glass
appearance with pathological fracture at the
subtrochanteric region.

32. Fig: X-Ray right thigh with hip A/P & lateral
view

33. Investigations
 Blood for
 TC of WBC  9,000 / cu mm
 DC of WBC
 N  56% B  0%
 L  26% M  5%
 E  4%
 ESR  15 mm in 1st hr
 Hb%  12 gm / dl
 Urine RME Normal study
 CXR-P/A view Normal Chest skiagram
 MT Not significant
 RBS  76 mgm / dl

34. Investigations
 S. creatinine 0.9 mgm/ dl
 Blood urea 30 mgm / dl
 S. calcium 9 mgm / dl
 S. alkaline phosphates 110 IU/ L
 FNAC  No malignant cell
found, only cellular fibrous tissue
present.

35. Confirmatory diagnosis
“Monostotic fibrous dysplasia
with Shephard’s Crook
deformity in upper end of right
femur with pathological
fracture”

36. Treatment
 This patient was under gone for surgical
treatment on 17-10-09
 Procedure:
 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 curate the cystic areas carefully.

37. Treatment….cont.
Procedure…cont.:
 After curettage valgus wedge osteotomy was
done at subtrochanteric region to correct
deformity, massive irradiated allograft with
fibular auto graft was applied to enhance
healing & incorporation of the cystic bony
lesion & fragments were fixed with proximal
femoral interlocking nail (PFN).

38. Treatment….cont.
Procedure…cont.:
 Wound was closed in layers by keeping a drain
inside, which was removed after 48 hrs.
 Abduction bar was applied
 Specimen was sent for histopathology.

39. Histop-athologicalReport
Shows loose cellular fibrous
tissue with wide spread patches of
immature bone - Suggestive of
Fibrous dysplasia.

40. Post operative management
&follow up
 Stitches were removed after 10th POD
 Only isometric quadriceps exercise advised.
 He was advised to take calcium&Bisphosphonates
preparation regularly.
 After removal of the abduction bar at 2 months
clinically
&radiologically bone was stable & uniting satisfactorily .
 Knee bending & quadriceps exercise advised.
 He was advised to use crutch for non weight bearing
up to 3 months.
 After 3 months partial weight bearing started with 2
cm shoe raised along with other exercise.

41. Fig: Post operative period

42. Last follow up (4 ½ months
after surgery)
• Clinical
• Pain & Deformity markedly reduced
• Can walk with single crutch
• Muscle power & wasting improving
• Now LLD - only 2 cm
• Radiological
• Deformity is almost corrected
• Now neck-shaft angle: 1350
• well incorporation of the grafted bone.
• Union process is satisfactory at the
osteotomy site.

43. Fig: Preoperative X-rayno 17.09.09

44. Peroperative X-ray on 17.10.09
Before &Afterosteotomy

45. Fig: Post operative X-ray Rt. Upper Femur
On 10th POD After 7 weeks

46. Fig: Post operative X-ray Rt. Upper Femur
After 4 ½ months
After 3 months