fibrous-dysplasia-case-presentation-at-shaheed-suhrawardy-medical-college-hospital-dhaka-bangladesh...
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By-
Dr. Md Nazrul IslamMBBS, M.sc. (Bio-medical Engineering).
FIBROUS DYSPLASIA
A case Report
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
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.
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.
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.
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 Shaheed Suhrawardy Medical college Hospital for better management.
History of past illness
He had no history of tuberculosis.
He is non Diabetic
Family history
None of his family member suffered from such illness.
Personal history
He is not smoker
Socio-economic
Lower middle class family
Immunization history
Immunized against tuberculosis & tetanus
Drug history
H/O taking NSAIDs to relieve pain
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
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
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.
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.
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
Systemic examination:
Locomotor system
Gait: Can walk with support
Inspection: Varus deformity - right hip Palpation: Tenderness – affected area
Spine: Normal
Nervous system examination
Higher psychic function: Normal
Cranial nerve examination: Normal
Motor function:
Inspection: Gross Muscle wasting in right hip, thigh & leg
Palpation: Bulk of muscle: Wasting Hip-4cm.
thigh: 4cm, Leg 3cm
Tone of muscle: muscle tone is normal
Nervous system examination…cont
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
Nervous system examination…cont.
Deep tendon reflex:
All jerks are present & normal
Sensory function test:
All the sensory functions are normal
Alimentary system examination
Inspection: nothing abnormality detected
Palpation: soft, non tender
Percussion: tympanic
Auscultation: bowel sound present
Per-rectal examination: normal findings
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
Cardiovascular system examination
Pulse: 76 bts/ min
B.P. 110 mm of Hg
JVP: Not raised
Inspection: NAD
Palpation: Apex beat in Lt 5th intercostal space, NAD
Percussion: superficial cardiac dullness present over the precordium
Auscultation: s1 & s2 is audibleGeneto - Urinary system examination
Reveals no abnormality
Salient feature
Mr. Rabiul Islam, a 20 years old man, coming from Fulbaria, Bagura admitted in Shaheed Suhrawardy 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.
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
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.
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 Coxa Vara. Overlying skin & local temperature was normal.
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.
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.
Provisional diagnosis
Fibrous dysplasia – upper third of the right femur
Differential diagnosis
Giant cell tumor
Enchondroma
Aneurysmal Bone Cyst
Brown tumor
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.
Fig: X-Ray right thigh with hip A/P & lateral view
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
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.
“Monostotic fibrous dysplasia with Shephard’s Crook
deformity in upper end of right femur with pathological
fracture”
Confirmatory diagnosis
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.
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).
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.
Histop-athological Report
Shows loose cellular fibrous tissue with wide spread patches of immature bone - Suggestive of Fibrous dysplasia.
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.
Fig: Post operative period
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.
Fig: Preoperative X-ray no 17.09.09
Peroperative X-ray on 17.10.09
Before & After osteotomy
Fig: Post operative X-ray Rt. Upper Femur
On 10th POD After 7 weeks
Fig: Post operative X-ray Rt. Upper Femur
After 3 months After 4 ½ months
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