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