A case of FIBROUS DYSPLASIA

PRESENTER

Dr.Karna Venkateswara Reddy

2nd yr PG

MODERATOR DR.V. Nageswara Rao DNB(ortho),FJR Associate professorDept of orthopaedicsG.S.L Medical college &hospital

• NAME : Marisetty Annavaram

• AGE: 36 Years

• GENDER: male

• PLACE : Kanumagraharam

• OCCUPATION: Labourer

• MARITAL STATUS: Married

• DATE OF ADMISSION: 15.06.16

CHIEF COMPLAINTS

• C/O Pain in left groin since 1 day

• Difficulty in walking since 1 day

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

• No h/o loss of consciousness

• No h/o head injury

• Bowel n bladder –normal

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

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

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

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

14- MAY – 2016

H/o fall from bike

GSL GENERAL HOSPITAL

• Not a known case of Diabetes / Hypertension

• No other significant medical history in past

• Personal history - Mixed diet

Non- smoker

Non –alcoholic

• Family history – Nothing significant

General examination

• Appearance: moderately built and nourished

• Pallor : present • Icterus : Absent

• Cyanosis: Absent

• edema : Absent

• Clubbing: Absent

• Lymph nodes: Not palpable

• Skin pigmentation: Absent

Vitals @ time of admission

• Pulse: 76 bts/min

• Blood pressure: 110/70 mm of Hg

• Respiratory rate: 20 /min

• Temperature: normal

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

Varus deformity of hip with shortening of the lower limb.

Wasting of the thigh, & gluteal muscles

No engorged veins.

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.

MOVEMENT

• Left Hip (ROM)– painful.

• Knee & ankle –ROM – normal

Measurements

• Shortening of limb – 3 cm.

(femoral component)

• Muscle wasting-

Thigh – 4 cm.

Leg – 2 cm

OTHER SYSTEMS

• Respiratory system examination - NAD

• Cardiovascular system examination - NAD

• P/A Abdomen - NAD

• CNS examination- No focal neurological deficit

Investigations

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

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

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

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

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

PROBABLE DIAGNOSIS

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

DIFFERENTIAL DIAGNOSIS

• Fibrous Dysplasia

• Pagets disease

• Nonossifying fibromas

• Aneurysmal Bone Cyst

• 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

PATHOLOGY

• 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)

Microscopic features

X 100H &E

Curvilinear trabeculae (Chinese

letters) of metaplastic woven bone

devoid of osteoblastic rimming

Normal osteoblastic rimming

X100 H&E

Sheets of benign spindle cells Foamy macrophages

Microscopic features

X 400 H&E

• OPINION –

Features consistent with FIBROUS DYSPLASIA – left femur

CONFIRMATORY DIAGNOSIS

“Monostotic fibrous dysplasia with Shepherd’s

Crook deformity in left femur with

pathological fracture at subtrochanteric region”

TREATMENT

• This patient was posted for surgery on 08-07-16

• Procedure:

• Under combined spinal and epidural anaesthesia

• Supine position

• Fracture table

Through lateral approach upper end of the femur was exposed

• 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

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

• Wound was closed in layers with drain insitu

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

Post op x ray on 2nd P O D

• 3rd POD- drain -50ml and

• drain removal done

• 5th POD- wound inspection done

- healthy

HB- 9.1 gm/dl

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.

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

1 Month

Discussion

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 ”

Demographics

• Age: < 50 = 80 %

• Sex- M : F = 1:1

SITES : Most Common in jaw bones,

long bones &

ribs & skull are favored sites

Idiopathic

Non hereditary

Caused by mutation in GNAS1 gene

ETIOLOGY & PATHOGENESIS

Types of fibrous dysplasia

Fibrous dysplasia

Monostoticfibrous

dysplasia

70%

Polystoticfibrous

dysplasia

30%

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

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

Curvilinear trabeculae often describing as resembling ‘Chinese characters’

COMPLICATIONS OF FIBROUS DYSPLASIA

1. Pathological fracture.

2. Bone deformity.

3. Massive cartilage hyperplasia.

4. Accelerated bone growth.

5. Sarcomatous degeneration.

PATHOLOGICAL FRACTURE

SHEPHERD CROCK DEFORMITY

SARCOMATOUS DEGENERATION• is very rare, it may occur

– spontaneously or

– following radiation therapy.

• Radiological Criteria:– Cortical destruction.

– Extra osseous soft tissue component.

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

LEONTIASIS OSSEA

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

CHERUBISM

• Familial fibrous dysplasia of the jaws.

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

Mc Cune Albright syndrome Neurofibromatosis

Never cross the midline and Cross the midline.

Irregular borders

(coast of Maine)

Smooth borders

(coast of California)

THANK YOU