Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

44
ORTHO PATHO MEET PRESENTER : Dr. SAUMYA AGARWAL Junior resident Dept of Orthopaedics J.N.Medical College and Dr. Prabhakar Kore Hospital and MRC, Belgaum

Transcript of Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

Page 1: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

ORTHO PATHO MEET

PRESENTER : Dr. SAUMYA AGARWAL

Junior resident Dept of Orthopaedics J.N.Medical College and Dr. Prabhakar Kore Hospital and MRC, Belgaum

Page 2: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

NAME : XYZ

AGE : 20 Yrs

SEX : MALE I.P NO. : 659161

ADDRESS : RESIDENT OF BELGAUM

OCCUPATION : FARMER

Page 3: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

CHIEF COMPLAINTS

Patient complaints of pain at right hip while working for a long time since 4 months.

Page 4: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

HISTORY OF PRESENTING ILLNESS

• Patient was apparently alright 4 months back when

he started complaining of pain in right hip.

• Pain was insidious in onset, gradually progressive,

aggravated on working and relieved on rest.

Page 5: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

• No history of :1) Trauma / injury at the site of deformity2) Fever3) Significant Loss of body weight4) Steroid intake5) Other joint pain

Page 6: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

PAST HISTORY

• No history of similar complaints in the past.

• Not a Known case of Diabetes Mellitus, Hypertension

and Ischemic heart disease.

• Not a known case of Tuberculosis, hyperthyroidism and

other chronic illness.

FAMILY HISTORY

• Nothing significant

Page 7: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

PERSONAL HISTORY

• Diet : Mixed

• Appetite : Not decreased

• Sleep : Undisturbed

• Bowel & Bladder : Normal and regular

• Takes alcohol occasionally , non smoker

Page 8: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

GENERAL EXAMINATION

• Patient is conscious, cooperative and well oriented to time , place and person .

• Moderately built• Afebrile• Pulse – 76 /min• Blood pressure - 116/80 mmHg• Respiratory rate – 20 / min• No pallor / cyanosis / edema / icterus /

clubbing / lymphadenopathy

Page 9: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

SYSTEMIC EXAMINATION

• CVS : S1 S2 heard, No murmurs

• RS : Air entry equal on both sides

• PA : Soft, Non tender, no organomegaly, Bowel

. sounds heard

• CNS : No focal neurological deficit

Page 10: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

LOCAL EXAMINATION

• Gait – normal

• Attitude – neutral

• INSPECTION – Left Hip – normal

• Right hip -

• No any swelling / sinus / scar / discharge / inflammatory

changes

• No evidence of shortening of both lower limbs.

Page 11: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

• PALPATION –

• No any local rise of temperature

• No Tenderness – present over the right hip

• Range of movement of right hip – normal

• Range of movement of right knee – normal

• No muscle wasting

• Toe movements – present

• Distal pulses – felt on both sides

• No neurological deficit

Page 12: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

INVESTIGATIONS

• Hb – 12.4 gm%• TLC – 8920/ cumm• RBC - 4,50000 / cumm• ESR – 14• Platelet count – 2,53000/ cumm• PCV - 36.3• Blood Group – B +

Page 13: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

• S. Creatinine – 0.9

• Blood Urea – 19

• S. Sodium – 138 meq/l

• S. Potassium – 4.8 meq/l

• S. Calcium - 9.8

• S. Alkaline phosphatase – 300

Page 14: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

X-Ray Pelvis AP view Showing Right Hip

Page 15: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

X-Ray Right Hip lateral view

Page 16: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

X-Ray Findings

• A lytic lesion seen in neck of femur showing well defined geographic type of destruction with sclerotic rim.

• Intramedullary radiolucencies with haziness seen.

Page 17: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

DIFFERENTIAL DIAGNOSIS

1) Osteo fibrous dyspalsia2) Osteoid Osteoma3) Osteochondroma4) Non Ossifying Fibroma5) Fibrous Dysplasia6) Unicameral bone cyst7) Aneurysmal bone cyst

Page 18: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

1) Osteo fibrous dyspalsia

Points in Favour : Age : 1st and 2nd decades Male preponderence Imaging : eccenteric intracortical osteolysis with

expansion of cortex

Points Against : pain is absent Site : diaphysis of long bones

Page 19: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 20: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

2) Osteoid Osteoma Points in Favour :• Age : 2nd and 3rd decades• Male : female :: 3:1• Site : lower extremity, long bones, diaphyseal/

metaphyseal• Pain Points Against :• Imaging : cortical radiolucent nidus <1.5cm

with marked cortical thickening

Page 21: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 22: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

3) Oteochondroma Points in Favour : Age : 2nd and 3rd decades Male preponderence Site : metaphysis of long bones

Points Against : Mass is present Imaging : pedunculated or sessile bone

lesion that communicates with intramedullary canal

Page 23: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 24: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

4) Non Ossifying Fibroma Points in Favour :• Age : 1st and 2nd decades• Site : Metaphysis of long bones• Asymptomatic

Points Against :• Imaging : geographic , eccentric lesion located

in metaphysis of long bones well defined sclerotic margins.

Page 25: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 26: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

5) Fibrous Dysplasia Points in Favour : • Age : 1st to 3rd decades• Male : female :: 1:1• Site : femur, tibia• Pain• Imaging : ground glass appearance with well

defined sclerotic rim

Page 27: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 28: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

6) Unicameral Bone Cyst Points in Favour :• Age : 1st and 2nd decades• Male : female :: 2:1• Site : proximal femur, proximal humerus• Asymptomatic unless pathological fracture

Points against: • Imaging : centrally located , purely radiolucent

lesion , no cortical destruction.

Page 29: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 30: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

7) Aneurysmal Bone Cyst

Points in Favour :• Age : 1st – 2nd decades• Pain

Points Against :• Female preponderence• Site: proximal humerus, distal femur, proximal

tibia, spine• Imaging : eccentric expansile radiolucent lesion

Page 31: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 32: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

Fibrous Dysplasia

HISTORY :• The term fibrous dysplasia was originally

proposed by Lichtenstein in 1938.• He along with Jaffe, McCune, and Albright,

described this disorder of bone, as well as other extra skeletal abnormalities with which it is occasionally associated.

Page 33: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

DEFINITION :• Fibrous Dysplasia is a benign, nonfamilial

disorder characterized by the presence of expanding intramedullary fibro-osseous tissue in one or more bones.

INCIDENCE :• Not known• Accounts for 5-7% of clinical benign tumors • Occur more frequently in girls than boys

Page 34: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

AGE :• Affects 1st - 3rd decades

SITE :• Fibrous dysplasia can affect any bone • Femur and Tibia being most common

Page 35: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

HISTOPATHOLOGY

• Irregular woven bone spicules with a fibrous stroma.

• Trabeculae of woven bone surrounded by cellular fibroblastic tissue.

• There is no osteoblastic rim at the bone-stromal interface.

Page 36: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 37: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

• Fibrous Dysplasia can be classified into one of four categories :

1. Monostotic : single bone2. Polyostotic : multiple bones3. Craniofacial fibrous dysplasia : skull and facial

bones alone4. Mandible and maxilla alone

Page 38: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

CLINICAL PRESENTATION

• Pain• Deformity• Cutaneous pigmentation• Endocrine abnormalities

Page 39: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

IMAGING

• Ground glass appearance with well defined sclerotic rim

• may be completely lucent (cystic) or sclerotic• well circumscribed lesions• no periosteal reaction

Page 40: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal
Page 41: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

TREATMENT

• Prophylactic fixation of impending fractures• Correction of deformity• Bisphosphonates for severe cases

Page 42: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

McCune-Albright Syndrome

• The triad of :• precocious puberty (endocrinopathy), • Café au lait spots, and • polyostotic bone involvement

Page 43: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal

Mazabraud syndrome

• Mazabraud’s syndrome is a rare disorder characterized by fibrous dysplasia, which can develop in a single bone (monostotic) or in multiple bones (polyostotic), associated with one or more soft-tissue myxomas.

Page 44: Ortho Patho Meet on Fibrous Dyspalsia by Dr. Saumya Agarwal