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dr. Bintang Soetjahjo, SpOT (K)


Love of Bleeding



X linked recessive
hereditary disorder that is
due to defective or deficient
factor VIII

Also known as Christmas
disease or Factor IX

Why does it
matter ?


80% in musculoskeletal
20% CNS & others

Clinical manifestations Severity Severe Moderate Mild F VIII activity Clinical manifestations <1% Spontaneous hemorrhage from early infancy Freq sp hemarthrosis 2-5% Hemorrhage sec to trauma or surgery Occ sp hemarthrosis >5% Hemorrhage sec to trauma or surgery Rare sp bleeding • Frequency and severity of bleeding are related to F VIII levels .

BASIC Hematological management Early days : Later on : ON DEMAND MANAGEMENT Factor was used iv only CONTINUOUS PROPHYLAXIS when the patient suffered a hemorrhage which can be primary (started when the children begin to walk or just after the first hemarthrosis) or secondary (started after several or many articular hemorrhages) Haemophillic arthropathy in 2ND / 3RD decade .

Musculoskeletal Problems Hemarthrosis Haemophilic Arthropathy ↓ Periartikular Bone Quality Muscle Hematoma Fracture .

erythema. economically and psychologically debilitating manifestation Clinical Finding o o o o o o Sensoris: tingling warm sensation Excruciating pain Generally affects one joint at the time MC: knee. warmth and LOM If treated early it can subside in 6 to 8 hs and disappear in 12 to 24 hs. painful and most physically.Hemarthrosis Most common. but there are others as elbows. wrists and ankles Edema. Complications: Chronic involvement with joint deformity complicated by muscle atrophy and soft tissue contractures .

Hemophilic Arthropathy Characterized • Chronic proliferative synovitis • Cartilage destruction Etiologi • Recurrent intraarticular bleeding .

Hemophilic Arthropathy Pathogenesis : Joint involvement .

inflammation.Hemophilic Arthropathy Hemophilic arthropathy pathology Haemorrhage into joint • synovial irritation. subsynovial fibrosis • Haemosiderin appears in synovial cells Repeated bleeds • Synovial becomes thick and heavily pigmented Vascular pannus (+) over articular surface • Cartilage gradually eroded Cartilage-degrading enzymes • Accumulated iron in cells • Proliferative synovitis • Prolonged/repeated joint immobilization Cartilage degradation • Subchondral bone exposed & penetrated • Large cysts develop at bone ends .

Arthritis Rheum 56:199–207 . both events resulting in a loss of matrix All these adverse effects can be partially prevented by N-acetylcysteine  involvement of oxygen metabolite Jansen (2007) Exposure of human cartilage tissue to low concentrations of blood for a short period of time leads to prolonged cartilage damage: an in vitro study. et.Hemophilic Arthropathy In Vitro Studies A 4-day duration of blood exposure produces : ↓↓ cartilage matrix formation ↑↑ release of matrix components.

Haemophilia 13(Suppl. Hoots WK (2007) Pathogenesis of haemophilic synovitis: experimental studies on blood-induced joint damage.Hemophilic Arthropathy In Vivo Experiment • A single joint hemorrhage results in lasting adverse changes in chondrocyte activity and cartilage matrix integrity • One episode of hemarthrosis • before the initiation of prophylactic treatment • may result in joint damage decades later. • Iron  induction of synovial changes  Haemophilic arthropathy Valentino LA. Hakobyan N. Rodriguez N. 3):10– 13 .

chronic hemophilic synovitis of the knee .

Muscle hematomas Characterized Common • Iliopsoas Risk • Compartment syndrome • Nerve compression .

Muscle Hematom as Hematolo gical treatment Resolv ed (weeks/months ) Rebleedi ng Hemophilic Pseudotum or .

dissecting deeper structures • Subcutaneous bleeding spreads in characteristic mannerin the site of origin the tissue is indurated purplish black and when it extends the origin starts to fade. .Muscle hematomas • Subcutaneous and muscular hematomas spread within fascial spaces.

Muscle hematomas May compress vital structures: • Airway if it is bleeding into the tongue throat or neck. especially of calves and forearms . • Arteries causing gangrene and ischemic contractures are common sequelae.

Muscle hematomas 1) 2) 3) 4) Calf Thigh Buttocks Forearms Psoas hematoma if right sided may mimic acute appendicitis Retroperitoneal hematoma: Can dissect through the diaphragm into the chest compromising the airway. It can also compromise the renal function if it compresses the ureter .

Pseudotumors • Dangerous and rare complication • Blood filled cysts that are gradually expanding • Occur in soft tissues or bones. . • Most commonly in the thigh • As they increase in size they erode contiguous structures.

Hemophilic pseudotumor . posterior thigh pseudotumor . surgical specimen which resulted from the removal of the pseudotumo .

fistulization. and pathological fractures (requiring even amputation of the affected limb) . Should only be carried ou in major hemophilia centers by a multidisciplinary surgica team Postoperative complications: Infection.Pseudotumors Management of Pseudotumor Surgical Removal Percutaneus Management Exeresis & Filling of Dead Cavity Irradiation Embolization Treatment of choice But.

• If a fracture is correctly treated in a hemophilic patient. • Closed POP casts are not recommended in hemophilia .Fracture • Common in hemophiliacs because of their active lifestyles. •  Rigid internal fixation to external fixation. it will progress to healing in a similar time frame to those occurring in the general population .risk of compartment syndrome. as usual under hematological control.

• Maintained at 60% of normal for 14 postoperative days.  • Clotting factor replacement is infused to obtain a level of 30% of normal prior to rehabilitation sessions for 8–10 weeks.Level of F-VIII & SURGERY   • Infused preoperatively until a minimum level of 100% of normal is achieved. .

↓ Periartikular Bone Quality Radiosynovectomy Indication : Chronic hemophilic synovitis causing recurrent hemarthroses. unresponsive to hematological treatment Agents : Yttrium-90 (knees) Rhenium-186 (elbows and ankles) Effects : ↓↓ the synovial and hypertrophy ↓↓ Hemarthroses Slows cartilage destruction .

↓ Periartikular Bone Quality Radiosynovectomy Procedure: Can be repeated 3x w/ 3-months interval < 3 joints in 1 session Advantages: • Simple • Virtually painless • Inexpensive If fail  Arthroscopic synovectomy .

↓ Periartikular Bone Quality Radiosynovectomy .

Anesthesia in hemophilia Spinal anesthesia • Intraneural hemorrhage • Neurological lesion General anesthesia (preferred choice) .

Compartment syndrome Preferred choice Recommended in most displaced fractures Best choice of initial stabilization . Treatment Closed plaster cast Rigid Internal Fixation Internal Stabilization External Fixation Not recommended.Fractures Goal Obtain an optimal outcome with the patient’s return to full activity as soon as possible.

physiotherapist  Satisfactory result • Factor supplementation • Especially post procedure • Avoid rebleeding • Continuous prophylaxis • Slows development • But complication still arise • Surgical procedures needed. . rehabilitation physician.CONCLUSIONS • Any surgical procedure • Under hematological control (factor cover) • Consult hematologist for assistance in procedure • Close cooperation • Orthopaedic surgeon.