HAEMOPHILIA & ORTHOPAEDICSUMESH YADAV

PGIMS, ROHTAK

fibrinolysis

(tPA)

PLASMINOGEN PLASMIN.

[FDPs]

Factor XII Factor XIIa+ high Mol.wt

kininogen

kallikrenparakallikren

tPA

plasminogen plasmin

fibrinFDPs

F V F VIII

COAGULATION

ROLE OF CALCIUM

INR Intro.by WHO(1983):itzthe ratio of PT that adjusts for

the sensitivity of the thromboplastin reagants,such

that normal coagulation profile is reported as an INR

of 1.0

USES of PT/INR:

• Hemophilia- “love of bleeding”• 2 types: A and B• Hemophilia A: –X linked recessive hereditary disorder that is

due to defective or deficient factor VIII.• Hemophilia B:

–Also known as Christmas disease or Factor IX Deficiency• It is the second most common inherited clotting

factor abnormality (after von Willebrand disease)• 1 in 5000-10000 live male births• No difference between racial groups

Definition

Clinical manifestations

• Frequency and severity of bleeding are related to F VIII levels

Severity F VIII activity Clinical manifestations

Severe <1%

Spontaneous hemorrhage from

early infancy

Freq sp hemarthrosis

Moderate 2-5%

Hemorrhage sec to trauma or

surgery

Occ sp hemarthrosis

Mild >5%

Hemorrhage sec to trauma or

surgery

Rare sp bleeding

DISADVViral transmission

Large volume needed.

HAEMOPHILIA & ORTHOPAEDICS

• 80% of hemorrhages occur in the musculo-skeletal system, while 20% take place in the CNS and other organ systems.

• Earlier ON DEMAND MANAGEMENT- Factor was used iv only when the patient suffered a hemorrhage.-Haemophillic arthropathy in 2ND /3RD

decade.

• Now a days-CONTINUOUS PROPHYLAXIS, which can be primary (started when the children begin to walk or just after the first hemarthrosis) or secondary (started after several or many articularhemorrhages)

Level of F-VIII & SURGERY

• Infused preoperatively until a minimum level of 100% of normal is achieved.

• 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.

PAIN MANAGEMENT

• Any intramuscular injections should be avoided due to the risk of producing hematomas at the injection site.

• Aspirin and NSAIDs should be avoided due to the risk of gastrointestinal bleeding.

• COX-2 agents may be prescribed and have been found to be very effective in the management of articular pain and swelling in affected joints.

ANAESTHESIA IN HAEMOPHILICS

• Many anesthesiologists fear spinal anesthesia for the risk of neurological lesion in case of an intraneural hemorrhage.

• General anesthesia is preferred in the majority of centers for hemophiliacs.

• General anesthesia is the obvious choice when, occasionally, a double or triple surgical procedure is planned for the same session.

Hemarthrosis• Most common, painful and most physically,

economically and psychologically debilitating manifestation.

• C/F: Aura: tingling warm sensation Excruciating pain Generally affects one joint at the time MC: knee; but there are others as elbows,

wrists and ankles. Edema, erythema, warmth and LOM If treated early it can subside in 6 to 8 hs and

disappear in 12 to 24 hs.

Complications: Chronic involvement with joint deformity complicated by muscle atrophy and soft tissue contractures

HAEMOPHILIC ARTHROPATHY

• Vicious cycle –

Haemarthrosis – Synovitis with synovial hypertrophy – Fragile & easily bleeding synovium – l/t Haemarthrosis.

- If untreated – Fixed flexion contractures

- Ultimately joint destruction- HA

RADIO SYNOVECTOMY

• Indications for radiosynovectomy include chronic hemophilic synovitis causing recurrent hemarthroses, unresponsive to hematological treatment.

• Involves the injection of Yttrium-90 for knees and Rhenium-186 for elbows and ankles.

• Radioactive synovectomy is a relatively simple, virtually painless, and inexpensive technique

Hematomas• Subcutaneous and muscular

hematomas spread within fascialspaces, dissecting deeper structures

• Subcutaneous bleeding spreads in characteristic manner- in the site of origin the tissue is indurated purplish black and when it extends the origin starts to fade.

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

• Muscle hematomas: 1)calf,2)thigh,3)buttocks,4)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.

A pseudotumor is deforming the cortex of the femur (arrow). Other ossified masses in the soft tissues (arrowheads) are probably soft-tissue pseudotumors.

MANAGEMENT OF PSEUDOTUMOR

• Surgical removal, percutaneous management, exeresis and filling of the dead cavity, irradiation, and embolization.

• Surgical excision is the treatment of choice but should only be carried out in major hemophilia centers by a multidisciplinary surgical team.

• The main postoperative complications are infection, fistulization, and pathological fractures (requiring even amputation of the affected limb).

Fractures in Haemophilics

• Common in hemophiliacs because of their active lifestyles.

• Closed POP casts are not recommended in hemophilia - risk of compartment syndrome.

• Rigid internal fixation to external fixation, as usual under hematological control.

• If a fracture is correctly treated in a hemophilic patient, it will progress to healing in a similar time frame to those occurring in the general population.

HAEMOPHILLIA & ARTHROPLASTY

• Indication for a prosthetic joint replacement is a joint with advanced degenerative disease that is painful and may have associated stiffness and deformity.

• PRE OPERATIVE EVALUATION---------

• Inhibitor status

• HIV antibody, viral load, and CD4 count

• Hepatitis C and viral load

• Fibrinogen, prothrombin time/INR, platelet count

• Cardiopulmonary status

• Inspection of venous access

• If possible- Lab for factor levels post op or atleastAPTT.

• DVT Prohpylaxis- CONTROVERSIAL ????

• If needed combine 2 procedures like – B/L TKR. TKR WITH THR.

• Risk of infection in hemophiliacs is higher than in normal population, especially in those who are HIV-positive.

• Surgeon should weigh the risks and benefits carefully considering clinical and immunological status.

CASE SCENERIO ?

• A person with hemophilia who has had a successful total joint replacement may experience is a sudden onset of pain and swelling of the replaced joint.

• DIAGNOSIS ??

• Differential- Acute bleeding episode

• (BUT---it is uncommon to have a spontaneous bleeding occurrence in a totally replaced joint)

• Failure to respond to factor replacement should be a clear signal that this may be something else other than a bleed.

• INFECTION IS THE DIAGNOSIS UNTIL PROVEN OTHERWISE.

• Immediate aspiration of the joint and studies of the fluid including gram stain and cultures should be performed.

TAKE HOME MESSAGE

Any surgical procedure in hemophilia must be performed under factor cover; that is to say, under hematological control.

Factor supplementation is also recommended for periods when intensive physiotherapy is needed especially after surgical procedures (to avoid rebleeding during the rehabilitation program).

Although continuous prophylaxis can slow the development of the orthopedic complications of hemophilia, these complications still arise.

Concomitant HIV infection increases risk of infections………