Haemophillia and challenges it offers to orthopaedicians..... Normal coagultion mechanism...

1. HAEMOPHILIA & ORTHOPAEDICS
UMESH YADAV
PGIMS, ROHTAK

9. fibrinolysis
(tPA)
PLASMINOGEN PLASMIN.
[FDPs]

10. Factor XII Factor XIIa
+ high Mol.wt
kininogen
kallikrenparakallikren
tPA
plasminogen plasmin
fibrin
FDPs
F V F VIII
COAGULATION

11. ROLE OF CALCIUM

12. INR Intro.by WHO(1983):itz
the 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

14. USES of PT/INR:

18. • 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

19. 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

20. DISADV
Viral transmission
Large volume
needed.

21. 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 articular
hemorrhages)

22. 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.

23. 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.

24. 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.

25. 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

26. 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

27. 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

28. Hematomas
• Subcutaneous and muscular
hematomas spread within fascial
spaces, 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

29. • 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

30. 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.

31. 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).

32. 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.

33. 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

34. • If possible- Lab for factor levels post op or atleast
APTT.
• 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.

35. 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 ??

36. • 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.

37. 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………