MUSCULOSKELETAL

COMPLICATIONS OF
HEMOPHILIA
dr. Bintang Soetjahjo, SpOT (K)

Definition
Hemophilia

Love of Bleeding

Hemophilia
A

Hemophilia
B

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

Also known as Christmas

disease or Factor IX
Deficiency

Why does it
matter ?

Haemorrhag
e

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

Hemarthrosis
Most common, painful and most physically,
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; 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

Hemophilic Arthropathy

Characterized
Chronic proliferative
synovitis
Cartilage destruction
Etiologi
Recurrent intraarticular bleeding

Hemophilic Arthropathy
Pathogenesis : Joint involvement

Hemophilic Arthropathy
Hemophilic arthropathy pathology
Haemorrhage into joint
synovial irritation, inflammation, 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

Hemophilic Arthropathy
In Vitro Studies
A 4-day duration of blood exposure
produces :
cartilage matrix formation
release of matrix components,
both events resulting in a loss of matrix
All these adverse effects can be partially prevented
by
N-acetylcysteine involvement of oxygen
metabolite
Jansen N. et.al (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. Arthritis Rheum
56:199207

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, Hoots WK (2007) Pathogenesis of haemophilic

synovitis: experimental studies on blood-induced joint damage. Haemophilia 13(Suppl. 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

Muscle hematomas

Subcutaneous and
muscular
hematomas spread
within fascial spaces,
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

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)
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

Pseudotumors
Management of Pseudotumor

Surgical
Removal
Percutaneus
Management
Exeresis &
Filling of Dead
Cavity
Irradiation
Embolization

Treatment of
choice
But, Should only be carried ou
in major hemophilia centers
by a multidisciplinary surgica
team
Postoperative complications:
Infection, fistulization, and
pathological fractures (requiring
even amputation of the affected
limb)

Fracture
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

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 810weeks.

 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)

Fractures
Goal
Obtain an optimal outcome with the
patients return to full activity as soon as
possible.
Treatment
Closed plaster
cast
Rigid Internal
Fixation
Internal
Stabilization
External Fixation

Not recommended, Compartment

syndrome
Preferred choice
Recommended in most displaced
fractures
Best choice of initial
stabilization

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

THANK YOU