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Stenosis parsial atau segmental dari hepatic

outflow dijumpai pada 60% pasien dan 25-

30% pasien dengan obstruksi vena hepatica.
Kondisi ini memerlukan tindakan
angioplasty atau pemasangan stent untuk
menjaga patensi. Pada pasien-pasien yang
tidak responsif terhadap terapi antikoagulan
dan juga bukan merupakan kandidat untuk
angioplasty atau pemasangan stent, dapat
dipikirkan untuk dilakukan tindakan
transjugular intrahepatic portosystemic
shunt (TIPS) atau orthotopic liver
transplantation (OLT).6
Etiologi dari BCS
• Proteins C and S are two vitamin K-dependent plasma proteins that work in concert as a natural
anticoagulant system. Activated protein C is the proteolytic component of the complex and protein S
serves as an activated protein C binding protein that is essential for assembly of the anticoagulant complex
on cell surfaces. The anticoagulant activity is expressed through the selective inactivation of Factors Va and
VIIIa. Many patients deficient in proteins C and S have been described and have an associated thrombotic
tendency, but not all heterozygous protein C and S deficient individuals experience thrombotic
complications. Multiple mechanisms and/or drugs can lead to acquired deficiencies of these proteins: oral
anticoagulation, liver disease, DIC and in the case of protein S, lupus erythematosus, nephrotic syndrome,
pregnancy and certain hormones. The anticoagulant activity of protein C decreases rapidly after
administration of warfarin (i.e., with a time course similar to Factor VII). This rapid decrease may lead to a
transient imbalance and contribute to coumarin induced skin necrosis. Protein S antigen levels do not
decrease as rapidly, but protein S functional levels are often low in patients with an acute thrombus. The
discrepancy between antigen and function results from elevations in C4b-binding protein, which complexes
reversibly with protein S. Unlike free protein S, the complex does not function in the anticoagulant pathway.
The available information all suggest that deficiency of protein C and protein S should be considered a risk
factor contributing to recurrent thrombotic disease and that the function of these proteins is altered by
many common clinical conditions which have associated an increased risk of thrombosis.
• Collateral pathways in Budd Chiari
syndrome: multiple collateral path
ways have been described in BCS.
These could be intrahepatic or extra
1) Intrahepatic collaterals: Two forms of
intrahepatic collaterals are seen
Those that drain into the systemic veins
through subcapsular venous plexus and
Those that shunt the segment of
occluded vein into the nonoccluded
venous segment and are seen as
“comma” shaped veno-venous
collaterals. These are best seen
on sonography [4] (Figure 1).
2) Left renal hemiazygous pathway
Left renal vein has complex anatomy due
to the fact that many veins like inferior
phrenic, gonadal and adrenal veins drain
into it. Left renal vein communicates with
the retroperitoneal veins namely lumbar,
ascending lumbar and hemiazygous vein.
These communications ultimately reach
into the right atrium.
This drainage pathway can form the major
drainage pathway in the cases of BCS [5]
(Figure 2).
3) Vertebrolumbar azygous pathway
Flow in the IVC could reverse and reach common iliac
vein and then into the ascending lumbar veins thereby
draining into the azygous vein. Ascending lumbar veins
are seen parallel to the spine and poster lateral to the
aorta and IVC. Reversal of flow could be seen in the
gonadal or ureteric veins towards the common iliac vein
and then into the azygous system [2]. It is the most
common collateral pathway in BCS (Figure 3).
4) Anterior abdominal wall collaterals
• The collaterals comprising this system
include one between the superior and inferior
epigastric veins and other between the
superficial epigastric and lateral thoracic
• Inferior epigastric vein arises from the
external iliac vein joins superior epigastric
vein above the level of umbilicus to drain into
the subclavian vein and ultimately right heart.
• Flow may also follow the common femoral
vein through superficial circumflex iliac vein to
superficial epigastric vein and anastomosing
with the lateral thoracic vein.
• Collaterals comprising the superior and
inferior epigastric communications are seen
medially in contrast to the posterolaterally
situated superficial epigastric and lateral
thoracic veins. The laterally situated
superficial epigastric veins are seen clinically
as caput medusa [6] (Figure 4).
5) Inferior phrenic pericardio-
phrenic collaterals
• Pericardiophrenic vein is a
tributary of the left brachiocephalic
vein and has communications with
inferior phrenic vein. Inferior
phrenic vein on right side drains
into the IVC while on left side can
drain into the IVC and into the left
renal vein. Left hepatic vein and
left phrenic vein have ostia close
to each other. Sometimes left
phrenic vein can drain into the left
hepatic vein. This pathway drains
the intrahepatic collaterals into the
phrenic vein and then into the
pericardiophrenic vein (Figure 5).
Myeloproliferative Neoplasms
• (1) Polisitemia vera,
• (2) trombositopenia esensial,
• (3) Chronic myelomonocytic leukaemia (CMML),
• (4) Chronic neutrophilic leukaemia,
• (5) Chronic eosinophilic leukaemia,
• (6) Idiopathic myelofibrosis
Tabel 3. Well’s score
Interpretasi: Skor ≥2 adalah DVT likely

Kriteria klinis Skor Pada pasien

Active cancer (any treatment within past 6 months) +1 0

Calf swelling where affected calf circumference measures >3 cm more than the other calf +1 +1
(measured 10 cm below tibial tuberosity)
Prominent superficial veins (non-varicose) +1 0
Pitting oedema (confined to symptomatic leg) +1 +1
Swelling of entire leg +1 +1
Localised pain along distribution of deep venous system +1 0
Paralysis, paresis, or recent cast immobilisation of lower extremities +1 0
Recent bed rest for >3 days or major surgery requiring regional or general anaesthetic within past +1 +1
12 weeks
Previous history of DVT or PE +1 0
Alternative diagnosis at least as probable -2 0
What about CVP?
• (MAP – CVP) = CO x SVR

• Since CVP is usually much less than MAP, we may be able to ignore or
estimate the CVP value and still get clinically useful estimates for SVR.

• So, MAP (approximately) = CO x SVR.