Budd Chiari Syndrome Modified

8/3/2019 Budd Chiari Syndrome Modified

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Case PresentationBudd Chiari Syndrome

Department of Gastroenterology

By

Dr Nargis Tasleem

PG Medicine


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Presenting Complaint 30 years old female with no co morbidity.

Presented with progressively increasing

abdominal distension for 09 months.


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HOPI Patient delivered a healthy baby boy by

SVD 11 months back. After delivery she

noticed that her abdominal distension wasnot relieved rather it increased.


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HOPI She also had nausea ,vomiting, decreased

appetite, weight loss(10 to 15 kg),

abdominal pain RUQ & discomfortassociated with it.


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ROS There was complain of low grade fever off

& on.

History of loose stools, decrease urinary

frequency, altered consciousness one week

before presentation to SIH.


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Social History Non smoker, Non addict, married woman with three

kids. FA pass. Worked as road hostess in Daewoo.

Husband is carpenter, belongs to lower middle class.


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Medications History of Diuretics use.

History of ATT for three months as a

suspicion for TB.


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Examination A weak, lethargic looking young female

with massive ascites.

Haemodynamically stable.

GPE: no remarkable finding.

Chest: B/L clear. Prominent veins on chest.

CVS: unremarkable.

Abdomen: Massive ascites, striae, dilated

veins, bulging umbilicus.


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Continued

CNS: oriented ,alert ,no focal deficit.

Pedal edema + + + B/L.


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Assessment

Ascites secondary to

Liver Disease?

Malignancy ?

Abdominal TB ?


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Radiology

USG Abdomen:

Coarse hepatic parenchymal echotexture

with irregular margins, thick walled gall

bladder and moderate abdominopelvic

ascites, features are suggestive of CLD.

Mild Hepatomegaly.

Sludge Gall bladder.


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Radiology

Abdominal Doppler(Hepatic & Portal

Vein)

Coarse ,nodular echotexture of liver,

hypertrophy of caudate lobe, splenomegaly

and ascites.

Portal vein is patent with normal flow.

Right & Middle hepatic veins are not

visualized.


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Radiology

CT Liver Dynamic Study

Findings are suggestive of extensive Budd

Chiari Syndrome with large ascites with

extensive collateral formation and

recanalization of umbilical vein.


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Diagnosis

Post-partum thrombosis of hepatic veins

leading to Budd Chiari Syndrome with

massive ascites but relatively preservedliver synthetic functions.


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Plan

Hepatobiliary surgeon planned her for Porto

systemic shunt.

Hematology advice was to send

hypercoaguable workup after surgery and

few months of anticoagulation.


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Surgery

She was taken to theatre. About 15 liters of

straw-coloured ascites was drained. Her

liver was congested with somemicronodularity. Caudate lobe was

hypertrophied.

A mesocaval shunt was not possible due tothe small size of SMV.


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Surgery(27-04-11)


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Surgery

A portocaval shunt with 10mm PTFE ringed

graft was done.

Portal pressure before the shunt was 32, and

IVC pressure was 7.

After porto-caval shunt the portal pressure

was 13 and IVC pressure was 7


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Post op clinical course

Her post-op recovery was complicated by

pulmonary edema and mild renal

insufficiency on 7th post op day, which was

managed medically.

She continued to pour large volumes ofascitic fluid in her drains for some time.


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Histopathology(cavernous

transformation of sinuses)


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Dilated & Congested sinuses


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Her follow up visits showed improvement

in her general condition and liver functions.

She is coming back to health, eating well,

gaining weight, visiting OPD clinic with a

smile full of life.

Though ascites is still there but notrequiring any more paracentesis.

Follow up


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Literature review

Budd Chiari Syndrome:

Includes hepatic venous outflow obstruction

at any level from the small hepatic veins tothe junction of the inferior vena cava andthe right atrium, regardless of the cause ofthe obstruction.

Cardiac and pericardial diseases, andsinusoidal obstruction syndrome areexcluded from this definition.


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Etiology

Hypercoagulable state

Antiphospholipid syndrome

Lupus anticoagulantFactor V Leiden mutation and prothrombin mutation

Myeloproliferative disorder

PNH

Protein C deficiency, Protein S deficiency,

Antithrombin Deficiency

Postpartum thrombocytopenic purpura, Pregnancy, Oral

Contraceptives


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Etiology

Hematologic disorders are the most

common cause for BCS.

Primary myeloproliferative disease,

particularly polycythemia rubra vera, may

account for 50% of cases.

Tumors, infections, and pregnancy eachaccount for 10% of cases.


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Classical triad of symptoms is abdominal

pain RUQ, ascites, and hepatomegaly.

Clinical Features


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Normal Histology


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Diagnosis

Colour and pulsed Doppler ultrasonography(Sensitivity and specificity of >85%) is the

first-line investigation for diagnosis.

Patients should also undergo testing for

hypercoagulable states to identify any

predisposition to venous thrombosis.


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Treatment The treatment options are

Radiological interventional procedures

Angioplasty of hepatic vein and/or inferior

vena cava.

Transjugular intrahepatic portosystemic

shunts (TIPS)


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Treatment

Surgical interventional procedures such as:

Surgical shunts.

Liver transplantation.


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Treatment

Medical treatment in the form of

Anticoagulation therapy.

Diuretics.

Treatment of underlying thrombophilic condition.

is given parallel to interventional therapy.


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Prognosis

Untreated, the syndrome has a reported

mortality of 80%. In the past few decades,

prognosis has dramatically improved due toadvances and new modalities in diagnosis

and treatment of the disease.


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DISCUSSION


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Budd Chiari Syndrome Modified

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