Case Presentation

Budd Chiari Syndrome Department of Gastroenterology

By Dr Nargis Tasleem PG Medicine

Presenting Complaint

30 years old female with no co morbidity. Presented with progressively increasing abdominal distension for 09 months.

HOPI

Patient delivered a healthy baby boy by SVD 11 months back. After delivery she noticed that her abdominal distension was not relieved rather it increased.

HOPI

She also had nausea ,vomiting, decreased appetite, weight loss(10 to 15 kg), abdominal pain RUQ & discomfort associated with it.

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.

Past Medical & Surgical History

Multiple visits to different doctors for abdominal distension. History of large volume paracentesis multiple times. Took ATT for ascites for 3 months with no benefit. History of tonsillectomy at age of 12 years

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.

Medications

History of Diuretics use. History of ATT for three months as a suspicion for TB.

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.

Continued

CNS: oriented ,alert ,no focal deficit. Pedal edema + + + B/L.

Assessment

Ascites secondary to

Liver Disease? Malignancy ? Abdominal TB ?

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.

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.

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.

Diagnosis

Post-partum thrombosis of hepatic veins leading to Budd Chiari Syndrome with massive ascites but relatively preserved liver synthetic functions.

Plan

Hepatobiliary surgeon planned her for Porto systemic shunt. Hematology advice was to send hypercoaguable workup after surgery and few months of anticoagulation.

Surgery

She was taken to theatre. About 15 liters of straw-coloured ascites was drained. Her liver was congested with some micronodularity. Caudate lobe was hypertrophied. A mesocaval shunt was not possible due to the small size of SMV.

Surgery(27-04-11)

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

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 of ascitic fluid in her drains for some time.

Histopathology(cavernous transformation of sinuses)

Dilated & Congested sinuses

Follow up

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 not requiring any more paracentesis.

Literature review

Budd Chiari Syndrome: Includes hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava and the right atrium, regardless of the cause of the obstruction. Cardiac and pericardial diseases, and sinusoidal obstruction syndrome are excluded from this definition.

Etiology

Hypercoagulable state
Antiphospholipid syndrome Lupus anticoagulant Factor V Leiden mutation and prothrombin mutation Myeloproliferative disorder PNH Protein C deficiency, Protein S deficiency, Antithrombin Deficiency Postpartum thrombocytopenic purpura, Pregnancy, Oral Contraceptives

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 each account for 10% of cases.

Clinical Features

Classical triad of symptoms is abdominal pain RUQ, ascites, and hepatomegaly.

Normal Histology

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.

Treatment

The treatment options are Radiological interventional procedures

Angioplasty of hepatic vein and/or inferior vena cava. Transjugular intrahepatic portosystemic shunts (TIPS)

Treatment

Surgical interventional procedures such as:

Surgical shunts. Liver transplantation.

Treatment

Medical treatment in the form of

Anticoagulation therapy. Diuretics. Treatment of underlying thrombophilic condition. is given parallel to interventional therapy.

Prognosis

Untreated, the syndrome has a reported mortality of 80%. In the past few decades, prognosis has dramatically improved due to advances and new modalities in diagnosis and treatment of the disease.

DISCUSSION