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

. decrease urinary frequency. History of loose stools.ROS   There was complain of low grade fever off & on. altered consciousness one week before presentation to SIH.

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

married woman with three kids. Worked as road hostess in Daewoo. Non addict. FA pass. belongs to lower middle class.Social History  Non smoker. Husband is carpenter. .

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

Abdomen: Massive ascites. lethargic looking young female with massive ascites. Haemodynamically stable. GPE: no remarkable finding. Chest: B/L clear. Prominent veins on chest. . striae. dilated veins. bulging umbilicus. CVS: unremarkable.Examination       A weak.

.no focal deficit. Pedal edema + + + B/L.alert .Continued……   CNS: oriented .

Assessment  Ascites secondary to    Liver Disease? Malignancy ? Abdominal TB ? .

Radiology     USG Abdomen: Coarse hepatic parenchymal echotexture with irregular margins. Mild Hepatomegaly. thick walled gall bladder and moderate abdominopelvic ascites. . Sludge Gall bladder. features are suggestive of CLD.

Right & Middle hepatic veins are not visualized. . hypertrophy of caudate lobe. splenomegaly and ascites.Radiology     Abdominal Doppler(Hepatic & Portal Vein) Coarse .nodular echotexture of liver. Portal vein is patent with normal flow.

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 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.

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

Surgery(27-04-11) .

After porto-caval shunt the portal pressure was 13 and IVC pressure was 7 . Portal pressure before the shunt was 32. and IVC pressure was 7.Surgery    A portocaval shunt with 10mm PTFE ringed graft was done.

Post op clinical course   Her post-op recovery was complicated by pulmonary edema and mild renal insufficiency on 7th post op day. She continued to pour large volumes of ascitic fluid in her drains for some time. which was managed medically. .

Histopathology(cavernous transformation of sinuses) .

Dilated & Congested sinuses .

She is coming back to health.Follow up    Her follow up visits showed improvement in her general condition and liver functions. visiting OPD clinic with a smile full of life. gaining weight. eating well. . Though ascites is still there but not requiring any more paracentesis.

. and sinusoidal obstruction syndrome are excluded from this definition. regardless of the cause of the obstruction. Cardiac and pericardial diseases.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.

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

Primary myeloproliferative disease. particularly polycythemia rubra vera. infections. may account for 50% of cases. . Tumors.Etiology    Hematologic disorders are the most common cause for BCS. and pregnancy each account for 10% of cases.

and hepatomegaly.Clinical Features  Classical triad of symptoms is abdominal pain RUQ. . ascites.

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

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

DISCUSSION .

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