Budd Chiari Syndrome With Shunt Obstruction In Pregnancy

Author Information

Kumari P*, More V**, Panchbudhe S**, Satia MN***.

(* Second Year Resident, ** Assistant Professor, *** Professor, Department of Obstetrics and Gynecology, Seth GS Medical College & KEM Hospital, Mumbai, India)

Abstract

Budd Chiari syndrome is rare and serious condition characterised by obstruction to the outflow of hepatic venous blood. It may develop due to various causes such as thrombophilia’s, malignancies, infections and pregnancy. We present a case of pregnancy with decompensated Budd Chiari syndrome complicated with complete blockade of previous shunt  requiring re- shuntogram with shuntoplasty.

Introduction

Budd Chiari syndrome is one of the rarest causes of liver disease in pregnancy. It may be primary (75%) due to thrombosis of hepatic vein or secondary (25%) due to compression of hepatic vein by an outside structure like tumors. It is usually characterized by hepatomegaly, ascites and abdominal pain. Disease is mostly diagnosed in young females with increased estrogen stimuli due to pregnancy and use of oral contraceptive pills. Pregnancy may worsen Budd Chiari syndrome and these patients may present with complications like acute & sub-acute liver disease, fulminant liver disease, bleeding oesophageal varices, portal hypertension, hepatic encephalopathy or liver failure. We present a case of decompensated  (post shunt block) Budd Chiari syndrome in pregnancy.

Case Report

A 24 years old women, married since 6 years,  Para 1, living 1 with 13.6 weeks of gestation by date and Budd Chiari syndrome was referred for obstetric treatment from department of Gastroenterology. Her previous pregnancy was uneventful .She diagnosed as a case of Budd Chiari syndrome with hepatosplenomegaly 2 years after her first delivery.  She had undergone shunt gram 4 years ago which had shown thrombosis of all 3 hepatic veins and inferior vena cava. Patient gave history of Tran’s jugular intrahepatic Porto systemic shunt (TIPS) surgery done 3 years back. She was receiving Warfarin 10 mg PO qi. Now she had developed complete block of the shunt and the hepatic veins and inferior vena cava, as seen on the hepatoportal Doppler study. Her hemoglobin was 8.3gm%, WBC 7200/cmm, platelet count 82000/cmm, bilirubin normal, Aspartate transaminase/Alanine transaminase were 21 and 7 respectively , international normalized ratio 1.13 and renal function tests normal, serological test for hepatitis B and C were negative. On examination her vital parameters were stable; abdomen was soft with presence of ascites. Vaginal examination revealed uterus  12 weeks with bilateral fornices free and non-tender. She had been advised shunt gram with shuntoplasty by gastroenterologist. The patient and relatives were counseled regarding her decompensated condition and procedure related radiation exposure to the fetus. They opted for medical termination of pregnancy. However in view of her portal hypertension, the procedure could not be done prior to the treatment of hepatic venous obstruction .So she was managed by shunt gram followed by TIPS. She withstood the procedure well. She was then treated with parenteral iron sucrose 200 mg in 100 ml of normal saline on 2 occasions 48 hours apart for correction of anemia. Her warfarin to Heparin conversion was done. When her coagulation profile was in normal limits, second trimester termination of pregnancy was done with vaginal misoprostol. The total dose used was 800 microgram and induction- abortion interval was 10 hours. Patient aborted en sac, uterus was well contracted and there was no active bleeding per vaginum and hence did not require curettage. Subsequently she  was started on warfarin for anticoagulation. She was advised regulars follow up in outpatient department of Gastroenterology department with reports of prothrombin time/international normalized ratio.

Discussion

The Budd Chiari syndrome is rare condition resulting from obstruction to outflow of blood from liver. It may primary or secondary. The aetiology may be unknown in 67% of cases.^[1] The incidence of Budd Chiari syndrome in pregnancy is not known. The various secondary causes are haematological disorders like polycythaemia rubra vera, paroxysmal nocturnal hemoglobinuria, lupus anticoagulant, antithrombin III deficiency, myeloproliferative disorders and use of oral contraceptive pills. The relative risk of hepatic vein thrombosis in women using oral contraceptive pills is 2.37.^[2] But due to the availability of safer contraceptives pills with lower estrogen content, now oral contraceptive pills are no longer a major risk factor.^[3] The various sign and symptoms of decompensating states are deterioration of hepatic function, development of ascites, occurrence of bleeding events, thrombosis, recurrent post thrombotic events and increase risk of surgical interventions. In most of the cases there is a hypercoagulability due to underlying cause, as in our case it was pregnancy. ^[4] The risk among pregnant women is high due to alternation of coagulation factors leading to a hypercoagulable state (VII, VIII, X and fibrinogen) which often leads to blockade of shunts. Blockage of such a shunt is problem and results in recurrence of the disease. The management of such patient is difficult and literature is limited. There is still much controversy over pregnancy in patient with Budd Chiari syndrome because of high risk involved.^[5] There are few cases in the literature associated with pregnancy. In most of the cases maternal complication and pregnancy loss occurs. In 2009, a multicentre study was published in which from a total of 24 pregnancies, 16 were delivered but only 4 of them were full term pregnancies.^[6] So it has been proposed not to contraindicate pregnancy in well controlled and treated patients. However the routine use of anticoagulation does not prevent the occurrence of shunt dysfunctions.^[7] The treatment of Budd Chiari syndrome depends on underlying causes, anatomic location, and extent of thrombotic process and functional capacity of liver. It includes medical treatment like anticoagulation and thrombolysis. Doppler ultrasonography is the investigation of choice for the initial evaluation, and once the diagnosis is confirmed, anticoagulation is first line of management. Another treatment option is Tran’s jugular intrahepatic Porto systemic shunt (TIPS). Estimated fluoroscopy time for a TIPS procedure is 45 minutes and it exposes the fetus to a radiation dose of more than 5 rad.The accepted safe cumulative dose of ionizing radiation during pregnancy is 5 rad. The most radiosensitive period is between 8 weeks and 15 weeks of gestation. The sensitivity of developing fetus to radiation vary with gestational age, magnitude of dose, length of time of total exposure The fetus central nervous system is sensitive to effect of radiation during this period results in low IQ, stunted growth and severe mental retardation. Most of diagnostic procedure exposes the embryo to less than 50mSv (unit of radiation absorbed dose) which does not increases the risk of birth defect or miscarriage. To conclude, well controlled Budd Chiari Syndrome is not a contraindication for pregnancy and it involves a multidisciplinary team involving obstetrician, gastroenterologist, interventional radiologist and neonatologist for good maternal and fetal outcome but the patient should always be informed of all the existing risks and complications. Medical termination of pregnancy is justified in pregnant women with shunt block due to high risk of radiation exposure to the fetus.

References

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Citation

Kumari P, More V, Panchbudhe S, Satia MN. Budd Chiari Syndrome With Shunt Obstruction In Pregnancy. JPGO 2015. Volume 2 No. 1. Available from: http://www.jpgo.org/2015/01/budd-chiari-syndrome-with-shunt.html