A case series of Budd-Chiari syndrome in pregnancy undergoing elective caesarean section: Anaesthetic considerations

INTRODUCTION

Budd–Chiari syndrome (BCS) is a rare hepatic disorder resulting from obstruction of hepatic venous outflow, leading to portal hypertension and liver dysfunction. The obstruction may involve the small hepatic veins, inferior vena cava (IVC), or both. Its estimated prevalence in Western countries is approximately 1 in 2.5 million people, but the incidence is higher in Asian regions, including India, China, and Nepal.^[1]

BCS management typically follows a stepwise approach, starting with anticoagulation therapy and progressing to interventional procedures such as thrombectomy, percutaneous angioplasty, or transjugular intrahepatic portosystemic shunt (TIPS), with liver transplantation reserved for refractory cases.^[2]

Pregnancy in women with BCS is uncommon and often complicated by hypercoagulability and hepatic congestion. We describe three cases of parturients with known BCS undergoing elective caesarean section, focusing on anaesthetic considerations and perioperative multidisciplinary coordination.

CASE SERIES

Case 1

A 24-year-old woman (gravida 2 para 1 living 1) with known BCS was on aspirin 75 mg and subcutaneous enoxaparin 60 mg daily throughout pregnancy, discontinued 24 hours before elective lower segment caeserean section (LSCS). Preoperative evaluation revealed mild hepatosplenomegaly and dilated abdominal veins [Figure 1]. Platelet count was 1.6 × 10⁵/cmm with a normal coagulation profile. Liver enzymes were within normal limits. High-risk consent was obtained considering potential blood loss.

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Figure 1:

Dilated abdominal veins

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Spinal anaesthesia was administered at the L3–L4 level using 2 ml of 0.5% hyperbaric bupivacaine with 10 µg fentanyl, achieving a T6 sensory block. A healthy neonate was delivered; estimated blood loss was 1000 mL. The intraoperative period was uneventful. Enoxaparin was restarted 12 hours postoperatively, and the patient recovered without complications. Follow-up was kept till 7 postoperative days, and the course was uneventful for both mother and baby.

DISCUSSION

Hepatobiliary diseases can pose great challenges for anaesthesiologists. Pregnancy-related BCS is well known and may manifest with symptoms such as abdominal pain, ascites, and hepatomegaly.^[3] Pregnancy itself is a hypercoagulable state, and it is accompanied by several physiological changes, like an increase in blood volume, development of hypoproteinaemia, rise in intra-abdominal pressure, the pressure exerted by the gravid uterus on the IVC and other abdominal vessels. The growing uterus displaces various intra-abdominal organs, resulting in changes in normal anatomical positioning and relationships.^[4] Therapeutic anticoagulation is the cornerstone of BCS management. During pregnancy, warfarin therapy should be transitioned to therapeutic doses of unfractionated heparin or LMWH within 6 weeks of conception and can be resumed postpartum to therapeutic doses of unfractionated heparin or LMWH within 6 weeks of becoming pregnant and can then resume warfarin postpartum.^[5] Warfarin can cross the placenta, leading to congenital abnormalities called foetal warfarin syndrome.^[6] There is a risk of spinal hematoma in patients undergoing anticoagulation therapy for neuraxial blockade. So, the decision to administer neuraxial or general anaesthesia should be made after weighing the risk-benefit ratio and individualised approach.^[7]

When general anaesthesia is indicated, special attention should be given to airway management. Pregnancy itself increases the risk of a difficult airway, and any traumatic intubation can precipitate airway bleeding.^[8] A gentle, well-planned induction is essential. Drugs with minimal hepatic metabolism and limited effects on hepatic blood flow, such as propofol- are preferred as they also preserve uterine tone during induction.^[9] Neuromuscular blockade, like atracurium or cisatracurium, can be used as its metabolism and elimination do not depend on the liver. Maternal mortality may occur due to liver decompensation, bleeding varices, intraoperative and postoperative haemorrhage, pulmonary embolism, and deep vein thrombosis. The surgery duration should be minimised to reduce postoperative complications due to increased bleeding caused by low platelets, which is also a contributing factor.^[10]

Postoperatively, intensive monitoring in an intensive care unit setting is recommended. Intravenous fluids should be titrated carefully to prevent dehydration–induced hypercoagulability, which may precipitate cerebral cortical venous thrombosis or deep vein thrombosis.^[11] It is recommended to begin early ambulation and start administering therapeutic injection LMWH after consulting with an expert.^[12] A multidisciplinary team with good monitoring can enhance maternal and foetal well-being in patients with BCS.

CONCLUSION

Pregnancy complicated by BCS presents a complex interplay of hepatic, haematologic, and obstetric risks. An individualised, multidisciplinary approach, including careful perioperative planning, coordination of anticoagulation, and vigilant monitoring, optimises outcomes. Elective caesarean delivery under a planned anaesthetic strategy in a tertiary care setting ensures safer outcomes for both mother and neonate.