Budd-Chiari syndrome following vaginal delivery in a patient with Crohn’s disease: a case report and review of the literature

Introduction

Budd-Chiari syndrome (BCS) might simply be defined as a hepatic venous outflow obstruction. The obstruction might be at the level of the hepatic venules, large hepatic veins, and inferior vena cava up to the confluence of the right atrium [7]. Presentation of the patient depends on the balance between the extent of the thrombosis and the rate of thrombolysis, and also on the efficacy of the collateral circulation and the development of liver fibrosis [7]. Therefore, acute and chronic forms of the syndrome exist.

Pregnancy-associated BCS is usually acute and fulminant. BCS in the postpartum period has been reported quite rarely in the literature [4–6, 13]. The main clinical presentation is sudden occurrence of abdominal pain and ascites following birth. The occurrence of the syndrome following delivery might be shortly afterwards or even 3–5 months later [3, 12]. The prognosis is generally poor, and a delay in diagnosis or treatment results in irreversible liver damage or a high death rate.

BCS and Crohn’s disease (CD) association is rare. Only two cases have been reported in the world medical literature [2, 8]. We present another case of a young multiparous woman with CD who developed BCS following an uneventful pregnancy and a normal vaginal delivery. The present case is unique in reporting the association of CD, BCS, and pregnancy.

Case

A 28-year-old woman, gravida 2 para 1, presented to another hospital 2 days following normal vaginal delivery with a fever of 42°C, weakness, dyspnea, and abdominal pain. Hepatosplenomegaly and ascites were detected on abdominal ultrasonography, and blood analysis showed a platelet count of 50×10³/μL. She was hospitalized with a possible diagnosis of sepsis, and antibiotherapy was started. However, over the course of her stay in that hospital for 2 days, the patient’s clinical condition did not improve and she was transferred to a university hospital for further evaluation. Her past medical history revealed CD diagnosed 1 year previously. She had no problem during the pregnancy related to CD and therefore discontinued medications. No disease activity occurred and the pregnancy was uneventful’ a 3100-g, healthy boy baby was delivered vaginally. No family or personal history of venous thrombosis was present.

On initial evaluation at the university hospital, the patient was pale and dyspneic, the abdomen was grossly distended and the sclera was icteric. Blood pressure was 90/60mm Hg, pulse was 78/min, and the temperature was 38.3°C. Laboratory tests showed the following results: hematocrit, 24%; hemoglobin, 8.7 g/dL; white blood cell count, 11.5×10³/μL (polymorphs 91%); platelet count, 45×10³/μL; serum glutamic oxaloacetic transaminase, 132 U/L; serum glutamic pyruvic transaminase, 42 U/L; alkaline phosphatase, 834 U/L; total bilirubin, 5.6 mg/dL; direct bilirubin, 3.5 mg/dL; lactate dehydrogenase, 321 U/L; serum total protein, 4.7 g/dL; albumin, 1.7 g/dL; blood urea, 40 mg/dL; creatinine, 0.9 mg/dL; prothrombin time, 15 s; activated partial thromboplastin time, 33.6 s. Serological test results for hepatitis virus A, B, and C were negative.

On transabdominal sonography, hepatosplenomegaly and ascites were noted. As the patient had fever and hepatosplenomegaly, consultation with the infectious diseases department was obtained. She was started on symptomatic treatment with ceftriaxon plus metronidazol, spironolactone, and furosemide. On color Doppler ultrasound and contrast-enhanced computed tomographic evaluation, a thrombus was detected in the 3-cm segment of suprahepatic inferior vena cava near the atrium causing significant obstruction and narrowing of the lumen of the inferior vena cava, hepatosplenomegaly, and total occlusion in the superior mesenteric vein (Figure 1). Upon this finding, the gastroenterology department was consulted. Upper gastrointestinal endoscopy was performed which revealed no esophageal varices. Paracentesis was not considered as the radiological scan indicated thrombus. She was diagnosed with BCS and started on anticoagulation. Despite the addition of imipenem to the antibiotherapy, fever continued until the initiation of anticoagulation. After low-molecular-weight heparin (enoxaparin, 2×60 mg/day) administration, the patient improved gradually with a decrease in temperature, ascitic fluid, and the intensity of dyspnea. Platelet count increased gradually as well and became normal 10 days after starting anticoagulation.

Figure 1

Axial contrast-enhanced digital computed tomography scan shows the thrombus obstructing the vascular lumen in between the inferior vena cava and the right atrium.

To further investigate the etiology of BCS, we performed evaluations of anticardiolipin antibody IgM and IgG; homocysteine; protein C, S, and antithrombin III levels; factor V Leiden; and prothrombin gene mutations, which were all within normal limits or negative.

She was discharged from hospital 25 days later. Permanent anticoagulation with warfarin and low-dose acetylsalicylic acid was continued. Result of follow-up color Doppler sonographic evaluation 2 months later was normal. It has now been 6 months since discharge, and the patient is currently doing well.

Discussion

Budd-Chiari syndrome (BCS) is a rare and serious thrombotic occlusion of the hepatic venous outflow involving the hepatic veins, inferior vena cava, or both with significant morbidity and even mortality. Hitherto, BCS has been reported to be found in 0.4–0.06% in autopsy material [1]. In the majority of patients with BCS, there is an underlying hypercoagulability state; however, in nearly 35% of cases the cause was not found [2]. This disorder should be suspected in any patient with acute, massive ascites. Herein, a woman with CD presented with acute BCS in the immediate postpartum period following an uneventful pregnancy. The classical triad of clinical manifestations in BCS includes hepatomegaly or liver function disturbances, right upper quadrant pain, and ascites. In our case, ascites, hepatosplenomegaly, elevated liver enzymes, and abdominal pain were all present.

Computed tomography, magnetic resonance imaging, and color Doppler studies are of value in demonstrating thrombosis of the hepatic veins or inferior vena cava [7]. However, in some complicated cases, angiography might become essential for a definitive diagnosis. In this case, color Doppler sonographic evaluation and computed tomography demonstrated a thrombus in the inferior vena cava.

Management of BCS includes medical treatment (anticoagulation), radiological intervention (stent application into the hepatic veins or vena cava, percutaneous balloon dilatation therapy for the stenosis), and surgical management (mesocaval/mesoatrial shunting, orthotopic liver transplantation). The therapeutic approach depends on the extent of the occlusion and also on the liver damage [10]. With prompt diagnosis, it might be possible to prevent progression of the disease before it involves the entire hepatic outflow tract, then the liver disease is completely reversible. A reason of mortality in BCS is delay in treatment. In the present case, clinical manifestations of BCS disappeared and the patient improved gradually with the initiation of anticoagulation. This might also be due to partial obstruction in the inferior vena cava. Complete obstruction of the inferior vena cava needs mesoatrial shunt application as the first-line method. Orthotopic liver transplantation with adjunctive anticoagulation may be considered when the disease causes severe hepatic failure [9].

Crohn’s disease complicated with BCS is extremely rare. There are only two cases describing the association of BCS and CD [2, 8]. Pathogenesis of hypercoagulability contributing to thromboembolic events in patients with inflammatory bowel diseases is not well known and is suggested to be multifactorial [15]. Although thrombosis is predominantly found in deep peripheral veins, involvement of the visceral veins has also been reported. Hepatic vein thrombosis might be the result of hypercoagulability during the exacerbation of the disease. Although patients with thromboembolic events had active or therapy-resistant CD in those reports, thromboembolism may also occur in a quiescent state [14]. In a retrospective review including 17 women with CD, the effect of the disease on pregnancy outcome and the course of the disease during pregnancy were evaluated [11]. The authors suggested that the active disease at the onset of pregnancy tended to remain active and that the quiescent disease tended to remain quiescent. In the present case, the patient was considered to be in remission from CD. Therefore, in this case, BCS might have been triggered by the hypercoagulability of puerperium. Patients with active disease should better be initiated with anticoagulant therapy; however, patients with quiescent disease should be informed about the symptoms and be observed closely for this complication both during the pregnancy and during puerperium.

BCS should be considered in the differential diagnosis of a woman who had hepatosplenomegaly and ascites in the postpartum period. Pregnancy and puerperium might further increase the risk of venous thrombosis in a woman with CD, potentiating the thrombogenic effect of CD.