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BUDD-CHIARI SYNDROME -
An Unusual Manifestation of Falciparum Malaria
Vrinda K Kulkarni*, Ds Asgaonkar**, Sa Kamath***
*Lecturer; **Associate Professor; ***Professor, Department of Medicine, BYL Nair Charitable Hospital, TN Medical College, Mumbai - 400 008.
Falciparum malaria can present with a vast spectrum of rare manifestations. Budd-Chiari syndrome (BCS) is the generic term for different forms of hepatic venous outflow obstruction resulting in a clinical picture of portal hypertension and hepatomegaly. We report a young female patient of falciparum malaria presenting as acute Budd-Chiari syndrome. Her abdominal ultrasound showed hepato-splenomegaly, free fluid and narrowed opening of the right hepatic vein into the inferior vena cava (IVC). Inferior vena cavogram revealed narrowing of the IVC in the intrahepatic course. Thus compression of the IVC caused by the hypertrophic caudate lobe in a case of falciparum malaria produced a clinical presentation of BCS. Patient was treated with antimalarials and she responded to the same. Repeat sonography and venous doppler done on follow-up were normal. Thus it was an unusual manifestation of falciparum malaria. Besides the uncommon mode of presentation, it was a reversible and medically treatable cause of BCS.INTRODUCTION
Budd-Chiari syndrome (BCS) is an uncommon form of portal hypertension caused by obstruction of the hepatic venous outflow.[1] Primary hepatic venous thrombosis (classical BCS) and an obliterative disease predominantly affecting the hepatic portion of the IVC account for most patients with venous outflow block.[2]
We had a young female patient with falciparum malaria presenting with high grade intermittent fever, jaundice, and pain in the right hypochondrium and abdominal distension.
Clinical picture and investigations were suggestive of Budd-Chiari syndrome. Patient responded to antimalarials and improved clinically. Repeat investigations were normal, suggesting a treatable cause i.e. malaria for Budd-Chiari syndrome.
CASE REPORT
A 21 year old female patient presented with high-grade intermittent fever with chills and rigors, pain in the right hypochondrium and epigastrium of seven days duration. She had h/o vomiting and watery loose motions for four days prior to the admission. Two days later she developed jaundice and abdominal distension. There was no h/o major illness of jaundice in the past. She did not have any addictions.
On examination, she was febrile. Vital parameters were stable. She had icterus. Abdomen was distended with the evidence of free fluid in abdomen. Liver as enlarged, tender and was palpable four cm below right costal margin in the mid clavicular line. Spleen could not be palpated.
Investigations were as follows - Hb 9.2 gm%, TC-5400/cmm, DC - P 72 L 28, ESR - 75 mm/hr. Peripheral smear was positive for ring forms of P. Falciparum. T. Bilirubin - 6.4 mg%, D. Bilirubin - 3.6 mg%, SGOT/SGPT - 66/56, T. Proteins - 4 gm%, alb - 1.8 gm%. PT/PI - 14 sec / 74.6%. Serum electrolytes, BUN and S. Creat. were normal. Urine routine showed sheets of pus cells and 8-10 RBCs. Widal test was negative. X-ray chest and ECG were within normal limits. USG abdomen showed massive hepatomegaly, mild splenomegaly, presence of free fluid and a narrowed opening of the right hepatic vein into the IVC. The middle and the left hepatic veins could not be seen. IVC was seen to enter normally into the right atrium. All these features on sonography suggested Budd-Chiari syndrome. IVC gram study revealed extrinsic compression of the intrahepatic portion of the IVC. The length of the narrowed segment was 6 cm (Fig. 1). There was no resistance in passing the catheter upto the right atrium ruling out the possibility of an IVC web.
Retrograde filling of hepatic veins was not seen and there were no collaterals. Thus there were features suggestive of exstrinsic compression of the IVC in the intrahepatic portion possibly due to hepatic enlargement.
Patient was treated with a course of antimalarials (chloroquine) and ciprofloxacin for urinary tract infection. She responded well to the above therapy within a week. She became afebrile. Peripheral smear was negative for malarial parasites. Her hepatomegaly regressed and LFT improved. Repeat abdominal ultrasound was normal. Venous Doppler done on follow-up was showing normal triphasic flow in the hepatic veins (Fig. 2) and normal flow and normal changes with respiration in the IVC (Fig. 3).
DISCUSSION
Budd-Chiari syndrome (BCS) was initially defined as a symptomatic occlusion of the hepatic veins.[2] Subsequent reports on various obliterative changes that occur in the hepatic portion of the IVC and hepatic vein orifices have resulted in a broadened and ambiguous definition. In the west, primary hepatic vein thrombosis giving rise to classical BCS is more common than obliterative disease predominantly affecting the hepatic portion of the IVC, which is seen in developing countries like India. 'Obliterative hepato-cavopathy' is the term suggested for the latter against classical BCS.[2]
Our patient had IVC compression possibly due to hepatic enlargement in falciparum malaria. Sowunmi A studied hepatomegaly in acute falciparum malaria in children and found that hepatomegaly was more common than splenomegaly.[3] He observed complete resolution of hepatomegaly in 41% of children after recovery from the acute illness which was also seen in our case. Thus caudate lobe enlargement in our patient caused the IVC compression and picture suggestive of Budd-Chiari syndrome. Medical therapy of the primary illness causing hepatomegaly resulted in reversal of compression. Severe intrahepatic compression of the IVC caused by the hypertrophic caudate lobe has been reported in the past.[4] There is a report of extrinsic compression of the intrahepatic IVC by intraparenchymal and/or subcapsular hepatic haematoma secondary to blunt hepatic trauma which was relieved by surgical drainage of haematoma.[5]
Ultrasound-Doppler being simple and noninvasive is recommended in the diagnosis of BCS.[6] We could diagnose and show the reversible nature of the disease with the help of ultrasonography and Doppler.
Hepatic venous outflow obstruction caused by hepatic vein thrombosis is a manifestation of hypercoaguable state. Underlying aetiologies can be myeloproliferative disorder, polycythaemia vera, essential thrombocytaemia, dysfibrinogenaemia, anti cardiolipin antibody syndrome and use of oral contraceptives.[7] BCS in association with falciparum malaria has not been reported so far. Sequestration in the hepatic microvasculature and increased coaguation cascade activity through intrinsic pathway activation have been proposed as likely mechanisms.[8] Antithrombin III depletion in severe falciparum malaria may produce a hypercoaguable state[8],[9] and hepatic vein thrombosis. Cytokine release is also procoagulant and may add to the mechanism of venous block.[10] Disseminated intravascular coagulation is important in the pathogenesis of severe malaria.[11],[12] Thus, coagulopathy can play a major role in producing a complication like Budd-Chiari syndrome in falciparum malaria.
This case was interesting, as the patient improved with medical therapy and the extrinsic compression of the IVC was reversible after the regression of hepatomegaly. The learning point here is that severe falciparum malaria can lead to a complication like acute Budd-Chiari syndrome, but with prompt medical therapy it can be treated successfully.
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