Grand Rounds

A child with Budd Chiari syndrome and recurrent thrombosis


Suhani Jain1, Ira Shah2
1Grant Government Medical College, Sir JJ Group of Hospitals, Mumbai, India, 2Consultant in Pediatric Infectious Diseases, Levioza Health Care, Mumbai, India

Address for Correspondence: Suhani Jain, Flat number 402, Ramdeo Arise, Behind Hotel Airport Centre Pt, Wardha Road, Nagpur-440025. Email: suhani2208@gmail.com


Keywords: Budd Chiari Syndrome, Thrombosis, Coagulopathy

Clinical Problem:
A 10-month-old girl presented with ascites. At 3 months of age, she had ascites and was diagnosed to have Budd Chiari syndrome (BCS) due to acute thrombosis of Middle and Right Hepatic veins (MHV and RHV). At the age of 3 months, coagulation studies showed decreased protein C activity, decreased Antithrombin III activity and increased serum homocysteine levels. Subsequently, she was started on injectable low molecular weight heparin, spironolactone, folic acid. She was also started on propranolol as ultrasound doppler of abdomen and portal system showed signs of portal hypertension. At 7 months of age she underwent venoplasty of Middle and Right Hepatic veins (MHV and RHV) and anticoagulation was continued with LMWH. Despite this, she had 2 hospital admissions for acute liver decompensation (ascites). She also developed gangrene of left middle finger at 8 months of age post venoplasty that auto-amputated inspite of being on LMWH. Despite being on treatment with diuretics, LMWH, propranolol, liver dysfunction continues. Current ultrasound of abdomen and doppler showed no demonstrable flow in hepatic veins, low velocity flow in inferior vena cava (IVC) along with liver cirrhosis and portal hypertension. INR was 2 with prothrombin time 24.2 sec (control 12 sec); partial thromboplastin time of 49.4 sec (control 28 sec). She underwent a repeat venoplasty of RHV and MHV. However, she continues to have persistent ascites post-venoplasty (diuretic resistant) and requires repeated ascitic tap. A repeat doppler after 1 week of venoplasty again showed that the MHV flow was blocked. She has had growth failure and her weight remained 6 kg in spite of all nutritional rehabilitation. Her albumin was 2.5 gm/dl and bilirubin was normal.

What is the cause of recurrent thrombosis in this child?


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