39. Budd-Chiari syndrome in children – outcome following radiological intervention

Abstract

Background and Aims: Hepatic vein outflow tract obstruction (HVOTO) or Budd-Chiari syndrome is a vascular disorder of liver with occlusion of hepatic veins with or without inferior vena cava (IVC). HVOTO in children is described predominantly in the Asian children. There is limited data on clinical profile and outcome of these cases. We aim to describe the etiological spectrum, clinical profile and outcome of children with HVOTO. Methods: From June 2010 to April 2018, all children under-18 years of age with HVOTO were enrolled. Diagnosis was based on ultrasound Doppler with or without contrast enhanced CT. Results: There were 36 children (25 males, 69%). Median age of presentation and duration of symptoms were 10 years and 12 months, respectively. Abdominal distension (85%) and pain (58%) were the commonest symptoms. Seven (19%) presented with predominant portal hypertension in the form of variceal bleeding while 6 (17%) had encephalopathy. Common findings at admission were ascites (69%), edema (47%) and jaundice (42%). Median Child Pugh, PELD and MELD scores at admission were 8, 8 and 13, respectively. Radiologically, there was isolated involvement of all 3 hepatic veins in 30 (85%), 5 (15%) had associated IVC occlusion; only 1 patient had isolated IVC block. Inherited prothrombotic mutations were found in 9 out of 22 children – MTHFR (6 heterozygous, 1 homozygous), 1 factor-V Leiden and janus kinase (JAK)-2 mutation in 1. Two children died before intervention due to acute kidney injury and multiorgan dysfunction. Radiological interventions were performed in 23 children-direct intrahepatic portosystemic shunt (DIPSS) (n = 8), transjugular intrahepatic portosystemic shunt (TIPSS)(n = 4), and angioplasty of right hepatic vein (RHV) (n = 6), left hepatic vein (LHV) (n = 1) or IVC (n = 4). Two patients had reversal of fulminant presentation following a successful intervention (DIPSS 1, LHV angioplasty 1). There were 9 post procedure complications seen in 7 children – congestive cardiac failure, bleeding (hemoperitoneum 2, hemobilia 1), encephalopathy, stent infection, sepsis, liver dysfunction in 3, 3, 1, 1 and 1 child, respectively. One child died of hemoperitoneum, had liver dysfunction post-DIPSS. Immediate success was seen in 21 – however, 5 (24%) required revision of stent due to stent block (4 DIPSS and 1 LHV angioplasty), 2 were related to non-compliance to anti-coagulants. Eleven children were managed conservatively in view of development of good collateral circulation (n = 9) or spontaneous recanalization (n = 1) and refusal (n = 1). Combined pre and post procedure mortality rate of 8.3% (3/36) was seen in this cohort. Conclusions: HVOTO presents as acute or chronic liver dysfunction with features of portal hypertension. Hepatic vein involvement is the commonest site of obstruction. Establishment of porto-systemic blood flow is the treatment of choice. Radiological therapeutic interventions are feasible, safe and effective in children with HVOTO even with fulminant presentation. Post procedure recurrence is seen in one-fourth. The authors have none to declare.