Abstract
Among the important etiologies of pediatric portal hypertension, Budd–Chiari syndrome (BCS) is a potentially treatable cause. Pediatric BCS has distinctive differences as compared to adults in terms of etiology, natural history, and management. Predominant clustering in Asia and absence of a true underlying thrombophilia are unique issues in children. During the management of ascites and portal hypertension, an early and optimal therapeutic window is sought for radiological intervention which is the cornerstone of outcome. Endovascular management has challenges in children due to varying age, weight, and size of pediatric liver. Choice of procedure and intervention hardware (guidewires, balloons, and appropriate sized stents) needs to be customized accordingly. The overall vascular patency rates after radiological intervention are 87%, 82%, and 62% at 1, 5, and 10 years of follow-up in chronic BCS. Procedural complications are seen in 1–3%. Prior to endovascular intervention, pediatric end-stage liver disease (PELD) score <4 predicts good response to intervention. Zeitoun index >4.3 in unintervened chronic BCS children require an urgent radiological procedure. Intervention is also recommended in an asymptomatic BCS. Concerns in children are issues related to stents, number of procedures, dangers of lifelong anticoagulation, and searching for the underlying etiology.
Keywords
Budd–Chiari Children Pediatric Angioplasty Stenting Transjugular intrahepatic portosystemic shunt OutcomeReferences
- 1.Yachha SK. Portal hypertension in children: an Indian perspective. J Gastroenterol Hepatol. 2002;17:S228–31.CrossRefGoogle Scholar
- 2.Alam S, Khanna R, Mukund A. Clinical and prothrombotic profile of hepatic vein outflow tract obstruction. Indian J Pediatr. 2014;81:434–40.CrossRefGoogle Scholar
- 3.Valla D. Hepatic venous outflow tract obstruction etipathogenesis: Asia versus the West. J Gastroenterol Hepatol. 2004;19:S204–11.CrossRefGoogle Scholar
- 4.Dilawari JB, Bambery P, Chawla Y, Kaur U, Bhusnurmath SR, Malhotra HS, et al. Hepatic outflow obstruction (Budd–Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine (Baltimore). 1994;73:21–36.CrossRefGoogle Scholar
- 5.Amarapurkar DN, Punamiya SJ, Patel ND. Changing spectrum of Budd–Chiari syndrome in India with special reference to non-surgical treatment. World J Gastroenterol. 2008;14:278–85.CrossRefGoogle Scholar
- 6.Kathuria R, Srivastava A, Yachha SK, Poddar U, Baijal SS. Budd-Chiari syndrome in children: clinical features, percutaneous radiological intervention, and outcome. Eur J Gastroenterol Hepatol. 2014;26:1030–8.CrossRefGoogle Scholar
- 7.Sharma VK, Ranade PR, Marar S, Nabi F, Nagral A. Long-term clinical outcome of Budd-Chiari syndrome in children after radiological intervention. Eur J Gastroenterol Hepatol. 2016;28:567–75.CrossRefGoogle Scholar
- 8.Singh SK, Sen Sarma M, Yadav R, Kumar S, Prasad R, Yachha SK, Srivastava A, Poddar U. Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd-Chiari syndrome in children. Liver Int. 2018;38:1308–15.CrossRefGoogle Scholar
- 9.Shukla A, Bhatt P, Gupta DK, Modi T, Patel J, Gupte A, Meshram M, Bhatia S. Budd-Chiari syndrome has different presentations and disease severity during adolescence. Hepatol Int. 2018;12:560–6.CrossRefGoogle Scholar
- 10.Meyers RL, Maibach R, Hiyama E, Häberle B, Krailo M, Rangaswami A, et al. Risk-stratified staging in paediatric hepatoblastoma: a unified analysis from the Children’s Hepatic tumors International Collaboration. Lancet Oncol. 2017;18:122–31.CrossRefGoogle Scholar
- 11.Karadag O, Akinci D, Aksoy DY, Bayraktar Y. Acute Budd-Chiari syndrome resulting from a pyogenic liver abscess. Hepatogastroenterology. 2005;52:1554–6.PubMedGoogle Scholar
- 12.Mehrotra G, Singh RP, Krishna A, Singh BK. Pyogenic liver abscess causing acute Budd-Chiari syndrome. Ann Trop Paediatr. 1992;12:451–3.CrossRefGoogle Scholar
- 13.Valla DC. Budd-Chiari syndrome/hepatic venous outflow tract obstruction. Hepatol Int. 2018;12:168–80.CrossRefGoogle Scholar
- 14.Nagral A, Hasija RP, Marar S, et al. Budd-Chiari syndrome in children: experience with therapeutic radiological intervention. J Pediatr Gastroenterol Nutr. 2010;50:74–8.CrossRefGoogle Scholar
- 15.Nobre S, Khanna R, Bab N, Kyrana E, Height S, Karani J, Kane P, Heaton N, Dhawan A. Primary Budd-Chiari syndrome in children: King’s College Hospital Experience. J Pediatr Gastroenterol Nutr. 2017;65:93–6.CrossRefGoogle Scholar
- 16.Squires RH Jr, Shneider BL, Bucuvalas J, Alonso E, Sokol RJ, Narkewicz MR, et al. Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group. J Pediatr. 2006;148:652–8.CrossRefGoogle Scholar
- 17.Sen Sarma M, Yachha SK, Bhatia V, Srivastava A, Poddar U. Safety, complications and outcome of large volume paracentesis with or without albumin therapy in children with severe ascites due to liver disease. J Hepatol. 2015;63:1126–32.CrossRefGoogle Scholar
- 18.Seijo S, Plessier A, Hoekstra J, Dell’era A, Mandair D, Rifai K, et al. European Network for Vascular Disorders of the Liver. Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology. 2013;57:1962–8.CrossRefGoogle Scholar
- 19.Mukund A, Gamanagatti S, Acharya SK. Radiological interventions in HVOTO--practical tips. Trop Gastroenterol. 2011;32:4–14.PubMedGoogle Scholar
- 20.Mancuso A. Management of Budd-Chiari syndrome in children: same debated issues as in adults. Eur J Gastroenterol Hepatol. 2015;27:107–8.CrossRefGoogle Scholar
- 21.Qi X, Han G, Guo X, De Stefano V, Xu K, Lu Z, et al. Review article: the aetiology of primary Budd-Chiari syndrome - differences between the West and China. Aliment Pharmacol Ther. 2016;44:1152–67.CrossRefGoogle Scholar