Successful treatment of Budd-Chiari Syndrome with Rivaroxaban in a six week old Bangladeshi infant
Abstract
Budd-Chiari syndrome (BCS) is the clinical spectrum presenting with hepatic venous outflow obstruction from the hepatic veins to the junction of the inferior vena cava (IVC) and the right atrium. This syndrome is rare in infants and children, which leads to misdiagnoses or delayed diagnoses. Clinical presentations may be non-specific. A high index of suspicion and imaging findings may help in early diagnosis of this condition. We report a rare case of BCS in a 6-week-old male infant who presented with jaundice and gradual abdominal distension. He was successfully treated with rivaroxaban.
References
2. Horton JD, San Miguel FL, Membreno F, Wright F, Paima J, Foster P, et al. Budd–Chiari syndrome: illustrated review of current management. Liver Int. 2008;28:455-6.
DOI: 10.1111/j.1478-3231.2008.01684.x
3. Rajani R, Melin T, Björnsson E, Broomé U, Sangfelt P, Danielsson Å, et al. Budd?Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival–an 18?year experience. Liver Int. 2009;29:253-9. DOI: 10.1111/j.1478-3231.2008.01838.x.
4. Okuda K, Kage M, Shrestha SM. Proposal of a new nomenclature for Budd–Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology. 1998;28:1191-1198. DOI: 10.1002/hep.510280505.
5. Valla DC. Hepatic vein thrombosis (Budd-Chiari syndrome). Semin Liver Dis. 2002;22:5-14. DOI: 10.1055/s-2002-23202.
6. Ferral H, Behrens G, Lopera J. Budd-Chiari syndrome. AJR Am J Roentgenol. 2012;199:737-45. DOI: 10.2214/AJR.12.9098.
7. 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. DOI: 10.1097/00005792-199401000-00003.
8. Gentil-Kocher S, Bernard O, Brunelle F, Hadchouel M, Maillard JN, Valayer J, et al. Budd-Chiari syndrome in children: a report of 22 cases. J Pediatr. 1988;113:30-8. DOI: 10.1016/S0022-3476(88)80524-9.
9. Hoffman HD, Stockland B, von der Heyden U. Membranous obstruction of the inferior vena cava with the Budd-Chiari syndrome in children: a report of nine cases. J Pediatr Gastroenterol Nutr. 1987;6:878-84. DOI: 10.1097/00005176-198711000-00010.
10. Kathuriaa R, Srivastavaa A, Yachhaa SK, Poddara U, Baijalb SS. Budd–Chiari syndrome in children: clinical features, percutaneous radiological intervention, and outcome. Eur J Gastroenterol Hepatol. 2014;26:1030-8. DOI: 10.1097/MEG.0000000000000144.
11. Bolondi L, Gaiani S, Li Bassi S, Zironi G, Bonino F, Brunetto M, et al. Diagnosis of Budd–Chiari syndrome by pulsed Doppler ultrasound. Gastroenterology. 1991;100:1324-31. DOI: 10.1016/0016-5085(91)70020-X.
12. Boozari B, Bahr MJ, Kubicka S, Klempnauer J, Manns MP, Gebel M. Ultrasonography in patients with Budd–Chiari syndrome: diagnostic signs and prognostic implications. J Hepatol. 2008;49:572-80. DOI: 10.1016/j.jhep.2008.04.025.
13. Misra V, Verma K, Singh DK, Misra SP. The Budd-Chiari syndrome in a child: a case report and review of the literature. J Clin Diagn Res. 2012;6:1783-5. DOI: 10.7860/JCDR/2012/4525.2613.
14. Sherlock S, Dooley J. Diseases of the liver and biliary system. 11th ed. Oxford: Blackwell Publishing; 2002. p. 192-8.
15. Nagral A, Hasija R, Marar S, Nabi F. Budd–Chiari syndrome in children: experience with therapeutic radiological intervention. J Pediatr Gastroenterol Nutr. 2010;50:74-8. DOI: 10.1097/MPG.0b013e3181aecb63.
16. Pedersen MR, Molloy P, Wood D, Seetharam A. Direct intrahepatic portocaval shunt for treatment of portal thrombosis and Budd-Chiari syndrome. Ann Hepatol. 2016;15:127-30. DOI: 10.5604/16652681.1184288.
17. Beyer-Westendorf J, Förster K, Pannach S, Ebertz F, Gelbricht V, Thieme C, et al. Rates, management, and outcome of rivaroxaban bleeding in daily care: results from the Dresden NOAC registry. Blood. 2014;124:955-62. DOI: 10.1182/blood-2014-03-563577.
18. Murad SD, Valla DC, de Groen PC, Zeitoun G, Hopmans JAM, Haagsma EB, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd–Chiari syndrome. Hepatology. 2004;39:500-8. DOI: 10.1002/hep.20064.
Copyright (c) 2022 Mukesh Khadga, Ferdous Ara Begum, Mohammad Wahiduzzaman Mazumder, Mohammad Benzamin, Nazmul Ahamed Rony, AZM Raihanur Rahman, Ayesha Siddiqua, Nazmul Hassan, ASM Bazlul Karim
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Authors who publish with this journal agree to the following terms:
Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Accepted 2022-03-04
Published 2022-03-04
