Download PDF
Eur J Pediatr Surg 2018; 28(05): 439-444
DOI: 10.1055/s-0037-1603987
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Variceal Bleeds in Patients with Biliary Atresia

Daan B. van Wessel
1   Department of Pediatric Surgery, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
,
Mauri Witt
1   Department of Pediatric Surgery, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
,
Niels Bax
1   Department of Pediatric Surgery, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
,
Henkjan J. Verkade
2   Department of Pediatric Gastroenterology and Hepatology, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
,
René Scheenstra
2   Department of Pediatric Gastroenterology and Hepatology, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
,
Ruben H. de Kleine
3   Department of Hepato-Pancreatico-Biliary Surgery and Liver Transplantation, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
,
Jan B. Hulscher
1   Department of Pediatric Surgery, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands
› Author Affiliations
Further Information

Publication History

23 February 2017

29 April 2017

Publication Date:
28 June 2017 (online)

    Permissions and Reprints

Abstract

Introduction Portal hypertension often occurs in biliary atresia (BA). The subsequent development of esophageal varices and bleeding from these varices are a well-known complication. We aim to describe the incidence and severity of variceal bleeding in patients with BA. In addition, we describe the characteristics of patients who experienced variceal bleeds.

Materials and Methods We included all infants treated for BA at our center between March 1987 and August 2015. Variceal bleeding was defined as hematemesis and/or melena with presence of varices at endoscopy. Findings at endoscopy and ultrasound, laboratory tests, clearance of jaundice, fibrosis-grade at Kasai portoenterostomy, and several varices prediction scores were documented. Routine endoscopies were not performed.

Results In this study, 74 patients were included. During follow-up, 18 out of 74 patients (24%) developed variceal bleeding at an age of 9 months (range, 4–111). Twelve patients were listed for liver transplantation at the time of bleeding. Patients who did not clear their jaundice developed variceal bleeds more often and earlier in life. Bleeds were treated with sclerotherapy, banding, or octreotide. Four patients did not receive treatment. No bleeding-related mortality occurred.

Conclusion One-fourth of the children diagnosed with BA experience variceal bleeds during follow-up. Most of these children are younger than 1 year and often already listed for transplantation. Major complications did not occur after variceal bleeding.

Keywords

biliary atresia - variceal bleeds - portal hypertension - endoscopy
 

  • References

  • 1 Davenport M, Ong E, Sharif K. , et al. Biliary atresia in England and Wales: results of centralization and new benchmark. J Pediatr Surg 2011; 46 (09) 1689-1694
    CrossrefPubMedGoogle Scholar
  • 2 Lampela H, Ritvanen A, Kosola S. , et al. National centralization of biliary atresia care to an assigned multidisciplinary team provides high-quality outcomes. Scand J Gastroenterol 2012; 47 (01) 99-107
    CrossrefPubMedGoogle Scholar
  • 3 Nio M, Ohi R, Miyano T, Saeki M, Shiraki K, Tanaka K. ; Japanese Biliary Atresia Registry. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg 2003; 38 (07) 997-1000
    CrossrefPubMedGoogle Scholar
  • 4 de Vries W, de Langen ZJ, Groen H. , et al; Netherlands Study Group of Biliary Atresia and Registry (NeSBAR). Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008. J Pediatr 2012; 160 (04) 638-644.e2
    CrossrefPubMedGoogle Scholar
  • 5 de Vries W, Homan-Van der Veen J, Hulscher JB, Hoekstra-Weebers JE, Houwen RH, Verkade HJ. ; Netherlands Study Group of Biliary Atresia Registry. Twenty-year transplant-free survival rate among patients with biliary atresia. Clin Gastroenterol Hepatol 2011; 9 (12) 1086-1091
    CrossrefPubMedGoogle Scholar
  • 6 Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet 2009; 374 (9702): 1704-1713
    CrossrefPubMedGoogle Scholar
  • 7 Scheenstra R, Peeters PM, Verkade HJ, Gouw AS. Graft fibrosis after pediatric liver transplantation: ten years of follow-up. Hepatology 2009; 49 (03) 880-886
    CrossrefPubMedGoogle Scholar
  • 8 de Franchis R. ; Baveno VI Faculty. Expanding consensus in portal hypertension: report of the Baveno VI Consensus Workshop: stratifying risk and individualizing care for portal hypertension. J Hepatol 2015; 63 (03) 743-752
    CrossrefPubMedGoogle Scholar
  • 9 Duché M, Habès D, Roulleau P, Haas V, Jacquemin E, Bernard O. Prophylactic endoscopic sclerotherapy of large esophagogastric varices in infants with biliary atresia. Gastrointest Endosc 2008; 67 (04) 732-737
    CrossrefPubMedGoogle Scholar
  • 10 Kobayashi H, Stringer MD. Biliary atresia. Semin Neonatol 2003; 8 (05) 383-391
    CrossrefPubMedGoogle Scholar
  • 11 Shneider BL, de Ville de Goyet J, Leung DH. , et al. Primary prophylaxis of variceal bleeding in children and the role of MesoRex bypass: summary of the Baveno VI Pediatric Satellite Symposium. Hepatology 2016; 63 (04) 1368-1380
    CrossrefPubMedGoogle Scholar
  • 12 Gana JC, Turner D, Mieli-Vergani G. , et al. A clinical prediction rule and platelet count predict esophageal varices in children. Gastroenterology 2011; 141 (06) 2009-2016
    CrossrefPubMedGoogle Scholar
  • 13 Gana JC, Turner D, Roberts EA, Ling SC. Derivation of a clinical prediction rule for the noninvasive diagnosis of varices in children. J Pediatr Gastroenterol Nutr 2010; 50 (02) 188-193
    CrossrefPubMedGoogle Scholar
  • 14 Colecchia A, Di Biase AR, Scaioli E. , et al. Non-invasive methods can predict oesophageal varices in patients with biliary atresia after a Kasai procedure. Dig Liver Dis 2011; 43 (08) 659-663
    CrossrefPubMedGoogle Scholar
  • 15 Isted A, Grammatikopoulos T, Davenport M. Prediction of esophageal varices in biliary atresia: derivation of the “varices prediction rule”, a novel noninvasive predictor. J Pediatr Surg 2015; 50 (10) 1734-1738
    CrossrefPubMedGoogle Scholar
  • 16 Witters P, Hughes D, Karthikeyan P. , et al. King's variceal prediction score: a novel noninvasive marker of portal hypertension in pediatric chronic liver disease. J Pediatr Gastroenterol Nutr 2017; 64 (04) 518-523
    CrossrefPubMedGoogle Scholar
  • 17 Bedossa P. Presentation of a grid for computer analysis for compilation of histopathologic lesions in chronic viral hepatitis C. Cooperative study of the METAVIR group [in French]. Ann Pathol 1993; 13 (04) 260-265
    PubMedGoogle Scholar
  • 18 The French METAVIR Cooperative Study Group. Intraobserver and interobserver variations in liver biopsy interpretation in patients with chronic hepatitis C. Hepatology 1994; 20 (1 Pt 1): 15-20
    CrossrefPubMedGoogle Scholar
  • 19 Lin ZH, Xin YN, Dong QJ. , et al. Performance of the aspartate aminotransferase-to-platelet ratio index for the staging of hepatitis C-related fibrosis: an updated meta-analysis. Hepatology 2011; 53 (03) 726-736
    CrossrefPubMedGoogle Scholar
  • 20 Wai CT, Greenson JK, Fontana RJ. , et al. A simple noninvasive index can predict both significant fibrosis and cirrhosis in patients with chronic hepatitis C. Hepatology 2003; 38 (02) 518-526
    CrossrefPubMedGoogle Scholar
  • 21 Shaheen AA, Myers RP. Diagnostic accuracy of the aspartate aminotransferase-to-platelet ratio index for the prediction of hepatitis C-related fibrosis: a systematic review. Hepatology 2007; 46 (03) 912-921
    CrossrefPubMedGoogle Scholar
  • 22 Lampela H, Kosola S, Koivusalo A. , et al. Endoscopic surveillance and primary prophylaxis sclerotherapy of esophageal varices in biliary atresia. J Pediatr Gastroenterol Nutr 2012; 55 (05) 574-579
    CrossrefPubMedGoogle Scholar
  • 23 Wanty C, Helleputte T, Smets F, Sokal EM, Stephenne X. Assessment of risk of bleeding from esophageal varices during management of biliary atresia in children. J Pediatr Gastroenterol Nutr 2013; 56 (05) 537-543
    CrossrefPubMedGoogle Scholar
  • 24 Miga D, Sokol RJ, Mackenzie T, Narkewicz MR, Smith D, Karrer FM. Survival after first esophageal variceal hemorrhage in patients with biliary atresia. J Pediatr 2001; 139 (02) 291-296
    CrossrefPubMedGoogle Scholar
  • 25 Duché M, Ducot B, Tournay E. , et al. Prognostic value of endoscopy in children with biliary atresia at risk for early development of varices and bleeding. Gastroenterology 2010; 139 (06) 1952-1960
    CrossrefPubMedGoogle Scholar
  • 26 Shneider BL, Abel B, Haber B. , et al; Childhood Liver Disease Research and Education Network. Portal hypertension in children and young adults with biliary atresia. J Pediatr Gastroenterol Nutr 2012; 55 (05) 567-573
    CrossrefPubMedGoogle Scholar
  • 27 Shneider BL, Magee JC, Karpen SJ. , et al; Childhood Liver Disease Research Network (ChiLDReN). Total serum bilirubin within 3 months of hepatoportoenterostomy predicts short-term outcomes in biliary atresia. J Pediatr 2016; 170: 211-7.e1 , 2
    CrossrefPubMedGoogle Scholar
  • 28 Pimenta JR, Ferreira AR, Fagundes ED. , et al. Factors associated with bleeding secondary to rupture of esophageal varices in children and adolescents with cirrhosis. J Pediatr Gastroenterol Nutr 2017; 64 (02) e44-e48
    CrossrefPubMedGoogle Scholar
  • 29 Lilly JR, Stellin G. Variceal hemorrhage in biliary atresia. J Pediatr Surg 1984; 19 (04) 476-479
    CrossrefPubMedGoogle Scholar
  • 30 van Heurn LW, Saing H, Tam PK. Portoenterostomy for biliary atresia: long-term survival and prognosis after esophageal variceal bleeding. J Pediatr Surg 2004; 39 (01) 6-9
    CrossrefPubMedGoogle Scholar
  • 31 Duché M, Fabre M, Kretzschmar B, Serinet MO, Gauthier F, Chardot C. Prognostic value of portal pressure at the time of Kasai operation in patients with biliary atresia. J Pediatr Gastroenterol Nutr 2006; 43 (05) 640-645
    CrossrefPubMedGoogle Scholar
  • 32 Kang N, Davenport M, Driver M, Howard ER. Hepatic histology and the development of esophageal varices in biliary atresia. J Pediatr Surg 1993; 28 (01) 63-66
    CrossrefPubMedGoogle Scholar
  • 33 de Vries W, de Langen ZJ, Aronson DC. , et al; NeSBAR. Mortality of biliary atresia in children not undergoing liver transplantation in the Netherlands. Pediatr Transplant 2011; 15 (02) 176-183
    PubMedGoogle Scholar
  • 34 Desai MS, Zainuer S, Kennedy C, Kearney D, Goss J, Karpen SJ. Cardiac structural and functional alterations in infants and children with biliary atresia, listed for liver transplantation. Gastroenterology 2011; 141 (04) 1264-1272
    CrossrefPubMedGoogle Scholar
  • 35 Concato J, Peduzzi P, Holford TR, Feinstein AR. Importance of events per independent variable in proportional hazards analysis. I. Background, goals, and general strategy. J Clin Epidemiol 1995; 48 (12) 1495-1501
    CrossrefPubMedGoogle Scholar
  • 36 Sarin SK, Kumar A, Angus PW. , et al; Asian Pacific Association for the Study of the Liver (APASL) Working Party on Portal Hypertension. Diagnosis and management of acute variceal bleeding: Asian Pacific Association for Study of the Liver recommendations. Hepatol Int 2011; 5 (02) 607-624
    CrossrefPubMedGoogle Scholar
  • 37 Ling SC, Walters T, McKiernan PJ, Schwarz KB, Garcia-Tsao G, Shneider BL. Primary prophylaxis of variceal hemorrhage in children with portal hypertension: a framework for future research. J Pediatr Gastroenterol Nutr 2011; 52 (03) 254-261
    CrossrefPubMedGoogle Scholar