Approaches to the management of pediatric portal hypertension: results of an informal survey

  • Benjamin L. Shneider
Conference paper

Abstract

Portal hypertension and its related complications are major clinical issues in pediatric hepatology. The leading cause of significant liver disease in children is biliary atresia. The biliary injury in this disorder is associated with prominent portal hypertension at disease stages where synthetic liver function is typically intact. Thus the relative contribution of complications of portal hypertension to morbidity and mortality in liver disease is greater in children than in adults. In children with biliary atresia, prominent portal hypertension is often present at the time of corrective hepatoportoenterostomy (Kasai procedure) and it progresses over relatively short periods of time1. The rate of progression of the portal hypertension is related to outcome after hepatoportoenterostomy, with more accelerated progression in children with failed procedures or with recurrent cholangitis2. Complications that are a direct result of this portal hypertension are common. Variceal hemorrhage occurs in a large percentage of children, often within the first 5 years of life2, 3, 4, 5, 6, 7. Ascites, hepatopulmonary syndrome, rectal varices/colopathy, stomal hemorrhage, and pulmonary hypertension have also been described in children with biliary atresia8, 9, 10, 11, 12, 13.

Keywords

Portal Hypertension Esophageal Varix Biliary Atresia Variceal Hemorrhage Primary Prophylaxis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
This is a preview of subscription content, log in to check access

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Kasai M, Okamoto A, Ohi R, Yabe K, Matsumura Y. Changes of portal vein pressure and intrahepatic blood vessels after surgery for biliary atresia. J Pediatr Surg. 1981;16:152–9.PubMedCrossRefGoogle Scholar
  2. 2.
    Miyata M, Satani M, Ueda T, Okamoto E. Long-term results of hepatic portoenterostomy for biliary atresia: special reference to postoperative portal hypertension. Surgery. 1974;76:234–7.PubMedGoogle Scholar
  3. 3.
    Lilly JR, Stellin G. Variceal hemorrhage in biliary atresia. J Pediatr Surg. 1984;19:476–9.PubMedCrossRefGoogle Scholar
  4. 4.
    Ohi R, Mochizuki I, Komatsu K, Kasai M. Portal hypertension after successful hepatic porto enterostomy in biliary atresia. J Pediatr Surg. 1986;21:271–4.PubMedCrossRefGoogle Scholar
  5. 5.
    Stringer M, Howard E, Mowat A. Endoscopic sclerotherapy in the management of esophageal varices in 61 children with biliary atresia. J Pediatr Surg. 1989;24:438–92.PubMedCrossRefGoogle Scholar
  6. 6.
    Miga D, Sokol R, MacKenzie T, Narkewicz M, Smith D, Karrer F. Survival after first esophageal variceal hemorrhage in patients with biliary atresia. J Pediatr. 2001;139:291–6.PubMedCrossRefGoogle Scholar
  7. 7.
    van Heurn LW, Saing H, Tam PK. Portoenterostomy for biliary atresia: long-term survival and prognosis after esophageal variceal bleeding. J Pediatr Surg. 2004;39:6–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Beath S, Pearmain G, Kelly D, McMaster P, Mayer A, Buckels J. Liver transplantation in babies and children with extrahepatic biliary atresia. J Pediatr Surg. 1993;28: 1044–7.PubMedCrossRefGoogle Scholar
  9. 9.
    Yonemura T, Yoshibayashi M, Uemoto S, Inomata Y, Tanaka K, Furusho K. Intrapulmonary shunting in biliary atresia before and after living-related liver transplantation. Br J Surg. 1999;86:1139–43.PubMedCrossRefGoogle Scholar
  10. 10.
    Heaton ND, Davenport M, Howard ER. Incidence of haemorrhoids and anorectal varices in children with portal hypertension. Br J Surg. 1993;80:616–18.PubMedCrossRefGoogle Scholar
  11. 11.
    Smith S, Wiener ES, Starzl TE, Rowe MI. Stoma-related variceal bleeding: an underrecognized complication of biliary atresia. J Pediatr Surg. 1988;23:243–5.PubMedCrossRefGoogle Scholar
  12. 12.
    Schuijtvlot ET, Bax NM, Houwen RH, Hruda J. Unexpected lethal pulmonary hypertension in a 5-year-old girl successfully treated for biliary atresia. J Pediatr Surg. 1995;30:589–90.PubMedCrossRefGoogle Scholar
  13. 13.
    Soh H, Hasegawa T, Sasaki T et al. Pulmonary hypertension associated with postoperative biliary atresia: report of two cases. J Pediatr Surg. 1999;34:1779–81.PubMedCrossRefGoogle Scholar
  14. 14.
    Shneider B. Portal hypertension. In: Suchy F, Sokol R, Balistreri W, editors. Liver Disease in Children. Philadelphia: Lippincott Williams & Wilkins; 2001:129–51.Google Scholar
  15. 15.
    D’Amico G, Pagliaro L, Bosch J. The treatment of portal hypertension: a meta-analytic review. Hepatology. 1995;22:332–54.PubMedGoogle Scholar
  16. 16.
    Lay C, Tsai Y, Teg C et al. Endoscopic variceal prophylaxis of first variceal bleeding in cirrhotic patients with high-risk esophageal varices. Hepatology. 1997;25:1346–50.PubMedCrossRefGoogle Scholar
  17. 17.
    Barbe T, Losay J, Grimon G et al. Pulmonary arteriovenous shunting in children with liver disease. J Pediatr. 1995;126:571–9.PubMedCrossRefGoogle Scholar
  18. 18.
    D’Amico G, Pagliaro L, Bosch J. Pharmacological treatment of portal hypertension: an evidence-based approach. Semin Liver Dis. 1999;19:475–505.PubMedCrossRefGoogle Scholar
  19. 19.
    Abraczinskas DR, Ookubo R, Grace ND et al. Propranolol for the prevention of first esophageal variceal hemorrhage: a lifetime commitment? Hepatology. 2001;34:1096–102.PubMedCrossRefGoogle Scholar
  20. 20.
    Ozsoylu S, Kocak N, Yuce A. Propranalol therapy for portal hypertension in children. J Pediatr. 1985;106:317–20.PubMedCrossRefGoogle Scholar
  21. 21.
    Shashidhar H, Langhans N, Grand RJ. Propranalol in prevention of portal hypertensive hemorrhage in children: a pilot study. J Pediatr Gastrocnterol Nutr. 1999;29:12–17.CrossRefGoogle Scholar
  22. 22.
    de Kolster C, Rapa de Higuera M, Carvajal A, Castro J, Callegari C, Kolster J. Propranolol in children and adolescents with portal hypertension: its dosage and the clinical, cardiovascular and biochemical effects. G.E.N. 1992;46:199–207.PubMedGoogle Scholar
  23. 23.
    Ozsoylu S, Kocak N, Demir H, Yuce A, Gurakan F, Ozen H. Propranalol for primary and secondary prophylaxis of variceal bleeding in children with cirrhosis. Turk J Pediatr. 2000;42:31–3.PubMedGoogle Scholar
  24. 24.
    Imperiale T, Chalasani N. A meta-analysis of endoscopic variceal ligation for primary prophylaxis of esophageal variceal bleeding. Hepatology. 2001;33:802–7.PubMedCrossRefGoogle Scholar
  25. 25.
    Sokal E, Van Hoorebeeck N, Van Obbergh L, Otte JB, Buts JP. Upper gastro-intestinal tract bleeding in cirrhotic children candidates for liver transplantation. Eur J Pediatr. 1992;151:326–R.PubMedCrossRefGoogle Scholar
  26. 26.
    Chen L, Groszmann RJ. Blood in the gastric lumen increases splanchnic blood flow and portal pressure in portal-hypertensive rats. Gastroenterology. 1996;111:1103–10.PubMedCrossRefGoogle Scholar
  27. 27.
    Corley D, Cello J, Adkisson K, Ko W, Kerlikowske K. Octreotide for acute esophageal variceal bleeding: a meta-analysis. Gastroenterology. 2001;120:946–54.PubMedCrossRefGoogle Scholar
  28. 28.
    Bernard B, Grange J, Khac E, Amiot X, Opolon P, Poynard T. Antibiotic prophylaxis for the prevention of bacterial infections in cirrhotic patients with gastrointestinal bleeding: a meta-analysis. Hepatology. 1999;29:1655–61.PubMedCrossRefGoogle Scholar
  29. 29.
    Zargar SA, Javid G, Khan BA et al. Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction. Hepatology. 2002;36:666–72.PubMedCrossRefGoogle Scholar
  30. 30.
    Botha J, Grant W, Chinnakotla S et al. Is there still a role for shunt surgery in children during the era ofliver transplantation. Hepatology. 2001;34:201A.Google Scholar
  31. 31.
    Evans S, Stovroff M, Heiss K, Ricketts R. Selective distal splenorenal shunts for intractable variceal bleeding in pediatric portal hypertension. J Pediatr Surg. 1995;30:1115–18.PubMedCrossRefGoogle Scholar
  32. 32.
    Fonkalsrud EW. Surgical management of portal hypertension in childhood. Arch Surg. 1980;115:1042–5.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2004

Authors and Affiliations

  • Benjamin L. Shneider
    • 1
  1. 1.Division of Pediatric HepatologyMount Sinai Medical CenterNew YorkUSA

Personalised recommendations