Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-5c6d5d7d68-tdptf Total loading time: 0 Render date: 2024-08-08T13:12:35.992Z Has data issue: false hasContentIssue false

Chapter 6 - Portal Hypertension in Children

from Section I - Pathophysiology of Pediatric Liver Disease

Published online by Cambridge University Press:  19 January 2021

By
Benjamin L. Shneider
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Edited in association with
Jorge A. Bezerra ,
Cara L. Mack  and
Benjamin L. Shneider
Frederick J. Suchy
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Ronald J. Sokol
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
William F. Balistreri
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Jorge A. Bezerra
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Cara L. Mack
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Benjamin L. Shneider
Affiliation:
Texas Children’s Hospital, Houston
Get access

Summary

A portal system is one, which by definition, begins and ends with capillaries. The major portal system in humans is one in which the capillaries originate in the mesentery of the intestines and spleen and end in the hepatic sinusoids. Capillaries of the superior mesenteric and splenic veins supply the portal vein with a nutrient- and hormone-rich blood supply. The partially oxygenated portal venous blood supplements the oxygenated hepatic arterial flow to give the liver unique protection against hypoxia. Blood flow from the hepatic artery and portal vein is well coordinated to maintain consistent flow and explains the ability of the liver to withstand thrombosis of either of these major vascular structures. This well-regulated blood flow in conjunction with the very low resistance found in the portal system results in a low baseline portal pressure in healthy individuals.

Type
Chapter
Information
Liver Disease in Children , pp. 74 - 93
Publisher: Cambridge University Press
Print publication year: 2021

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Teckman, JH, Rosenthal, P, Abel, R, Bass, LM, Michail, S, Murray, KF, Rudnick, DA, et al. Baseline analysis of a young alpha-1-antitrypsin deficiency liver disease cohort reveals frequent portal hypertension. J Pediatr Gastroenterol Nutr 2015;61:94–101.Google Scholar
D’Antiga, L, Betalli, P, De Angelis, P, Davenport, M, Di Giorgio, A, McKiernan, PJ, McLin, V, et al. Interobserver agreement on endoscopic classification of oesophageal varices in children. J Pediatr Gastroenterol Nutr 2015;61:176–81.Google Scholar
Koot, BG, Alders, M, Verheij, J, Beuers, U, Cobben, JM. A de novo mutation in KCNN3 associated with autosomal dominant idiopathic non-cirrhotic portal hypertension. J Hepatol 2016;64:974–7.Google Scholar
Duche, M, Ducot, B, Ackermann, O, Guerin, F, Jacquemin, E, Bernard, O. Portal hypertension in children: high-risk varices, primary prophylaxis and consequences of bleeding. J Hepatol 2017;66:320–7.CrossRefGoogle ScholarPubMed
Cardey, J, Le Gall, C, Michaud, L, Dabadie, A, Talbotec, C, Bellaiche, M, Lamireau, T, et al. Screening of esophageal varices in children using esophageal capsule endoscopy: a multicenter prospective study. Endoscopy 2019;51:10–17.Google Scholar
Shneider, BL, Bosch, J, de Franchis, R, Emre, SH, Groszmann, RJ, Ling, SC, Lorenz, JM, et al. Portal hypertension in children: expert pediatric opinion on the report of the Baveno v Consensus Workshop on Methodology of Diagnosis and Therapy in Portal Hypertension. Pediatr Transplant 2012;16:426–37.Google Scholar
Fernandez, M. Molecular pathophysiology of portal hypertension. Hepatology 2015;61:1406–15.CrossRefGoogle ScholarPubMed
McConnell, M, Iwakiri, Y. Biology of portal hypertension. Hepatol Int 2018;12:11–23.CrossRefGoogle ScholarPubMed
Konigshofer, P, Brusilovskaya, K, Schwabl, P, Reiberger, T. Animal models of portal hypertension. Biochim Biophys Acta Mol Basis Dis 2019;1865:1019–30.CrossRefGoogle ScholarPubMed
Kowalski, HJ, Abelmann, WH. The cardiac output at rest in Laennec’s cirrhosis. J Clin Invest 1953;32:1025–33.Google Scholar
Valla, DC. Budd-Chiari syndrome/hepatic venous outflow tract obstruction. Hepatol Int 2018;12:168–80.Google Scholar
Gentil-Kocher, S, Bernard, O, Brunelle, F, Hadchouel, M, Maillard, JN, Valayer, J, Hay, JM, et al. Budd-Chiari syndrome in children: report of 22 cases. J Pediatr 1988;113:30–8.Google Scholar
Nobre, S, Khanna, R, Bab, N, Kyrana, E, Height, S, Karani, J, Kane, P, et al. Primary Budd-Chiari syndrome in children: King’s College Hospital Experience. J Pediatr Gastroenterol Nutr 2017;65:93–6.Google Scholar
Franceschet, I, Zanetto, A, Ferrarese, A, Burra, P, Senzolo, M. Therapeutic approaches for portal biliopathy: a systematic review. World J Gastroenterol 2016;22:9909–20.CrossRefGoogle ScholarPubMed
Thompson, RJ, Taylor, MA, McKie, LD, Diamond, T. Sinistral portal hypertension. Ulster Med J 2006;75:175–7.Google ScholarPubMed
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;139:291–6.CrossRefGoogle ScholarPubMed
D’Amico, G, De Franchis, R. Upper digestive bleeding in cirrhosis. Post-therapeutic outcome and prognostic indicators. Hepatology 2003;38:599–612.Google Scholar
Etzion, O, Koh, C, Heller, T. Noncirrhotic portal hypertension: an overview. Clin Liver Dis 2015;6:72–4.Google Scholar
Sood, V, Lal, BB, Khanna, R, Rawat, D, Bihari, C, Alam, S. Noncirrhotic portal fibrosis in pediatric population. J Pediatr Gastroenterol Nutr 2017;64:748–53.CrossRefGoogle ScholarPubMed
Besmond, C, Valla, D, Hubert, L, Poirier, K, Grosse, B, Guettier, C, Bernard, O, et al. Mutations in the novel gene FOPV are associated with familial autosomal dominant and non-familial obliterative portal venopathy. Liver Int 2018;38:358–64.Google Scholar
Arab, JP, Barrera, F, Arrese, M. Bile acids and portal hypertension. Ann Hepatol 2017;16:s83–s86.Google Scholar
Eblimit, Z, Thevananther, S, Karpen, SJ, Taegtmeyer, H, Moore, DD, Adorini, L, Penny, DJ, et al. TGR5 activation induces cytoprotective changes in the heart and improves myocardial adaptability to physiologic, inotropic, and pressure-induced stress in mice. Cardiovasc Ther 2018;36:e12462.CrossRefGoogle ScholarPubMed
Desai, MS, Mathur, B, Eblimit, Z, Vasquez, H, Taegtmeyer, H, Karpen, SJ, Penny, DJ, et al. Bile acid excess induces cardiomyopathy and metabolic dysfunctions in the heart. Hepatology 2017;65:189–201.CrossRefGoogle ScholarPubMed
Gorgis, NM, Kennedy, C, Lam, F, Thompson, K, Coss-Bu, J, Akcan Arikan, A, Nguyen, T, et al. Clinical consequences of cardiomyopathy in children with biliary atresia requiring liver transplantation. Hepatology 2019;69:1206–18.CrossRefGoogle ScholarPubMed
Tripathi, D, Hayes, PC. The role of carvedilol in the management of portal hypertension. Eur J Gastroenterol Hepatol 2010;22:905–11.Google Scholar
Howard, ER, Stringer, MD, Mowat, AP. Assessment of injection sclerotherapy in the management of 152 children with oesophageal varices. Br J Surg 1988;75:404–8.Google Scholar
Mitra, SK, Kumar, V, Datta, DV, Rao, PN, Sandhu, K, Singh, GK, Sodhi, JS, et al. Extrahepatic portal hypertension: a review of 70 cases. J Pediatr Surg 1978;13:51–7.Google Scholar
Webb, LJ, Sherlock, S. The aetiology, presentation and natural history of extra-hepatic portal venous obstruction. Q J Med 1979;48:627–39.Google Scholar
Waisbourd-Zinman, O, Shah, A, Lin, HC, Rand, EB. Splenic rupture in children with portal hypertension. J Pediatr Gastroenterol Nutr 2018;66:447–50.Google Scholar
Lee, WS, Wong, SY, Ivy, DD, Sokol, RJ. Hepatopulmonary syndrome and portopulmonary hypertension in children: recent advances in diagnosis and management. J Pediatr 2018;196:14–21 e11.Google Scholar
Abrams, GA, Jaffe, CC, Hoffer, PB, Binder, HJ, Fallon, MB. Diagnostic utility of contrast echocardiography and lung perfusion scan in patients with hepatopulmonary syndrome. Gastroenterology 1995;109:1283–8.Google Scholar
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.Google ScholarPubMed
Barbe, T, Losay, J, Grimon, G, Devictor, D, Sardet, A, Gauthier, F, Houssin, D, et al. Pulmonary arteriovenous shunting in children with liver disease. J Pediatr 1995;126:571–9.Google Scholar
Hoerning, A, Raub, S, Neudorf, U, Muntjes, C, Kathemann, S, Lainka, E, Stehling, F, et al. Pulse oximetry is insufficient for timely diagnosis of hepatopulmonary syndrome in children with liver cirrhosis. J Pediatr 2014;164:546–52 e541–2.Google Scholar
Cartin-Ceba, R, Krowka, MJ. Portopulmonary hypertension. Clin Liver Dis 2014;18:421–38.Google Scholar
Ecochard-Dugelay, E, Lambert, V, Schleich, JM, Duche, M, Jacquemin, E, Bernard, O. Portopulmonary hypertension in liver disease presenting in childhood: study of fourteen patients and literature review. J Pediatr Gastroenterol Nutr 2015;61:346–54.Google Scholar
AbuHalimeh, B, Krowka, MJ, Tonelli, AR. Treatment barriers in portopulmonary hypertension. Hepatology 2019;69:431–43.CrossRefGoogle ScholarPubMed
Yousef, N, Habes, D, Ackermann, O, Durand, P, Bernard, O, Jacquemin, E. Hepatorenal syndrome: diagnosis and effect of terlipressin therapy in 4 pediatric patients. J Pediatr Gastroenterol Nutr 2010;51:100–2.Google Scholar
Deep, A, Saxena, R, Jose, B. Acute kidney injury in children with chronic liver disease. Pediatr Nephrol 2019;34:45–59.CrossRefGoogle ScholarPubMed
Mack, CL, Zelko, FA, Lokar, J, Superina, R, Alonso, EM, Blei, AT, Whitington, PF. Surgically restoring portal blood flow to the liver in children with primary extrahepatic portal vein thrombosis improves fluid neurocognitive ability. Pediatrics 2006;117:e405–12.Google Scholar
Srivastava, A, Chaturvedi, S, Gupta, RK, Malik, R, Mathias, A, Jagannathan, NR, Jain, S, et al. Minimal hepatic encephalopathy in children with chronic liver disease: prevalence, pathogenesis and magnetic resonance-based diagnosis. J Hepatol 2017;66:528–36.Google Scholar
D’Amico, G, Morabito, A, D’Amico, M, Pasta, L, Malizia, G, Rebora, P, Valsecchi, MG. Clinical states of cirrhosis and competing risks. J Hepatol 2018;68:563–76.Google Scholar
Sarin, SK, Agarwal, SR. Extrahepatic portal vein obstruction. Semin Liver Dis 2002;22:43–58.Google Scholar
Shneider, BL, de Ville de Goyet, J, Leung, DH, Srivastava, A, Ling, SC, Duche, M, McKiernan, P, 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;63:1368–80.Google Scholar
Bezerra, JA, Wells, RG, Mack, CL, Karpen, SJ, Hoofnagle, JH, Doo, E, Sokol, RJ. Biliary atresia: clinical and research challenges for the twenty-first century. Hepatology 2018;68:1163–73.Google Scholar
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.Google Scholar
Shneider, BL, Abel, B, Haber, B, Karpen, SJ, Magee, JC, Romero, R, Schwarz, K, et al. Portal hypertension in children and young adults with biliary atresia. J Pediatr Gastroenterol Nutr 2012;55:567–73.Google Scholar
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.CrossRefGoogle ScholarPubMed
van Wessel, DB, Witt, M, Bax, N, Verkade, HJ, Scheenstra, R, de Kleine, RH, Hulscher, JB. Variceal bleeds in patients with biliary atresia. Eur J Pediatr Surg 2018;28:439–44.Google Scholar
Gana, JC, Turner, D, Mieli-Vergani, G, Davenport, M, Miloh, T, Avitzur, Y, Yap, J, et al. A clinical prediction rule and platelet count predict esophageal varices in children. Gastroenterology 2011;141:2009–16.Google Scholar
Bass, LM, Shneider, BL, Henn, L, Goodrich, NP, Magee, JC, Childhood liver disease research. Clinically evident portal hypertension: an operational research definition for future investigations in the pediatric population. J Pediatr Gastroenterol Nutr 2019;68:763–7.Google Scholar
de Franchis, R, Baveno, VIF. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension. J Hepatol 2015;63:743–52.Google Scholar
Kim, DW, Yoon, HM, Jung, AY, Lee, JS, Oh, SH, Kim, KM, Cho, YA. Diagnostic performance of ultrasound elastography for evaluating portal hypertension in children: a systematic review and meta-analysis. J Ultrasound Med 2019;38:747–59.Google Scholar
Yokoyama, S, Ishigami, M, Honda, T, Kuzuya, T, Ishizu, Y, Ito, T, Hirooka, Y, et al. Spleen stiffness by 2-D shear wave elastography is the most accurate predictor of high-risk esophagogastric varices in children with biliary atresia. Hepatol Res 2019;49:1162–8.CrossRefGoogle Scholar
Sokal, EM, 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;151:326–8.Google Scholar
Bosch, J, Abraldes, JG, Berzigotti, A, Garcia-Pagan, JC. The clinical use of HVPG measurements in chronic liver disease. Nat Rev Gastroenterol Hepatol 2009;6:573–82.Google Scholar
Miraglia, R, Luca, A, Maruzzelli, L, Spada, M, Riva, S, Caruso, S, Maggiore, G, et al. Measurement of hepatic vein pressure gradient in children with chronic liver diseases. J Hepatol 2010;53:624–9.Google Scholar
Garcia-Tsao, G, Abraldes, JG, Berzigotti, A, Bosch, J. Portal hypertensive bleeding in cirrhosis: risk stratification, diagnosis, and management: 2016 practice guidance by the American Association for the study of liver diseases. Hepatology 2017;65:310–35.Google Scholar
Sharma, M, Singh, S, Desai, V, Shah, VH, Kamath, PS, Murad, MH, Simonetto, DA. Comparison of therapies for primary prevention of esophageal variceal bleeding: a systematic review and network meta-analysis. Hepatology 2019;69:1657–75.Google Scholar
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;52:254–61.Google Scholar
Goncalves, ME, Cardoso, SR, Maksoud, JG. Prophylactic sclerotherapy in children with esophageal varices: long-term results of a controlled prospective randomized trial. J Pediatr Surg 2000;35:401–5.Google Scholar
Zargar, SA, Javid, G, Khan, BA, Yattoo, GN, Shah, AH, Gulzar, GM, Singh, J, et al. Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction. Hepatology 2002;36:666–72.Google Scholar
Carneiro de Moura, M, Chen, S, Kamath, BM, Ng, VL, Ling, SC. Acute variceal bleeding causes significant morbidity. J Pediatr Gastroenterol Nutr 2018;67:371–6.Google Scholar
Colli, A, Gana, JC, Yap, J, Adams-Webber, T, Rashkovan, N, Ling, SC, Casazza, G. Platelet count, spleen length, and platelet count-to-spleen length ratio for the diagnosis of oesophageal varices in people with chronic liver disease or portal vein thrombosis. Cochrane Database Syst Rev 2017;4:CD008759.Google Scholar
Zacharias, AP, Jeyaraj, R, Hobolth, L, Bendtsen, F, Gluud, LL, Morgan, MY. Carvedilol versus traditional, non-selective beta-blockers for adults with cirrhosis and gastroesophageal varices. Cochrane Database Syst Rev 2018;10:CD011510.Google ScholarPubMed
Shashidhar, H, Langhans, N, Grand, RJ. Propranalol in prevention of portal hypertensive hemorrhage in children: a pilot study. J Pediatr Gastroenterol Nutr 1999;29:12–17.Google Scholar
Samanta, T, Purkait, R, Sarkar, M, Misra, A, Ganguly, S. Effectiveness of beta blockers in primary prophylaxis of variceal bleeding in children with portal hypertension. Trop Gastroenterol 2011;32:299–303.Google Scholar
Pimenta, JR, Ferreira, AR, Bittencourt, PF, Resende, CB, Fagundes, ED, Silva, IM. Evaluation of primary prophylaxis with propranolol and elastic band ligation in variceal bleeding in cirrhotic children and adolescents. Arq Gastroenterol 2016;53:257–61.Google Scholar
Shashidhar, H, Langhans, N, Grand, RJ. Propranolol in prevention of portal hypertensive hemorrhage in children: a pilot study. J Pediatr Gastroenterol Nutr 1999;29:12–17.Google Scholar
Tripodi, A, Primignani, M, Mannucci, PM, Caldwell, SH. Changing concepts of cirrhotic coagulopathy. Am J Gastroenterol 2017;112:274–81.Google Scholar
O’Leary, JG, Greenberg, CS, Patton, HM, Caldwell, SH. AGA clinical practice update: coagulation in cirrhosis. Gastroenterology 2019;157:34–43 e31.Google Scholar
Eroglu, Y, Emerick, KM, Whitingon, PF, Alonso, EM. Octreotide therapy for control of acute gastrointestinal bleeding in children. J Pediatr Gastroenterol Nutr 2004;38:41–7.Google Scholar
Jayakumar, S, Odulaja, A, Patel, S, Davenport, M, Ade-Ajayi, N. Surviving Sengstaken. J Pediatr Surg 2015;50:1142–6.Google Scholar
Leung, DH, Narkewicz, MR. Cystic fibrosis-related cirrhosis. J Cyst Fibros 2017;16 (Suppl 2):S50–S61.Google Scholar
Srinath, A, Shneider, BL. Congenital hepatic fibrosis and autosomal recessive polycystic kidney disease. J Pediatr Gastroenterol Nutr 2012;54:580–7.Google Scholar
McKiernan, PJ, Beath, SV, Davison, SM. A prospective study of endoscopic esophageal variceal ligation using a multiband ligator. J Pediatr Gastroenterol Nutr 2002;34:207–11.Google Scholar
Oh, SH, Kim, SJ, Rhee, KW, Kim, KM. Endoscopic cyanoacrylate injection for the treatment of gastric varices in children. World J Gastroenterol 2015;21:2719–24.Google Scholar
Emre, S, Dugan, C, Frankenberg, T, Hudgins, LC, Gagliardi, R, Artis, AT, Rodriguez-Laiz, G, et al. Surgical portosystemic shunts and the Rex bypass in children: a single-centre experience. HPB 2009;11:252–7.Google Scholar
Guerin, F, Charre, L, Jasienski, S, Duche, M, Franchiabella, S, Bernard, O, Jacquemin, E, et al. The efficacy of surgical shunts to treat severe portal hypertension after a Kasai procedure for biliary atresia. J Pediatr Surg 2019;54:531–6.Google Scholar
Lemoine, C, Lokar, J, McColley, SA, Alonso, EM, Superina, R. Cystic fibrosis and portal hypertension: distal splenorenal shunt can prevent the need for future liver transplant. J Pediatr Surg 2019;54:1076–82.Google Scholar
Superina, R, Shneider, B, Emre, S, Sarin, SK, de Ville de Goyet, J. Surgical guidelines for the management of extra-hepatic portal vein obstruction. Pediatric Transplantation 2006;10:908–13.Google Scholar
Carollo, V, Marrone, G, Cortis, K, Mamone, G, Caruso, S, Milazzo, M, Maruzzelli, L, et al. Multimodality imaging of the Meso-Rex bypass. Abdom Radiol 2019;44:1379–94.CrossRefGoogle ScholarPubMed
Heyman, MB, LaBerge, JM, Somberg, KA, Rosenthal, P, Mudge, C, Ring, EJ, Snyder, JD. Transjugular intrahepatic portosystemic shunts (TIPS) in children. J Pediatr 1997;131:914–19.Google Scholar
Lorenz, JM. Placement of transjugular intrahepatic portosystemic shunts in children. Tech Vasc Interv Radiol 2008;11:235–40.Google Scholar
Vo, NJ, Shivaram, G, Andrews, RT, Vaidya, S, Healey, PJ, Horslen, SP. Midterm follow-up of transjugular intrahepatic portosystemic shunts using polytetrafluoroethylene endografts in children. J Vasc Interv Radiol 2012;23:919–24.Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×