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DOI: 10.1055/s-0042-1745857
Pediatric Portal Hypertension: Etiology and Management
Abstract
Portal hypertension is a known cause of morbidity and mortality in children. With a paucity of data and no standardized algorithm for treatment, a comprehensive understanding of this disease process is necessary for pediatric interventional radiologists, hepatologists, and surgeons who collaborate to care for these patients. While associations can be drawn to adult disease, the specifics of pediatric portal hypertension necessitate a separate, multidisciplinary approach. This review provides a summary and update on the evaluation, management, and treatment of pediatric portal hypertension.
Publication History
Received: 09 November 2021
Accepted: 24 January 2022
Article published online:
16 May 2022
© 2022. Thieme. All rights reserved.
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References
- 1 Gugig R, Rosenthal P. Management of portal hypertension in children. World J Gastroenterol 2012; 18 (11) 1176-1184 DOI: 10.3748/wjg.v18.i11.1176.
- 2 Grammatikopoulos T, McKiernan PJ, Dhawan A. Portal hypertension and its management in children. Arch Dis Child 2018; 103 (02) 186-191 DOI: 10.1136/archdischild-2015-310022.
- 3 Dehghani SM, Gholami S, Bahador A. et al. Comparison of Child-Turcotte-Pugh and pediatric end-stage liver disease scoring systems to predict morbidity and mortality of children awaiting liver transplantation. Transplant Proc 2007; 39 (10) 3175-3177 DOI: 10.1016/j.transproceed.2007.07.080.
- 4 Vogel CB. Pediatric portal hypertension: A review for primary care. Nurse Pract 2017; 42 (05) 35-42 DOI: 10.1097/01.NPR.0000515427.91649.91.
- 5 Shneider B, Emre S, Groszmann R. et al. Expert pediatric opinion on the Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. Pediatr Transplant 2006; 10 (08) 893-907 DOI: 10.1111/j.1399-3046.2006.00597.x.
- 6 Cullen JM, Stalker MJ. Liver and Biliary System. Pathol Domest Animals 2016; 2: 258
- 7 Si-Tayeb K, Lemaigre FP, Duncan SA. Organogenesis and development of the liver. Dev Cell 2010; 18 (02) 175-189 DOI: 10.1016/j.devcel.2010.01.011.
- 8 Giancotti A, Monti M, Nevi L. et al. Functions and the Emerging Role of the Foetal Liver into Regenerative Medicine. Cells 2019; 8 (08) E914 DOI: 10.3390/cells8080914.
- 9 Collardeau-Frachon S, Scoazec JY. Vascular development and differentiation during human liver organogenesis. Anat Rec (Hoboken) 2008; 291 (06) 614-627
- 10 Ober EA, Lemaigre FP. Development of the liver: Insights into organ and tissue morphogenesis. J Hepatol 2018; 68 (05) 1049-1062 DOI: 10.1016/j.jhep.2018.01.005.
- 11 Vauthey JN, Zimmitti G, Shindoh J. From Couinaud to molecular biology: the seven virtues of hepato-pancreato-biliary surgery. HPB (Oxford) 2012; 14 (08) 493-499 DOI: 10.1111/j.1477-2574.2012.00502.x.
- 12 Pauli EM, Staveley-O'Carroll KF, Brock MV, Efron DT, Efron G. A handy tool to teach segmental liver anatomy to surgical trainees. Arch Surg 2012; 147 (08) 692-693 DOI: 10.1001/archsurg.2012.689.
- 13 Strasberg SM. Nomenclature of hepatic anatomy and resections: a review of the Brisbane 2000 system. J Hepatobiliary Pancreat Surg 2005; 12 (05) 351-355 DOI: 10.1007/s00534-005-0999-7.
- 14 Carneiro C, Brito J, Bilreiro C. et al. All about portal vein: a pictorial display to anatomy, variants and physiopathology. Insights Imaging 2019; 10 (01) 38 DOI: 10.1186/s13244-019-0716-8.
- 15 Turco L, Garcia-Tsao G. Portal Hypertension: Pathogenesis and Diagnosis. Clin Liver Dis 2019; 23 (04) 573-587 DOI: 10.1016/j.cld.2019.07.007.
- 16 Rodríguez-Vilarrupla A, Fernández M, Bosch J, García-Pagán JC. Current concepts on the pathophysiology of portal hypertension. Ann Hepatol 2007; 6 (01) 28-36
- 17 Iwakiri Y. Pathophysiology of portal hypertension. Clin Liver Dis 2014; 18 (02) 281-291 DOI: 10.1016/j.cld.2013.12.001.
- 18 Gonçalves 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 (03) 401-405 DOI: 10.1016/s0022-3468(00)90203-3.
- 19 Yachha SK. Portal hypertension in children: an Indian perspective. J Gastroenterol Hepatol 2002; 17 (Suppl. 03) S228-S231 DOI: 10.1046/j.1440-1746.17.s3.5.x.
- 20 Giouleme O, Theocharidou E. Management of portal hypertension in children with portal vein thrombosis. J Pediatr Gastroenterol Nutr 2013; 57 (04) 419-425 DOI: 10.1097/MPG.0b013e3182a1cd7f.
- 21 DeLeve LD, Valla D-C, Garcia-Tsao G. American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology 2009; 49 (05) 1729-1764 DOI: 10.1002/hep.22772.
- 22 Shatzel JJ, O'Donnell M, Olson SR. et al. Venous thrombosis in unusual sites: A practical review for the hematologist. Eur J Haematol 2019; 102 (01) 53-62 DOI: 10.1111/ejh.13177.
- 23 Grama A, Pîrvan A, Sîrbe C. et al. Extrahepatic Portal Vein Thrombosis, an Important Cause of Portal Hypertension in Children. J Clin Med 2021; 10 (12) 2703 DOI: 10.3390/jcm10122703.
- 24 Simonetto DA, Liu M, Kamath PS. Portal Hypertension and Related Complications: Diagnosis and Management. Mayo Clin Proc 2019; 94 (04) 714-726 DOI: 10.1016/j.mayocp.2018.12.020.
- 25 Steiner PE. Nodular regenerative hyperplasia of the liver. Am J Pathol 1959; 35: 943-953
- 26 Hartleb M, Gutkowski K, Milkiewicz P. Nodular regenerative hyperplasia: evolving concepts on underdiagnosed cause of portal hypertension. World J Gastroenterol 2011; 17 (11) 1400-1409 DOI: 10.3748/wjg.v17.i11.1400.
- 27 Khanna R, Sarin SK. R K. Non-cirrhotic portal hypertension - diagnosis and management. J Hepatol 2014; 60 (02) 421-441 DOI: 10.1016/J.JHEP.2013.08.013.
- 28 Yokoda RT, Carey EJ. RT Y. Primary Biliary Cholangitis and Primary Sclerosing Cholangitis. Am J Gastroenterol 2019; 114 (10) 1593-1605 DOI: 10.14309/AJG.0000000000000268.
- 29 Erden A, Altuntaş B, Olçer T, Cumhur T. Presinusoidal portal hypertension does not affect hepatic artery resistance index. Eur Radiol 1999; 9 (06) 1131-1134 DOI: 10.1007/s003300050806.
- 30 Grus T, Lambert L, Grusová G, Banerjee R, Burgetová A. Budd-Chiari Syndrome. Prague Med Rep 2017; 118 (2-3): 69-80 DOI: 10.14712/23362936.2017.6.
- 31 Plessier A, Valla D-C. Budd-Chiari syndrome. Semin Liver Dis 2008; 28 (03) 259-269 DOI: 10.1055/s-0028-1085094.
- 32 Aydinli M, Bayraktar Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol 2007; 13 (19) 2693-2696 DOI: 10.3748/wjg.v13.i19.2693.
- 33 Hernández-Guerra M, López E, Bellot P. et al. Systemic hemodynamics, vasoactive systems, and plasma volume in patients with severe Budd-Chiari syndrome. Hepatology 2006; 43 (01) 27-33 DOI: 10.1002/hep.20990.
- 34 Wong F. Portal hypertensive gastropathy. Gastroenterol Hepatol (N Y) 2007; 3 (06) 428-473
- 35 Hyams JS, Treem WR. Portal hypertensive gastropathy in children. J Pediatr Gastroenterol Nutr 1993; 17 (01) 13-18 DOI: 10.1097/00005176-199307000-00002.
- 36 Urrunaga NH, Rockey DC. Portal hypertensive gastropathy and colopathy. Clin Liver Dis 2014; 18 (02) 389-406 DOI: 10.1016/j.cld.2014.01.008.
- 37 McKenzie CV, Colonne CK, Yeo JH, Fraser ST. Splenomegaly: Pathophysiological bases and therapeutic options. Int J Biochem Cell Biol 2018; 94: 40-43 DOI: 10.1016/j.biocel.2017.11.011.
- 38 Weissenborn K. Hepatic Encephalopathy: Definition, Clinical Grading and Diagnostic Principles. Drugs 2019; 79 (Suppl. 01) 5-9 DOI: 10.1007/s40265-018-1018-z.
- 39 Cartin-Ceba R, Krowka MJ. Pulmonary Complications of Portal Hypertension. Clin Liver Dis 2019; 23 (04) 683-711 DOI: 10.1016/j.cld.2019.06.003.
- 40 McAdams HP, Erasmus J, Crockett R, Mitchell J, Godwin JD, McDermott VG. The hepatopulmonary syndrome: radiologic findings in 10 patients. AJR Am J Roentgenol 1996; 166 (06) 1379-1385 DOI: 10.2214/ajr.166.6.8633451.
- 41 Machicao VI, Fallon MB. Hepatopulmonary syndrome. Semin Respir Crit Care Med 2012; 33 (01) 11-16 DOI: 10.1055/S-0032-1301730.
- 42 Moore CM, Van Thiel DH, Christopher T, Moore M, Van Thiel DH. Cirrhotic ascites review: Pathophysiology, diagnosis and management. World J Hepatol 2013; 5 (05) 251-263 DOI: 10.4254/wjh.v5.i5.251.
- 43 Kassam AF, Goddard GR, Johnston ME. et al. Natural Course of Pediatric Portal Hypertension. Hepatol Commun 2020; 4 (09) 1346-1352 DOI: 10.1002/hep4.1560.
- 44 Ebel NH, Carlin K, Shaffer ML. et al. Hepatic Venous Pressure Gradient Measurements in Children: Correlation With Hepatic Histology and Clinical Indicators of Portal Hypertension. J Pediatr Gastroenterol Nutr 2019; 68 (06) 788-792 DOI: 10.1097/MPG.0000000000002327.
- 45 Ravaioli F, Montagnani M, Lisotti A, Festi D, Mazzella G, Azzaroli F. Noninvasive Assessment of Portal Hypertension in Advanced Chronic Liver Disease: An Update. Gastroenterol Res Pract 2018; 2018: 4202091 DOI: 10.1155/2018/4202091.
- 46 Berzigotti A, Seijo S, Reverter E, Bosch J. Assessing portal hypertension in liver diseases. Expert Rev Gastroenterol Hepatol 2013; 7 (02) 141-155 DOI: 10.1586/egh.12.83.
- 47 Berzigotti A. Non-invasive evaluation of portal hypertension using ultrasound elastography. J Hepatol 2017; 67 (02) 399-411 DOI: 10.1016/j.jhep.2017.02.003.
- 48 Talwalkar JA, Kurtz DM, Schoenleber SJ, West CP, Montori VM. Ultrasound-based transient elastography for the detection of hepatic fibrosis: systematic review and meta-analysis. Clin Gastroenterol Hepatol 2007; 5 (10) 1214-1220 DOI: 10.1016/j.cgh.2007.07.020.
- 49 Stebbing J, Farouk L, Panos G. et al. A meta-analysis of transient elastography for the detection of hepatic fibrosis. J Clin Gastroenterol 2010; 44 (03) 214-219 DOI: 10.1097/MCG.0b013e3181b4af1f.
- 50 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 DOI: 10.1016/J.JHEP.2015.05.022.
- 51 Shneider BL, Bosch J, de Franchis R. et al; expert panel of the Children's Hospital of Pittsburgh of UPMC. 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 (05) 426-437 DOI: 10.1111/j.1399-3046.2012.01652.x.
- 52 Rockey DC. A New Treatment for Portal Hypertension?. Gastroenterology 2016; 150 (05) 1077-1080 DOI: 10.1053/j.gastro.2016.03.015.
- 53 Di Giorgio A, Nicastro E, Agazzi R, Colusso M, D'Antiga L. Long-term Outcome of Transjugular Intrahepatic Portosystemic Shunt in Children With Portal Hypertension. J Pediatr Gastroenterol Nutr 2020; 70 (05) 615-622 DOI: 10.1097/MPG.0000000000002597.
- 54 Monroe EJ, Shivaram GM. Pediatric Hepatobiliary Interventions in the Setting of Intrahepatic Vascular Malformations, Portal Hypertension, and Liver Transplant. Semin Roentgenol 2019; 54 (04) 311-323 DOI: 10.1053/j.ro.2019.06.002.
- 55 Bertino F, Hawkins CM, Shivaram G. et al. Technical Feasibility and Clinical Effectiveness of Transjugular Intrahepatic Portosystemic Shunt Creation in Pediatric and Adolescent Patients. J Vasc Interv Radiol 2019; 30 (02) 178-186.e5 DOI: 10.1016/j.jvir.2018.10.003.
- 56 Dabrowiecki A, Monroe EJ, Romero R, Gill AE, Hawkins CM. Endovascular and Percutaneous Treatment of Pediatric Portosystemic Varices: A Case Review. Dig Dis Interv 2019; 03: 214-226 DOI: 10.1055/s-0039-1694978.
- 57 Clinical Efficacy of Percutaneous Transhepatic Portal Vein Angioplasty for Late-onset Portal Vein Stenosis in Pediatric Liver Transplant Patients - PubMed. 2021 https://pubmed-ncbi-nlm-nih-gov.proxy.library.emory.edu/29470353/ (accessed November 9, 2021).
- 58 Kallini JR, Gabr A, Kulik L. et al. Noncirrhotic complete obliterative portal vein thrombosis: Novel management using trans-splenic transjugular intrahepatic portosystemic shunt with portal vein recanalization. Hepatology 2016; 63 (04) 1387-1390 DOI: 10.1002/hep.28429.
- 59 Lautz TB, Keys LA, Melvin JC, Ito J, Superina RA. Advantages of the meso-Rex bypass compared with portosystemic shunts in the management of extrahepatic portal vein obstruction in children. J Am Coll Surg 2013; 216 (01) 83-89 DOI: 10.1016/j.jamcollsurg.2012.09.013.
- 60 Guérin F, Bidault V, Gonzales E, Franchi-Abella S, De Lambert G, Branchereau S. Meso-Rex bypass for extrahepatic portal vein obstruction in children. Br J Surg 2013; 100 (12) 1606-1613 DOI: 10.1002/bjs.9287.
- 61 Kumar A, Sharma N, Bajpai M, Panda SS. Spontaneous resolution of splenic infarcts after distal splenorenal shunt in children with extra hepatic portal venous obstruction: our experience. Afr J Paediatr Surg 2014; 11 (01) 48-51 DOI: 10.4103/0189-6725.129223.
- 62 Cortez AR, Kassam AF, Jenkins TM. et al. The role of surgical shunts in the treatment of pediatric portal hypertension. Surgery 2019; 166 (05) 907-913 DOI: 10.1016/J.SURG.2019.05.009.