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Digestive Disease Interventions
DOI: 10.1055/s-0044-1782668
Review Article

An Overview of Surgical Management of Portal Hypertension in Children

Mazhar Khalil
1   Department of General Surgery, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio
,
Koji Hashimoto
1   Department of General Surgery, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio
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Abstract

Pediatric portal hypertension is a relatively rare but a life-threatening problem. With advancements in understanding of portal hypertension physiology and lessons learned from management in adults, medical management is playing an increasing role. However, certain conditions are amenable to surgical management. Key considerations for surgical options are state of liver parenchyma and preservation of liver function. In cases of extrahepatic portal vein obstruction, Meso-Rex bypass or shunt can offer a long-term solution. Similarly in select group of patients, selective or central shunts can be considered. With advancement in liver transplantation, there are very few indications for non-transplant surgical procedures for the management of portal hypertension.

Keywords

pediatric portal hypertension - portosystemic shunts - surgical treatment of portal - hypertension

Financial Disclosure

Authors have no financial disclosure.




Publication History

Received: 21 February 2024

Accepted: 22 February 2024

Article published online:
02 April 2024

© 2024. Thieme. All rights reserved.

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  • References

  • 1 Eipel C, Abshagen K, Vollmar B. Regulation of hepatic blood flow: the hepatic arterial buffer response revisited. World J Gastroenterol 2010; 16 (48) 6046-6057
    CrossrefPubMedGoogle Scholar
  • 2 Kawada N, Tran-Thi TA, Klein H, Decker K. The contraction of hepatic stellate (Ito) cells stimulated with vasoactive substances. Possible involvement of endothelin 1 and nitric oxide in the regulation of the sinusoidal tonus. Eur J Biochem 1993; 213 (02) 815-823
    CrossrefPubMedGoogle Scholar
  • 3 Iwakiri Y, Kim MY. Nitric oxide in liver diseases. Trends Pharmacol Sci 2015; 36 (08) 524-536
    CrossrefPubMedGoogle Scholar
  • 4 Ballet F, Chretien Y, Rey C, Poupon R. Differential response of normal and cirrhotic liver to vasoactive agents. A study in the isolated perfused rat liver. J Pharmacol Exp Ther 1988; 244 (01) 283-289
    PubMedGoogle Scholar
  • 5 Iwakiri Y, Groszmann RJ. The hyperdynamic circulation of chronic liver diseases: from the patient to the molecule. Hepatology 2006; 43 (2, Suppl 1): S121-S131
    CrossrefPubMedGoogle Scholar
  • 6 Kim MY, Baik SK. [Hyperdynamic circulation in patients with liver cirrhosis and portal hypertension]. Korean J Gastroenterol 2009; 54 (03) 143-148
    CrossrefPubMedGoogle Scholar
  • 7 Møller S, Bendtsen F. The pathophysiology of arterial vasodilatation and hyperdynamic circulation in cirrhosis. Liver Int 2018; 38 (04) 570-580
    CrossrefPubMedGoogle Scholar
  • 8 Nobre S, Khanna R, Bab N. et al. Primary Budd-Chiari syndrome in children: King's College Hospital experience. J Pediatr Gastroenterol Nutr 2017; 65 (01) 93-96
    CrossrefPubMedGoogle Scholar
  • 9 Samanta A, Sen Sarma M, Yadav R. Budd-Chiari syndrome in children: challenges and outcome. World J Hepatol 2023; 15 (11) 1174-1187
    CrossrefPubMedGoogle Scholar
  • 10 Mukhiya G, Zhou X, Han X. et al. Evaluation of outcome from endovascular therapy for Budd-Chiari syndrome: a systematic review and meta-analysis. Sci Rep 2022; 12 (01) 16166
    CrossrefPubMedGoogle Scholar
  • 11 Garmpis N, Damaskos C, Prevezanos D. et al. Liver transplantation and Budd-Chiari syndrome: when the cause becomes the solution. Maedica (Buchar) 2022; 17 (02) 415-419
    PubMedGoogle Scholar
  • 12 Emamaullee J, Zaidi AN, Schiano T. et al. Fontan-associated liver disease: screening, management, and transplant considerations. Circulation 2020; 142 (06) 591-604
    CrossrefPubMedGoogle Scholar
  • 13 Maruyama H, Yokosuka O. Ultrasonography for noninvasive assessment of portal hypertension. Gut Liver 2017; 11 (04) 464-473
    CrossrefPubMedGoogle Scholar
  • 14 de Franchis R, Faculty BV. 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
  • 15 de Franchis R. Updating consensus in portal hypertension: report of the Baveno III Consensus Workshop on definitions, methodology and therapeutic strategies in portal hypertension. J Hepatol 2000; 33 (05) 846-852
    CrossrefPubMedGoogle Scholar
  • 16 Groszmann RJ, Bosch J, Grace ND. et al. Hemodynamic events in a prospective randomized trial of propranolol versus placebo in the prevention of a first variceal hemorrhage. Gastroenterology 1990; 99 (05) 1401-1407
    CrossrefPubMedGoogle Scholar
  • 17 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
  • 18 Giefer MJ, Murray KF, Colletti RB. Pathophysiology, diagnosis, and management of pediatric ascites. J Pediatr Gastroenterol Nutr 2011; 52 (05) 503-513
    CrossrefPubMedGoogle Scholar
  • 19 Karnsakul W, Ingviya T, Seaberg E. et al. Ascites in children: a single-center experience of 27 years. J Pediatr Gastroenterol Nutr 2017; 64 (01) 83-88
    CrossrefPubMedGoogle Scholar
  • 20 Mohammad S, Kaurs E, Aguirre VP, Varni JW, Alonso EM. Health-related quality of life in infants with chronic liver disease. J Pediatr Gastroenterol Nutr 2016; 62 (05) 751-756
    CrossrefPubMedGoogle Scholar
  • 21 Pugliese R, Fonseca EA, Porta G. et al. Ascites and serum sodium are markers of increased waiting list mortality in children with chronic liver failure. Hepatology 2014; 59 (05) 1964-1971
    CrossrefPubMedGoogle Scholar
  • 22 Mahajan S, Lal BB, Sood V, Khillan V, Khanna R, Alam S. Difficult-to-treat ascetic fluid infection is a predictor of transplant-free survival in childhood decompensated chronic liver disease. Indian J Gastroenterol 2020; 39 (05) 465-472
    CrossrefPubMedGoogle Scholar
  • 23 Bolia R, Srivastava A, Marak R, Yachha SK, Poddar U. Prevalence and impact of bacterial infections in children with liver disease - a prospective study. J Clin Exp Hepatol 2018; 8 (01) 35-41
    CrossrefPubMedGoogle Scholar
  • 24 Sharma P, Sharma BC, Puri V, Sarin SK. Minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction. Am J Gastroenterol 2008; 103 (06) 1406-1412
    CrossrefPubMedGoogle Scholar
  • 25 Srivastava A, Chaturvedi S, Gupta RK. et al. Minimal hepatic encephalopathy in children with chronic liver disease: prevalence, pathogenesis and magnetic resonance-based diagnosis. J Hepatol 2017; 66 (03) 528-536
    CrossrefPubMedGoogle Scholar
  • 26 Pinto RB, Schneider AC, da Silveira TR. Cirrhosis in children and adolescents: an overview. World J Hepatol 2015; 7 (03) 392-405
    CrossrefPubMedGoogle Scholar
  • 27 de Ville de Goyet J, Alberti D, Clapuyt P. et al. Direct bypassing of extrahepatic portal venous obstruction in children: a new technique for combined hepatic portal revascularization and treatment of extrahepatic portal hypertension. J Pediatr Surg 1998; 33 (04) 597-601
    CrossrefPubMedGoogle Scholar
  • 28 Chen VT, Wei J, Liu YC. A new procedure for management of extrahepatic portal obstruction. Proximal splenic-left intrahepatic portal shunt. Arch Surg 1992; 127 (11) 1358-1360
    CrossrefPubMedGoogle Scholar
  • 29 Chiu B, Pillai SB, Sandler AD, Superina RA. Experience with alternate sources of venous inflow in the Meso-Rex bypass operation: the coronary and splenic veins. J Pediatr Surg 2007; 42 (07) 1199-1202
    CrossrefPubMedGoogle Scholar
  • 30 Ateş O, Hakgüder G, Olguner M, Akgür FM. Extrahepatic portal hypertension treated by anastomosing inferior mesenteric vein to left portal vein at Rex recessus. J Pediatr Surg 2003; 38 (10) E10-E11
    CrossrefPubMedGoogle Scholar
  • 31 Lv Y, Pu L, Song J. et al. Meso-Rex bypass shunt vs. transposition shunt for cavernous transformation of portal vein in children. Front Pediatr 2022; 10: 935828
    CrossrefPubMedGoogle Scholar
  • 32 de Ville de Goyet J, Lo Zupone C, Grimaldi C. et al. Meso-Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: review and perspectives. Pediatr Transplant 2013; 17 (01) 19-26
    CrossrefPubMedGoogle Scholar
  • 33 Mack CL, Zelko FA, Lokar J. et al. Surgically restoring portal blood flow to the liver in children with primary extrahepatic portal vein thrombosis improves fluid neurocognitive ability. Pediatrics 2006; 117 (03) e405-e412
    CrossrefPubMedGoogle Scholar
  • 34 Chiu B, Superina R. Extrahepatic portal vein thrombosis is associated with an increased incidence of cholelithiasis. J Pediatr Surg 2004; 39 (07) 1059-1061
    CrossrefPubMedGoogle Scholar
  • 35 Sarin SK, Guptan RC, Malhotra S. Increased frequency of gallstones in cirrhotic and non-cirrhotic portal hypertension. J Assoc Physicians India 2002; 50: 518-522
    PubMedGoogle Scholar
  • 36 Chaudhary A, Dhar P, Sarin SK. et al. Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: surgical management. Br J Surg 1998; 85 (03) 326-329
    CrossrefPubMedGoogle Scholar
  • 37 Rangari M, Gupta R, Jain M, Malhotra V, Sarin SK. Hepatic dysfunction in patients with extrahepatic portal venous obstruction. Liver Int 2003; 23 (06) 434-439
    CrossrefPubMedGoogle Scholar
  • 38 de Ville de Goyet J, D'Ambrosio G, Grimaldi C. Surgical management of portal hypertension in children. Semin Pediatr Surg 2012; 21 (03) 219-232
    CrossrefPubMedGoogle Scholar
  • 39 Warren WD, Salam AA, Hutson D, Zeppa R. Selective distal splenorenal shunt. Technique and results of operation. Arch Surg 1974; 108 (03) 306-314
    CrossrefPubMedGoogle Scholar
  • 40 Moon SB, Jung SE, Ha JW, Park KW, Seo JK, Kim WK. The usefulness of distal splenorenal shunt in children with portal hypertension for the treatment of severe thrombocytopenia and leukopenia. World J Surg 2008; 32 (03) 483-487
    CrossrefPubMedGoogle Scholar
  • 41 Jenkins RL, Gedaly R, Pomposelli JJ, Pomfret EA, Gordon F, Lewis WD. Distal splenorenal shunt: role, indications, and utility in the era of liver transplantation. Arch Surg 1999; 134 (04) 416-420
    CrossrefPubMedGoogle Scholar
  • 42 Linton RR, Ellis DS, Geary JE. Critical comparative analysis of early and late results of splenorenal and direct portacaval shunts performed in 169 patients with portal cirrhosis. Ann Surg 1961; 154 (03) 446-459
    CrossrefPubMedGoogle Scholar
  • 43 Mitra SK, Rao KL, Narasimhan KL. et al. Side-to-side lienorenal shunt without splenectomy in noncirrhotic portal hypertension in children. J Pediatr Surg 1993; 28 (03) 398-401 , discussion 401–402
    CrossrefPubMedGoogle Scholar
  • 44 Sarfeh IJ, Rypins EB, Conroy RM, Mason GR. Portacaval H-graft: relationships of shunt diameter, portal flow patterns and encephalopathy. Ann Surg 1983; 197 (04) 422-426
    CrossrefPubMedGoogle Scholar
  • 45 Tainaka T, Hinoki A, Tanaka Y. et al. Long-term outcomes of the partial splenectomy for hypersplenism after portoenterostomy of patients with biliary atresia. Nagoya J Med Sci 2021; 83 (04) 765-771
    PubMedGoogle Scholar
  • 46 Chen J, Yu S, Xu L. Laparoscopic partial splenectomy: a safe and feasible treatment for splenic benign lesions. Surg Laparosc Endosc Percutan Tech 2018; 28 (05) 287-290
    CrossrefPubMedGoogle Scholar
  • 47 Amin MA, el-Gendy MM, Dawoud IE, Shoma A, Negm AM, Amer TA. Partial splenic embolization versus splenectomy for the management of hypersplenism in cirrhotic patients. World J Surg 2009; 33 (08) 1702-1710
    CrossrefPubMedGoogle Scholar
  • 48 Sugiura M, Futagawa S. A new technique for treating esophageal varices. J Thorac Cardiovasc Surg 1973; 66 (05) 677-685
    CrossrefPubMedGoogle Scholar
  • 49 Shah SR, Nagral SS, Mathur SK. Results of a modified Sugiura's devascularisation in the management of “unshuntable” portal hypertension. HPB Surg 1999; 11 (04) 235-239
    CrossrefPubMedGoogle Scholar
  • 50 Starzl TE, Marchioro TL, Vonkaulla KN, Hermann G, Brittain RS, Waddell WR. Homotransplantation of the liver in humans. Surg Gynecol Obstet 1963; 117: 659-676
    PubMedGoogle Scholar
  • 51 Bismuth H, Houssin D. Reduced-sized orthotropic liver graft in hepatic transplantation in children. Surgery 1984; 95 (03) 367-370
    PubMedGoogle Scholar
  • 52 Broelsch CE, Emond JC, Thistlethwaite JR, Rouch DA, Whitington PF, Lichtor JL. Liver transplantation with reduced-size donor organs. Transplantation 1988; 45 (03) 519-524
    CrossrefPubMedGoogle Scholar
  • 53 Pichlmayr R, Ringe B, Gubernatis G, Hauss J, Bunzendahl H. [Transplantation of a donor liver to 2 recipients (splitting transplantation) – a new method in the further development of segmental liver transplantation]. Langenbecks Arch Chir 1988; 373 (02) 127-130
    PubMedGoogle Scholar
  • 54 Bismuth H, Morino M, Castaing D. et al. Emergency orthotropic liver transplantation in two patients using one donor liver. Br J Surg 1989; 76 (07) 722-724
    CrossrefPubMedGoogle Scholar
  • 55 Raia S, Nery JR, Mies S. Liver transplantation from live donors. Lancet 1989; 2 (8661): 497
    CrossrefPubMedGoogle Scholar
  • 56 Strong RW, Lynch SV, Ong TH, Matsunami H, Koido Y, Balderson GA. Successful liver transplantation from a living donor to her son. N Engl J Med 1990; 322 (21) 1505-1507
    CrossrefPubMedGoogle Scholar
  • 57 McDiarmid SV, Anand R, Lindblad AS. Principal Investigators and Institutions of the Studies of Pediatric Liver Transplantation (SPLIT) Research Group. Development of a pediatric end-stage liver disease score to predict poor outcome in children awaiting liver transplantation. Transplantation 2002; 74 (02) 173-181
    CrossrefPubMedGoogle Scholar
  • 58 Mogul DB, Luo X, Bowring MG. et al. Fifteen-year trends in pediatric liver transplants: split, whole deceased, and living donor grafts. J Pediatr 2018; 196: 148-153.e2
    CrossrefPubMedGoogle Scholar
  • 59 McDiarmid SV, Merion RM, Dykstra DM, Harper AM. Selection of pediatric candidates under the PELD system. Liver Transpl 2004; 10 (10, Suppl 2): S23-S30
    CrossrefPubMedGoogle Scholar
  • 60 Goldaracena N, Echeverri J, Kehar M. et al. Pediatric living donor liver transplantation with large-for-size left lateral segment grafts. Am J Transplant 2020; 20 (02) 504-512
    CrossrefPubMedGoogle Scholar
  • 61 Ma N, Song Z, Dong C. et al. Risk factors of hepatic artery thrombosis in pediatric deceased donor liver transplantation. Pediatr Surg Int 2019; 35 (08) 853-859
    CrossrefPubMedGoogle Scholar
  • 62 Martinez M, Kang E, Beltramo F. et al. Vascular thrombosis after pediatric liver transplantation: Is prevention achievable?. J Liver Transpl 2023; 12: 100185
    CrossrefPubMedGoogle Scholar
  • 63 SRTR. Accessed January 16, 2024 at: https://srtr.transplant.hrsa.gov/annual_reports/2020/Liver.aspx
    PubMed
  • 64 Bowring MG, Massie AB, Chu NM. et al. Projected 20- and 30-year outcomes for pediatric liver transplant recipients in the United States. J Pediatr Gastroenterol Nutr 2020; 70 (03) 356-363
    CrossrefPubMedGoogle Scholar
  • 65 Shingina A, Vutien P, Uleryk E. et al. Long-term outcomes of pediatric living versus deceased donor liver transplantation recipients: a systematic review and meta-analysis. Liver Transpl 2022; 28 (03) 437-453
    CrossrefPubMedGoogle Scholar