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Published in: Indian Journal of Pediatrics 9/2017

01-09-2017 | Review Article

Extrahepatic Portal Venous Obstruction: What Should be the Mainstay of Treatment?

Authors: Richa Lal, Moinak Sen Sarma, Manish K. Gupta

Published in: Indian Journal of Pediatrics | Issue 9/2017

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Abstract

The two cornerstones of management for Extrahepatic portal vein obstruction (EHPVO) are endotherapy and surgery [Porto-systemic shunts (PSS)/Mesorex bypass (MRB)]. Endotherapy is the mainstay of treatment for acute variceal bleed control and has also been used extensively for secondary prophylaxis till variceal eradication is achieved. However, long-term follow-up beyond endoscopic eradication of esophageal varices (EEEV) indicates that there are numerous delayed bleed and non bleed sequelae of EHPVO, which merit surgery as a definitive procedure to decompress the hypertensive portal venous system. While endotherapy obliterates natural porto-systemic collaterals in the gastroesophageal region, persistently raised portal pressures manifest as an increase in secondary isolated gastric varices, ectopic varices, portal hypertensive vasculopathy, issues related to massive splenomegaly, portal biliopathy, growth retardation and hence impaired quality of life (QOL). An ideal management strategy should address both bleed and non-bleed consequences of EHPVO and translate into a near normal QOL. Further, MRB has opened up new dimensions to the management philosophy of EHPVO. This review article critically evaluates the role of surgery and endotherapy based on available literature and authors’ own experience.
Surgery and endotherapy are complementary. However, with increasing duration of follow-up post EEEV, it is evident that there is resurgence in the role of surgery (PSS/MRB) as a single one time definitive procedure for alleviating all bleed and delayed non bleed sequelae of EHPVO.
Surgery for EHPVO (PSS/MRB) should not be allowed to become a dying art and future generations of surgeons should continue to receive training in this specialized area of surgery.
Literature
1.
go back to reference Sarin SK, Sollano JD, Chawla YK, et al; Members of the APASL Working Party on Portal Hypertension. Consensus on extra-hepatic portal vein obstruction. Liver Int. 2006;26:512–9. Sarin SK, Sollano JD, Chawla YK, et al; Members of the APASL Working Party on Portal Hypertension. Consensus on extra-hepatic portal vein obstruction. Liver Int. 2006;26:512–9.
2.
go back to reference Thomas V, Jose T, Kumar S. Natural history of bleeding after esophageal variceal eradication in patients with extrahepatic portal venous obstruction: a 20 year follow up. Indian J Gastroenterol. 2009;28:206–11.CrossRefPubMed Thomas V, Jose T, Kumar S. Natural history of bleeding after esophageal variceal eradication in patients with extrahepatic portal venous obstruction: a 20 year follow up. Indian J Gastroenterol. 2009;28:206–11.CrossRefPubMed
3.
go back to reference Maksoud-Filho JG, Gonçalves MEP, Cardoso SR, Gibelli NEM, Tannuri U. Long term follow up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality. J Pediatr Surg. 2009;44:1877–83.CrossRefPubMed Maksoud-Filho JG, Gonçalves MEP, Cardoso SR, Gibelli NEM, Tannuri U. Long term follow up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality. J Pediatr Surg. 2009;44:1877–83.CrossRefPubMed
4.
go back to reference Zargar SA, Yattoo GN, Javid G, et al. Fifteen-year follow up of endoscopic injection sclerotherapy in children with extrahepatic portal venous obstruction. J Gastroenterol Hepatol. 2004;19:139–45.CrossRefPubMed Zargar SA, Yattoo GN, Javid G, et al. Fifteen-year follow up of endoscopic injection sclerotherapy in children with extrahepatic portal venous obstruction. J Gastroenterol Hepatol. 2004;19:139–45.CrossRefPubMed
5.
go back to reference Itha S, Yachha SK. Endoscopic outcome beyond esophageal variceal eradication in children with extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr. 2006;42:196–200.CrossRefPubMed Itha S, Yachha SK. Endoscopic outcome beyond esophageal variceal eradication in children with extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr. 2006;42:196–200.CrossRefPubMed
7.
go back to reference Alvarez F, Bernard O, Brunelle F, Hadchouel P, Odièvre M, Alagille D. Portal obstruction in children. II. Results of surgical portosystemic shunts. J Pediatr. 1983;103:703–7.CrossRefPubMed Alvarez F, Bernard O, Brunelle F, Hadchouel P, Odièvre M, Alagille D. Portal obstruction in children. II. Results of surgical portosystemic shunts. J Pediatr. 1983;103:703–7.CrossRefPubMed
8.
go back to reference Mitra SK, Rao KLN, Narasimhan KL, et al. Side-to-side lienorenal shunt without splenectomy in non cirrhotic portal hypertension in children. J Pediatr Surg. 1993;28:398–402.CrossRefPubMed Mitra SK, Rao KLN, Narasimhan KL, et al. Side-to-side lienorenal shunt without splenectomy in non cirrhotic portal hypertension in children. J Pediatr Surg. 1993;28:398–402.CrossRefPubMed
10.
go back to reference Orloff MJ, Orloff MS, Barbara G. Bleeding esophagogastric varices from extrahepatic portal hypertension: 40 years experience with porto-systemic shunt. J Am Coll Surg. 2002;194:717–28.CrossRefPubMed Orloff MJ, Orloff MS, Barbara G. Bleeding esophagogastric varices from extrahepatic portal hypertension: 40 years experience with porto-systemic shunt. J Am Coll Surg. 2002;194:717–28.CrossRefPubMed
11.
go back to reference 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;216:83–9.CrossRefPubMed 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;216:83–9.CrossRefPubMed
12.
go back to reference 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;63:1368–80. 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;63:1368–80.
13.
go back to reference Zuberi BF, Baloch Q. Comparison of endoscopic variceal sclerotherapy alone and in combination with octreotide in controlling acute variceal hemorrhage and early rebleeding in patients with low-risk cirrhosis. Am J Gastroenterol. 2000;95:768–71.CrossRefPubMed Zuberi BF, Baloch Q. Comparison of endoscopic variceal sclerotherapy alone and in combination with octreotide in controlling acute variceal hemorrhage and early rebleeding in patients with low-risk cirrhosis. Am J Gastroenterol. 2000;95:768–71.CrossRefPubMed
14.
go back to reference 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.CrossRefPubMed 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.CrossRefPubMed
15.
go back to reference Yachha SK, Ghoshal UC, Gupta R, Sharma BC, Ayyagari A. Portal hypertensive enteropathy in children with extrahepatic portal venous obstruction: role of variceal obliteration by endoscopic sclerotherapy and Helicobacter pylori infection. J Pediatr Gastroenterol Nutr. 1996;23:20–3. Yachha SK, Ghoshal UC, Gupta R, Sharma BC, Ayyagari A. Portal hypertensive enteropathy in children with extrahepatic portal venous obstruction: role of variceal obliteration by endoscopic sclerotherapy and Helicobacter pylori infection. J Pediatr Gastroenterol Nutr. 1996;23:20–3.
16.
go back to reference Chaudhary A, Aranya RC. Devascularization following endoscopic sclerotherapy of esophageal varices: dangers and difficulties. Br J Surg. 1991;78:1249–51.CrossRefPubMed Chaudhary A, Aranya RC. Devascularization following endoscopic sclerotherapy of esophageal varices: dangers and difficulties. Br J Surg. 1991;78:1249–51.CrossRefPubMed
17.
go back to reference Chaudhary A, Tatke M, Aranya RC. Endoscopic sclerotherapy: the far and near effects. Br J Surg. 1990;77:963.CrossRefPubMed Chaudhary A, Tatke M, Aranya RC. Endoscopic sclerotherapy: the far and near effects. Br J Surg. 1990;77:963.CrossRefPubMed
18.
go back to reference Yachha SK, Dhiman RK, Gupta R, Ghoshal UC. Endosonographic evaluation of the rectum in children with extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr. 1996;23:438–41.CrossRefPubMed Yachha SK, Dhiman RK, Gupta R, Ghoshal UC. Endosonographic evaluation of the rectum in children with extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr. 1996;23:438–41.CrossRefPubMed
19.
go back to reference Krishna YR, Yachha SK, Srivastava A, Negi D, Lal R, Poddar U. Quality of life in children managed for extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr. 2010;50:531–6.PubMed Krishna YR, Yachha SK, Srivastava A, Negi D, Lal R, Poddar U. Quality of life in children managed for extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr. 2010;50:531–6.PubMed
20.
go back to reference Gauthier-Villars M, Franchi S, Gauthier F, Fabre M, Pariente D, Bernard O. Cholestasis in children with portal vein obstruction. J Pediatr. 2005;146:568–73.CrossRefPubMed Gauthier-Villars M, Franchi S, Gauthier F, Fabre M, Pariente D, Bernard O. Cholestasis in children with portal vein obstruction. J Pediatr. 2005;146:568–73.CrossRefPubMed
21.
go back to reference Menon P, Rao KLN, Bhattacharya A, et al. Extrahepatic portal hypertension: quality of life and somatic growth after surgery. Eur J Pediatr Surg. 2005;15:82–7.CrossRefPubMed Menon P, Rao KLN, Bhattacharya A, et al. Extrahepatic portal hypertension: quality of life and somatic growth after surgery. Eur J Pediatr Surg. 2005;15:82–7.CrossRefPubMed
22.
go back to reference Sarin SK, Bansal A, Sasan S, Nigam A. Portal-vein obstruction in children leads to growth retardation. Hepatology. 1992;15:229–33.CrossRefPubMed Sarin SK, Bansal A, Sasan S, Nigam A. Portal-vein obstruction in children leads to growth retardation. Hepatology. 1992;15:229–33.CrossRefPubMed
23.
go back to reference Wani AH, Shah OJ, Zargar SA. Management of variceal hemorrhage in children with extrahepatic portal venous obstruction-shunt surgery versus endoscopic sclerotherapy. Indian J Surg. 2011;73:409–13.CrossRefPubMedPubMedCentral Wani AH, Shah OJ, Zargar SA. Management of variceal hemorrhage in children with extrahepatic portal venous obstruction-shunt surgery versus endoscopic sclerotherapy. Indian J Surg. 2011;73:409–13.CrossRefPubMedPubMedCentral
24.
go back to reference Rao KLN, Goyal A, Menon P. Extrahepatic portal hypertension in children: observations on three surgical procedures. Pediatr Surg Int. 2004;20:679–84.CrossRefPubMed Rao KLN, Goyal A, Menon P. Extrahepatic portal hypertension in children: observations on three surgical procedures. Pediatr Surg Int. 2004;20:679–84.CrossRefPubMed
25.
go back to reference de Franchis R. Evolving consensus in portal hypertension. Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol. 2005;43:167–76.CrossRefPubMed de Franchis R. Evolving consensus in portal hypertension. Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol. 2005;43:167–76.CrossRefPubMed
26.
go back to reference Sen Sarma M, Lal R, Yachha SK, Behari A, Srivastava A. Portosystemic shunt surgery for extrahepatic portal vein obstruction in children: shifting indications beyond endoscopic variceal eradication (abstract OP285). Abstracts of the 26th Annual Conference of APASL 2017. Hepatol Int. 2017;11:S181. Sen Sarma M, Lal R, Yachha SK, Behari A, Srivastava A. Portosystemic shunt surgery for extrahepatic portal vein obstruction in children: shifting indications beyond endoscopic variceal eradication (abstract OP285). Abstracts of the 26th Annual Conference of APASL 2017. Hepatol Int. 2017;11:S181.
28.
go back to reference 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;85:326–9.CrossRefPubMed 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;85:326–9.CrossRefPubMed
29.
go back to reference Kato T, Romero R, Koutouby R, et al. Portosystemic shunting in children during the era of endoscopic therapy: improved post operative growth parameters. J Pediatr Gastroenterol Nutr. 2000;30:419–25.CrossRefPubMed Kato T, Romero R, Koutouby R, et al. Portosystemic shunting in children during the era of endoscopic therapy: improved post operative growth parameters. J Pediatr Gastroenterol Nutr. 2000;30:419–25.CrossRefPubMed
30.
go back to reference Mehrotra RN, Bhatia V, Dabadghao P, Yachha SK. Extrahepatic portal vein obstruction in children: anthropometry, growth hormone and insulin like growth factor I. J Pediatr Gastroenterol Nutr. 1997;25:520–3.CrossRefPubMed Mehrotra RN, Bhatia V, Dabadghao P, Yachha SK. Extrahepatic portal vein obstruction in children: anthropometry, growth hormone and insulin like growth factor I. J Pediatr Gastroenterol Nutr. 1997;25:520–3.CrossRefPubMed
31.
go back to reference Mohapatra MK, Mohapatra AK, Acharya SK, Sahni P, Nundy S. Encephalopathy in patients with extrahepatic obstruction after lienorenal shunts. Br J Surg. 1992;79:1103–6.CrossRefPubMed Mohapatra MK, Mohapatra AK, Acharya SK, Sahni P, Nundy S. Encephalopathy in patients with extrahepatic obstruction after lienorenal shunts. Br J Surg. 1992;79:1103–6.CrossRefPubMed
32.
go back to reference Yadav SK, Srivastava A, Srivastava A, et al. Encephalopathy assessment in children with extrahepatic portal vein obstruction with MR, psychometry and critical flicker frequency. J Hepatol. 2010;52:348–54.CrossRefPubMed Yadav SK, Srivastava A, Srivastava A, et al. Encephalopathy assessment in children with extrahepatic portal vein obstruction with MR, psychometry and critical flicker frequency. J Hepatol. 2010;52:348–54.CrossRefPubMed
33.
go back to reference Srivastava A, Yadav SK, Lal R, et al. Effect of surgical portosystemic shunt on prevalence of minimal hepatic encephalopathy in children with extrahepatic portal venous obstruction: assessment by magnetic resonance imaging and psychometry. J Pediatr Gastroenterol Nutr. 2010;51:766–72.CrossRefPubMed Srivastava A, Yadav SK, Lal R, et al. Effect of surgical portosystemic shunt on prevalence of minimal hepatic encephalopathy in children with extrahepatic portal venous obstruction: assessment by magnetic resonance imaging and psychometry. J Pediatr Gastroenterol Nutr. 2010;51:766–72.CrossRefPubMed
34.
go back to reference Luoto T, Pakarinen M, Mattila I, Rintala R. Mesoportal bypass using a constructed saphenous vein graft for extrahepatic portal vein obstruction--technique, feasibility and outcome. J Pediatr Surg. 2012;47:688–93.CrossRefPubMed Luoto T, Pakarinen M, Mattila I, Rintala R. Mesoportal bypass using a constructed saphenous vein graft for extrahepatic portal vein obstruction--technique, feasibility and outcome. J Pediatr Surg. 2012;47:688–93.CrossRefPubMed
35.
go back to reference Guérin F, Bidault V, Gonzales E, Franchi-Abella S, De Lambert G, Branchereau S. Mesorex bypass for extrahepatic portal vein obstruction in children. Br J Surg. 2013;100:1606–13.CrossRefPubMed Guérin F, Bidault V, Gonzales E, Franchi-Abella S, De Lambert G, Branchereau S. Mesorex bypass for extrahepatic portal vein obstruction in children. Br J Surg. 2013;100:1606–13.CrossRefPubMed
36.
go back to reference Sharif K, McKiernan P, de Ville de Goyet J. Mesoportal bypass for extrahepatic portal vein obstruction in children: close to a cure for most! J Pediatr Surg. 2010;45:272–6.CrossRefPubMed Sharif K, McKiernan P, de Ville de Goyet J. Mesoportal bypass for extrahepatic portal vein obstruction in children: close to a cure for most! J Pediatr Surg. 2010;45:272–6.CrossRefPubMed
Metadata
Title
Extrahepatic Portal Venous Obstruction: What Should be the Mainstay of Treatment?
Authors
Richa Lal
Moinak Sen Sarma
Manish K. Gupta
Publication date
01-09-2017
Publisher
Springer India
Published in
Indian Journal of Pediatrics / Issue 9/2017
Print ISSN: 0019-5456
Electronic ISSN: 0973-7693
DOI
https://doi.org/10.1007/s12098-017-2390-5

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