The role of variceal bleeding primary prophylaxis in the management of extrahepatic portal vein obstruction in children




children, portal hypertension, liver, hepatocytes, bleeding, shunt surgery


Extrahepatic portal vein obstruction is a major cause of pediatric symptomatic portal hypertension and can lead to profuse variceal hemorrhaging.

Purpose - to evaluate the role of primary prophylaxis of the variceal bleeding in children with extrahepatic portal vein obstruction.

Materials and methods. A one-center prospective cohort clinical study involved 120 patients with extrahepatic portal vein obstruction who underwent primary or secondary variceal bleeding prophylaxis in 2016-2021. The laboratory, ultrasound, and endoscopy data before and after treatment were collected and evaluated.

Results. Variceal bleeding episodes occurred in 5.8% (n=3) of patients who underwent primary prophylaxis and rebleeding occurred in 27.9% (n=19) of those who underwent secondary prophylaxis. Bleeding episodes occurred less frequently in patients from primary prophylaxis group (p=0.013). In patients treated endoscopically (n=53, 44.17%) Variceal bleeding appeared less frequently (n=3, 5.66%) after treatment compared to patients who underwent surgery (n=67 (55.83%), 19 had rebleeding (28.35%)) (p=0.001). In 69.17% patients (n=83) esophageal varices eradication was achieved: in 53.01% (n=44) patients from the primary prophylaxis group and 46.99% (n=39) from the secondary prophylaxis group. Bleeding episodes occurred less frequently after the eradication achievement (p<0.001). The primary prophylaxis led to varices eradication more often than secondary (p=0.003). The varices recurrence episodes rates after the eradication achievement were not different between groups (p=0.51).

Conclusions. Primary prophylaxis can reduce the bleeding risk in extrahepatic portal vein obstruction with high risk of variceal bleeding. The prophylaxis by endoscopic variceal banding is an important bridge in the treatment for pediatric patients with portal hypertension that along with portosystemic shunting could potentially improve prophylactic treatment results.

The Committee on Clinical Investigation of Bogomolets National Medical University approved this study (Protocol No.141, 27.01.2021). All the studies were conducted according to implemented guidelines in consideration of GCP-ICH and Declaration of Helsinki. The written informed consent of all participants’ parents/guardians was achieved.

No conflict of interests was declared by the authors.

Author Biography

O.S. Godik, Bogomolets National Medical University, Kyiv

National Specialized Children’s Hospital “OKHMATDYT”, Kyiv, Ukraine


Abby Philips C, Sahney A. (2016). Oesophageal and gastric varices: historical aspects, classification and grading: everything in one place. Gastroenterology report. 4 (3): 186-195.; PMid:27324725 PMCid:PMC4976684

Attard TM et al. (2017). Readmission after Gastrointestinal Bleeding in Children: A Retrospective Cohort Study. The Journal of pediatrics. 184: 106-113.e4.; PMid:28237379

Benzamin Md, Mazumder W, Begum F, Khadga M, Ferdous Z, Rukunuzzaman Md, Karim ASM. (2018). Extra Hepatic Portal Hypertension with Clubbing, Cyanosis, Chest Deformity-A Case Report. doi: 10.21276/sjmcr.2018.6.11.19.

Cardey J et al. (2019). Screening of esophageal varices in children using esophageal capsule endoscopy: a multicenter prospective study. Endoscopy. 51 (1): 10-17.; PMid:30184608

Celińska-Cedro D, Teisseyre M, Woynarowski M, Socha P, Socha J, Ryzko J. (2003, Jul). Endoscopic ligation of esophageal varices for prophylaxis of first bleeding in children and adolescents with portal hypertension: preliminary results of a prospective study. J Pediatr Surg. 38 (7): 1008-1011.; PMid:12861528

De Franchis R et al. (2022). Baveno VII - Renewing consensus in portal hypertension. Journal of hepatology. 76 (4): 959-974.; PMid:35120736

De Franchis R, Baveno VI F. (2015). Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension. Journal of hepatology. 63 (3): 743-752.; PMid:26047908

De Franchis R, Bosch J, Garcia-Tsao G, Reiberger T, Ripoll C, Baveno VII Faculty. (2022, Apr). Baveno VII - Renewing consensus in portal hypertension. J Hepatol. 76 (4): 959-974. Epub 2021 Dec 30. doi: 10.1016/j.jhep.2021.12.022. Erratum in: J Hepatol. 2022 Apr 14. PMID: 35120736.

De Vries W, de Langen ZJ, Aronson DC et al. (2011). Mortality of biliary atresia in children not undergoing liver transplantation in the Netherlands. Pediatr Transplant. 15: 176-183.; PMid:21199212

Desai MS, Zainuer S, Kennedy C et al. (2011). Cardiac structural and functional alterations in infants and children with biliary atresia, listed for liver transplantation. Gastroenterology. 141: 1264-1272.; PMid:21762660 PMCid:PMC3740524

Duche M, Ducot B, Ackermann O, Guerin F, Jacquemin E, Bernard O. (2017). Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding. J of Hepatol. 66: 320-327.; PMid:27663417

Eroglu Y, Emerick KM, Whitingon PF et al. (2004). Octreotide therapy for control of acute gastrointestinal bleeding in children. J Pediatric Gastroenterology Nutr. 38: 41-47.; PMid:14676593

Gana JC, Valentino PL, Morinville V, O'Connor C, Ling SC. (2011). Variation in care for children with esophageal varices: a study of physicians', patients', and families' approaches and attitudes. Journal of pediatric gastroenterology and nutrition. 52 (6): 751-755.; PMid:21593647

Giouleme O, Theocharidou E. (2013). Management of Portal Hypertension in Children With Portal Vein Thrombosis. J Pediatric Gastroenterology Nutr. 57: 419-425.; PMid:23820400

Godik O, Diehtiarova D, Dubrovin A. (2022). Meso-Rex shunt for extrahepatic portal vein obstruction in children. International Journal of Surgery. 100: 106374.

Gonçalves ME, Cardoso SR, Maksoud JG. (2000, Mar). Prophylactic sclerotherapy in children with esophageal varices: long-term results of a controlled prospective randomized trial. J Pediatr Surg. 35 (3): 401-405.; PMid:10726678

Itha S, Yachha SK. (2006). Endoscopic outcome beyond esophageal variceal eradication in children with extrahepatic portal venous obstruction. J Pediatr Gastroenterol Nutr. 42: 196-200.; PMid:16456415

Khanna R, Sarin SK. (2014). Non-cirrhotic portal hypertension - diagnosis and management. J Hepatol. 60: 421-441.; PMid:23978714

Lee WS, Song ZL, Em JM, Chew KS, Ng RT. (2021, May). Role of primary prophylaxis in preventing variceal bleeding in children with gastroesophageal varices. Pediatr Neonatol. 62 (3): 249-257. Epub 2021 Jan 19.; PMid:33546933

Maksoud JG, Gonçalves ME, Porta G, Miura I, Velhote MC. (1991, Feb). The endoscopic and surgical management of portal hypertension in children: analysis of 123 cases. J Pediatr Surg. 26 (2): 178-181.; PMid:2023079

Oliveira APP, Ferreira AR, Fagundes EDT, Queiroz TCN, Carvalho SD, Neto JAF, Bittencourt PFS. (2020, Nov-Dec). Endoscopic prophylaxis and factors associated with bleeding in children with extrahepatic portal vein obstruction. J Pediatr (Rio J). 96 (6): 755-762. Epub 2019 Oct 27.; PMid:31666182 PMCid:PMC9432056

Parsons ЕC, Nelson R, Book LS et al. (2014). Renal replacement therapy in infants and children with hepatorenal syndrome awaiting liver transplantation: a case-control study. Liver Transpl. 20: 1468-1474.; PMid:25179803

Peck‐Radosavljevic M. (2017). Thrombocytopenia in chronic liver disease. Liver International. 37 (6): 778-793.; PMid:27860293

Pimenta JR, Ferreira AR, Fagundes EDT, Queiros TCN et al. (2017). Factors Associated With Bleeding Secondary to Rupture of Esophageal Varices in Children and Adolescents With Cirrhosis. J Pediatr Gastroenterol Nutr. 64: 44-48.; PMid:27496799

Reis dos Santos JM, Rodrigez Ferreira A, Tavares Fagundes ED et al. (2013). Endoscopic and Pharmacological Secondary Prophylaxis in Children and Adolescents With Esophageal Varices. J Pediatric Gastroenterology Nutr. 56: 93-98.; PMid:22785415

Sanada Y, Mizuta K, Urahashi T et al. (2011). Indication of liver transplantation for jaundice-free biliary atresia with portal hypertension. Ann Transplant. 16: 7-11.; PMid:22210415

Sarin SK, Kumar A. (1989). Gastric varices: profile, classification, and management. The American journal of gastroenterology. 84 (10): 1244-1249.

Shneider BL et al. (2012). 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. Pediatric transplantation. 16 (5): 426-437.; PMid:22409296

Wani ZA, Bhat RA, Bhadoria AS, Maiwall R. (2015, May-Jun). Extrahepatic portal vein obstruction and portal vein thrombosis in special situations: Need for a new classification. Saudi J Gastroenterol. 21 (3): 129-138.; PMid:26021771 PMCid:PMC4455142

Zargar SA, Javid G, Khan BA et al. (2002). Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction. Hepatology. 36: 666-672.; PMid:12198659






Original articles. Abdominal surgery