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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 Oct; 44(10):1877-83.JP

Abstract

BACKGROUND

Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up.

METHOD

We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction.

RESULTS

Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count.

CONCLUSION

Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence.

Authors+Show Affiliations

Pediatric Surgery Service, Instituto da Criança, University of São Paulo Medical School, São Paulo, Brazil. joao.maksoud@icr.usp.brNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

19853741

Citation

Maksoud-Filho, João Gilberto, et al. "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." Journal of Pediatric Surgery, vol. 44, no. 10, 2009, pp. 1877-83.
Maksoud-Filho JG, Gonçalves ME, Cardoso SR, et al. 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(10):1877-83.
Maksoud-Filho, J. G., Gonçalves, M. E., Cardoso, S. R., Gibelli, N. E., & Tannuri, U. (2009). 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. Journal of Pediatric Surgery, 44(10), 1877-83. https://doi.org/10.1016/j.jpedsurg.2009.02.074
Maksoud-Filho JG, et al. 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(10):1877-83. PubMed PMID: 19853741.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Maksoud-Filho,João Gilberto, AU - Gonçalves,Manuel Ernesto Peçanha, AU - Cardoso,Silvia Regina, AU - Gibelli,Nelson Elias Mendes, AU - Tannuri,Uenis, PY - 2008/12/22/received PY - 2009/02/13/revised PY - 2009/02/16/accepted PY - 2009/10/27/entrez PY - 2009/10/27/pubmed PY - 2009/12/16/medline SP - 1877 EP - 83 JF - Journal of pediatric surgery JO - J Pediatr Surg VL - 44 IS - 10 N2 - BACKGROUND: Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up. METHOD: We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction. RESULTS: Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count. CONCLUSION: Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence. SN - 1531-5037 UR - https://www.unboundmedicine.com/medline/citation/19853741/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_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022-3468(09)00155-9 DB - PRIME DP - Unbound Medicine ER -