BACKGROUND:: The management of extrahepatic portal vein obstruction (EHPVO) in children is controversial. We report our experience with a prospective evaluation of a stepwise protocol based on severity of portal hypertension and feasibility of mesoportal bypass (MPB). METHODS:: After diagnosis, children with EHPVO underwent surveillance endoscopies and received nonselective β-blockers (NSBBs) or endoscopic variceal obliteration (EVO) when large varices were detected. In patients who failed NSBBs and EVO, we considered MPB as first-line and shunts or transjugular intrahepatic portosystemic shunt (TIPS) as second-line options. RESULTS:: Sixty-five children, median age 12.5 (range 1.6-25.8), whose age at diagnosis was 3.5 (0.2-17.5) years, were referred to our unit. Forty-three (66%) had a neonatal illness, 36 (55%) an umbilical vein catheterisation. Thirty-two (49%) presented with bleeding at a median age of 3.8 years (0.5-15.5); during an 8.4-year follow-up period (1-16), 43 (66%) had a bleeding episode, 52 (80%) were started on NSBBs, 55 (85%) required EVO, and 33 (51%) required surgery or TIPS. The Rex recessus was patent in 24 of 54 (44%), negatively affected by a history of umbilical catheterisation (P=0.01). Thirty-four (53%) patients underwent a major procedure: MPB (13), proximal splenorenal (13), distal splenorenal (2), mesocaval shunt (3), TIPS (2), and OLT (1). At the last follow-up, 2 patients died, 53 of 57 (93%) are alive with bleeding control, 27 of 33 (82%) have a patent conduit. CONCLUSIONS:: Children with EHPVO have a high rate of bleeding episodes early in life. A stepwise approach comprising of medical, endoscopic, and surgical options provided excellent survival and bleeding control in this population.

Alberti, D., Colusso, M., Cheli, M., Ravelli, P., Indriolo, A., Signorelli, S., et al. (2013). Results of a stepwise approach to extrahepatic portal vein obstruction in children. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 57(5), 619-626 [10.1097/MPG.0b013e31829fad46].

Results of a stepwise approach to extrahepatic portal vein obstruction in children

Fagiuoli S;
2013

Abstract

BACKGROUND:: The management of extrahepatic portal vein obstruction (EHPVO) in children is controversial. We report our experience with a prospective evaluation of a stepwise protocol based on severity of portal hypertension and feasibility of mesoportal bypass (MPB). METHODS:: After diagnosis, children with EHPVO underwent surveillance endoscopies and received nonselective β-blockers (NSBBs) or endoscopic variceal obliteration (EVO) when large varices were detected. In patients who failed NSBBs and EVO, we considered MPB as first-line and shunts or transjugular intrahepatic portosystemic shunt (TIPS) as second-line options. RESULTS:: Sixty-five children, median age 12.5 (range 1.6-25.8), whose age at diagnosis was 3.5 (0.2-17.5) years, were referred to our unit. Forty-three (66%) had a neonatal illness, 36 (55%) an umbilical vein catheterisation. Thirty-two (49%) presented with bleeding at a median age of 3.8 years (0.5-15.5); during an 8.4-year follow-up period (1-16), 43 (66%) had a bleeding episode, 52 (80%) were started on NSBBs, 55 (85%) required EVO, and 33 (51%) required surgery or TIPS. The Rex recessus was patent in 24 of 54 (44%), negatively affected by a history of umbilical catheterisation (P=0.01). Thirty-four (53%) patients underwent a major procedure: MPB (13), proximal splenorenal (13), distal splenorenal (2), mesocaval shunt (3), TIPS (2), and OLT (1). At the last follow-up, 2 patients died, 53 of 57 (93%) are alive with bleeding control, 27 of 33 (82%) have a patent conduit. CONCLUSIONS:: Children with EHPVO have a high rate of bleeding episodes early in life. A stepwise approach comprising of medical, endoscopic, and surgical options provided excellent survival and bleeding control in this population.
Articolo in rivista - Articolo scientifico
children; extrahepatic portal vein onstruction; mesoportal bypass; portal hypertension; shunt surgery; transjugular intrahepatic portosystemic shunt; variceal bleeding;
English
619
626
8
Alberti, D., Colusso, M., Cheli, M., Ravelli, P., Indriolo, A., Signorelli, S., et al. (2013). Results of a stepwise approach to extrahepatic portal vein obstruction in children. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 57(5), 619-626 [10.1097/MPG.0b013e31829fad46].
Alberti, D; Colusso, M; Cheli, M; Ravelli, P; Indriolo, A; Signorelli, S; Fagiuoli, S; D'Antiga, L
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/353511
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