title: Results of a stepwise approach to extrahepatic portal vein obstruction in children.
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.
authors.affiliation: *Paediatric Surgery †Gastroenterology Unit ‡Liver Unit §Paediatric Hepatology, Gastroenterology, and Transplantation. Hospital Papa Giovanni XXIII, Bergamo, Italy.
authors.forename: Daniele
authors.initials: D
authors.lastname: Alberti
authors.forename: Mara
authors.initials: M
authors.lastname: Colusso
authors.forename: Maurizio
authors.initials: M
authors.lastname: Cheli
authors.forename: Paolo
authors.initials: P
authors.lastname: Ravelli
authors.forename: Amedeo
authors.initials: A
authors.lastname: Indriolo
authors.forename: Sergio
authors.initials: S
authors.lastname: Signorelli
authors.forename: Stefano
authors.initials: S
authors.lastname: Fagiuoli
authors.forename: Lorenzo
authors.initials: L
authors.lastname: D'Antiga
mesh_terms: D055011:Ablation Techniques; D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003131:Combined Modality Therapy; D003663:Decision Trees; D004724:Endoscopy; D005500:Follow-Up Studies; D006504:Hepatic Veno-Occlusive Disease / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000517:prevention & control; D007223:Infant; D007558:Italy; D011169:Portal Vein / Q000601:surgery*; D011170:Portasystemic Shunt, Surgical; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014648:Varicose Veins / Q000209:etiology; D055815:Young Adult
chemical_list: D000319:Adrenergic beta-Antagonists