title: Portal Cavernoma in the Era of Mesoportal Shunt (Rex) and Liver Transplant in Children.
abstract: OBJECTIVE
  The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only "curative" technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO.
  
  
  METHODS
  A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time.
  
  
  RESULTS
  Of the 73 children with EHPVO, 39 were operated (12 posttransplant and 27 idiopathic). The median age at surgery was 9.36 years (range, 1.60-17.42 years). The MPS was the technique of choice; it was offered in 21 patients but eventually could be performed in only 18 (9 posttransplant and 9 idiopathic). The results of MPS were better in idiopathic (just one thrombosis successfully converted into mesocaval bypass). In the MPS after LT (n = 9), six shunts are permeable, two became thrombotic (one patient requiring retransplantation), and one late thrombosis occurred and the patient died eventually because of gastrointestinal bleeding. In the remaining patients (21 out of the 39), MPS was not performed because of intrahepatic cavernoma, portal vein hypoplasia, or insufficient length of vascular graft (especially in left lateral segment graft LT, since the portal vein is usually located in the right upper quadrant). They underwent 10 distal splenorenal shunts (DSRS) (1 posttransplant and 9 idiopathic), 5 proximal splenorenal shunts (PSRS) (1/4), 6 mesocaval shunts (1/5), and 1 modified Sugiura procedure. The results with DSRS have been favorable (one thrombosis, converted into mesocaval bypass). In the PSRS no thrombosis was identified and in the mesocaval shunt one early thrombosis occurred. Posttransplantation group had higher risk of shunt thrombosis, regardless of the surgical technique (chi-square, 0.021). The total increase of platelets after 6 months was not different in MPS as compared with other surgical techniques (analysis of variance, 0.110).
  
  
  CONCLUSIONS
  The MPS is the technique of choice in EHPVO for idiopathic thrombosis as well as secondary to LT. Not all cases are favorable for MPS, so the surgeon must consider the possibility of alternative techniques for EHPVO. The results in terms of shunt patency are much better in idiopathic cavernoma that posttransplant patients.
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Gloria
authors.initials: G
authors.lastname: Chocarro
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Paloma Triana
authors.initials: PT
authors.lastname: Junco
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Eva
authors.initials: E
authors.lastname: Dominguez
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: María Virginia
authors.initials: MV
authors.lastname: Amesty
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Vanesa
authors.initials: V
authors.lastname: Nuñez Cerezo
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Hernandez
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Murcia
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Juan Antonio
authors.initials: JA
authors.lastname: Tovar
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Lopez Santamaria
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery*; D007223:Infant; D016031:Liver Transplantation; D008297:Male; D011169:Portal Vein / Q000601:surgery*; D011170:Portasystemic Shunt, Surgical / Q000379:methods*; D011183:Postoperative Complications / Q000601:surgery*; D012189:Retrospective Studies; D020246:Venous Thrombosis / Q000209:etiology / Q000601:surgery*