title: Surgical strategy according to the anatomical types of congenital portosystemic shunts in children.
abstract: BACKGROUND
  Congenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV.
  
  
  METHODS
  Twenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test. CPSS were categorized into 5 types according to the anatomical shunt location.
  
  
  RESULTS
  The median age at diagnosis was 34months. Three of 23 total patients, who had an extrahepatic portosystemic shunt with a hypoplastic IHPV, died before treatment initiation because of severe PH. Fourteen cases received surgical or interventional treatment at the median age of 5years. A total of 6 cases received surgical therapy, including liver transplants for 2 absent IHPV cases. The remaining 8 cases received interventional coiling. All shunt ligations were successfully accomplished in 1-stage ligation without any complications. After the treatment, the hypoplastic IHPV gradually enlarged with an efficient portal inflow.
  
  
  CONCLUSIONS
  A precise pretreatment anatomical evaluation of CPSS and IHPV types is mandatory for the selection of surgical treatment.
  
  
  METHODS
  Diagnostic study - level II and treatment study - level III.
authors.affiliation: Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. Electronic address: matsuura@pedsurg.med.kyushu-u.ac.jp.
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Matsuura
authors.affiliation: Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Takahashi
authors.affiliation: Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Yanagi
authors.affiliation: Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Koichiro
authors.initials: K
authors.lastname: Yoshimaru
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Kenichiro
authors.initials: K
authors.lastname: Yamamura
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Eiji
authors.initials: E
authors.lastname: Morihana
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Hazumu
authors.initials: H
authors.lastname: Nagata
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Uike
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Takada
authors.affiliation: Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Taguchi
mesh_terms: D000792:Angiography; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000453:epidemiology / Q000517:prevention & control*; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000601:surgery; D011183:Postoperative Complications / Q000175:diagnosis / Q000453:epidemiology / Q000517:prevention & control*; D017410:Practice Guidelines as Topic; D015996:Survival Rate / Q000639:trends; D014057:Tomography, X-Ray Computed; D054079:Vascular Malformations / Q000175:diagnosis / Q000601:surgery*; D014656:Vascular Surgical Procedures / Q000379:methods / Q000592:standards*
keywords: Congenital portosystemic shunt; Interventional treatment; Surgical ligation