title: Management and classification of type II congenital portosystemic shunts.
abstract: BACKGROUND
  Congenital portosystemic shunts (PSS) with preserved intrahepatic portal flow (type II) present with a range of clinical signs. The indications for and benefits of repair of PSS remain incompletely understood. A more comprehensive classification may also benefit comparative analyses from different institutions.
  
  
  METHODS
  All children treated at our institution for type II congenital PSS from 1999 through 2009 were reviewed for presentation, treatment, and outcome.
  
  
  RESULTS
  Ten children (7 boys) with type II PSS were identified at a median age of 5.5 years. Hyperammonemia with varying degrees of neurocognitive dysfunction occurred in 80%. The shunt arose from a branch of the portal vein (type IIa; n = 2), from the main portal vein (type IIb; n = 7), or from a splenic or mesenteric vein (type IIc; n = 1). Management included operative ligation (n = 6), endovascular occlusion (n = 3), or a combined approach (n = 1). Shunt occlusion was successful in all cases. Serum ammonia decreased from 130 ± 115 μmol/L preoperatively to 31 ± 15 μmol/L postoperatively (P = .03). Additional benefits included resolution of neurocognitive dysfunction (n = 3), liver nodules (n = 1), and vaginal bleeding (n = 1).
  
  
  CONCLUSIONS
  Correction of type II PSS relieves a wide array of symptoms. Surgery is indicated for patients with clinically significant shunting. A refined classification system will permit future comparison of patients with similar physiology.
authors.affiliation: Department of Surgery, Children's Memorial Hospital, Feinberg School of Medicine of Northwestern University, Box 57, Chicago, IL 60614, USA.
authors.forename: Timothy B
authors.initials: TB
authors.lastname: Lautz
authors.forename: Niramol
authors.initials: N
authors.lastname: Tantemsapya
authors.forename: Erin
authors.initials: E
authors.lastname: Rowell
authors.forename: Riccardo A
authors.initials: RA
authors.lastname: Superina
mesh_terms: D000015:Abnormalities, Multiple / Q000145:classification / Q000601:surgery; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D057510:Endovascular Procedures / Q000379:methods; D005260:Female; D006501:Hepatic Encephalopathy / Q000145:classification / Q000601:surgery; D006801:Humans; D022124:Hyperammonemia / Q000145:classification / Q000601:surgery; D007223:Infant; D008026:Ligation / Q000379:methods; D008297:Male; D008642:Mesenteric Veins / Q000002:abnormalities / Q000601:surgery; D011168:Portal System / Q000002:abnormalities* / Q000601:surgery*; D011169:Portal Vein / Q000002:abnormalities* / Q000601:surgery; D013162:Splenic Vein / Q000002:abnormalities / Q000601:surgery; D013577:Syndrome; D016157:Vascular Fistula / Q000145:classification / Q000151:congenital / Q000601:surgery*