title: Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children.
abstract: OBJECTIVE
  Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported.
  
  
  METHODS
  Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ~50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device.
  
  
  RESULTS
  Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications.
  
  
  CONCLUSIONS
  Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.
authors: Section of Pediatric Cardiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel. elchananb@bezeqint.net.
authors: Elchanan
authors: E
authors: Bruckheimer
authors: Section of Pediatric Cardiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
authors: Tamir
authors: T
authors: Dagan
authors: Section of Radiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
authors: Eli
authors: E
authors: Atar
authors: Section of Radiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
authors: Michael
authors: M
authors: Schwartz
authors: Section of Anesthesiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
authors: Ludmila
authors: L
authors: Kachko
authors: Section of Pediatric Cardiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
authors: Riccardo
authors: R
authors: Superina
authors: Section of Pediatric Cardiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
authors: Gabriel
authors: G
authors: Amir
authors: Section of Gastroenterology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
authors: Rivka
authors: R
authors: Shapiro
authors: Section of Pediatric Cardiology, Schneider Children's Medical Center Israel, Kaplan 14, Petach Tikva, Israel.
authors: Einat
authors: E
authors: Birk
mesh_terms: D000293:Adolescent; D015901:Angiography, Digital Subtraction / Q000379:methods; D017130:Angioplasty / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D022124:Hyperammonemia / Q000150:complications / Q000628:therapy; D006975:Hypertension, Portal / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D007223:Infant; D008297:Male; D011168:Portal System / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011169:Portal Vein / Q000002:abnormalities / Q000000981:diagnostic imaging; D011172:Portography / Q000379:methods; D015607:Stents; D016896:Treatment Outcome; D054079:Vascular Malformations / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*