TY - JOUR
T1 - Management and classification of type II congenital portosystemic shunts
AU - Lautz, Timothy B.
AU - Tantemsapya, Niramol
AU - Rowell, Erin
AU - Superina, Riccardo A.
PY - 2011/2/1
Y1 - 2011/2/1
N2 - 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). Conclusion: 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.
AB - 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). Conclusion: 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.
KW - Abernethy malformation
KW - Congenital portocaval shunt
KW - Congenital portosystemic shunt
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U2 - 10.1016/j.jpedsurg.2010.11.009
DO - 10.1016/j.jpedsurg.2010.11.009
M3 - Article
C2 - 21292079
AN - SCOPUS:79551574821
VL - 46
SP - 308
EP - 314
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 2
ER -