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). 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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 308-314 |
| Number of pages | 7 |
| Journal | Journal of Pediatric Surgery |
| Volume | 46 |
| Issue number | 2 |
| DOIs | |
| State | Published - Feb 1 2011 |
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Keywords
- Abernethy malformation
- Congenital portocaval shunt
- Congenital portosystemic shunt
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Surgery
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Management and classification of type II congenital portosystemic shunts. / Lautz, Timothy B; Tantemsapya, Niramol; Rowell, Erin; Superina, Riccardo A.
In: Journal of Pediatric Surgery, Vol. 46, No. 2, 01.02.2011, p. 308-314.Research output: Contribution to journal › Article
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
UR - http://www.scopus.com/inward/record.url?scp=79551574821&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=79551574821&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2010.11.009
DO - 10.1016/j.jpedsurg.2010.11.009
M3 - Article
VL - 46
SP - 308
EP - 314
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 2
ER -