Document Detail

Management and classification of type II congenital portosystemic shunts.
MedLine Citation:
PMID:  21292079     Owner:  NLM     Status:  In-Data-Review    
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.
Timothy B Lautz; Niramol Tantemsapya; Erin Rowell; Riccardo A Superina
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  46     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-02-04     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  308-14     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 Elsevier Inc. All rights reserved.
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