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