| Portal vascular anomalies in Down syndrome: spectrum of clinical presentation and management approach. | |
| | |
MedLine Citation:
|
PMID: 20713219 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
PURPOSE: The occurrence of portal vascular anomalies in Down syndrome has been sporadically reported in the literature. These rare disorders have a wide spectrum of anatomical and clinical presentations. The aim of this communication was to describe the clinical course, imaging features, and management approaches in patients with this association. METHODS: We conducted a comprehensive search of the databases of the Vascular Anomalies Center and the Department of Radiology at Children's Hospital Boston for patients with Down syndrome and portal vascular anomalies. Medical records and imaging studies of varying modalities were reviewed. RESULTS: Three children with Down syndrome and portal anomalies (portosystemic shunt, simple arterioportal shunt, complex arterioportal shunt) were managed at our institution. The portosystemic shunt was clinically insignificant and resolved without any intervention. The simple arterioportal shunt was successfully treated with embolization. The complex arterioportal shunt was associated with major congenital cardiac defects and the child ultimately expired despite a decrease in the arterioportal shunting after embolization. CONCLUSIONS: Three is a wide spectrum of clinical and anatomical features of portal vascular shunts in Down syndrome. The management approach should be tailored based on the severity of symptoms. Percutaneous embolization can offer a safe, effective, and minimally invasive alternative to the surgical approach in selective cases. |
| | |
Authors:
|
Nazar Golewale; Harriet J Paltiel; Steven J Fishman; Ahmad I Alomari |
Related Documents
:
|
18454409 - Fibrotic racemose haemangioma of the retina. 11490339 - Unusual finding of cor triatriatum in a newborn with hypoplastic left heart syndrome. 16547749 - A novel form of ischio-vertebral syndrome. 3793859 - Oral and craniofacial morphology of a patient with larsen syndrome. 16949239 - Caudal regression syndrome associated with the white matter lesions and chromosome 18p1... 12116249 - Anorectal anomalies associated with or as part of other anomalies. 22021959 - A giant trichobezoar causing rapunzel syndrome in a 12-year-old female. 11730039 - Morphometric analysis of elastic skin fibres from patients with: cutis laxa, anetoderma... 16877859 - Jervell-lange nielsen syndrome in a family with the long qt syndrome (lqts). |
Publication Detail:
|
Type: Case Reports; Journal Article |
Journal Detail:
|
Title: Journal of pediatric surgery Volume: 45 ISSN: 1531-5037 ISO Abbreviation: J. Pediatr. Surg. Publication Date: 2010 Aug |
Date Detail:
|
Created Date: 2010-08-17 Completed Date: 2010-12-07 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 0052631 Medline TA: J Pediatr Surg Country: United States |
Other Details:
|
Languages: eng Pagination: 1676-81 Citation Subset: IM |
Copyright Information:
|
Copyright 2010 Elsevier Inc. All rights reserved. |
Affiliation:
|
Department of Radiology, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Down Syndrome
/
genetics,
pathology* Embolization, Therapeutic / methods* Female Humans Infant Male Portal System / abnormalities*, pathology Portal Vein / abnormalities*, pathology Treatment Outcome Vascular Malformations / diagnosis*, pathology, therapy* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Biliary reconstruction in pediatric live donor liver transplantation: duct-to-duct or Roux-en-Y hepa...
Next Document: Institutional experience with laparoscopic partial splenectomy for hereditary spherocytosis.