Portal vascular anomalies in Down syndrome: spectrum of clinical presentation and management approach |
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Authors: | Nazar Golewale Steven J. Fishman Ahmad I. Alomari |
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Affiliation: | a Department of Radiology, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA b Department of Surgery, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA c Vascular Anomalies Center, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA |
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Abstract: | PurposeThe 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.MethodsWe 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.ResultsThree 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.ConclusionsThree 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. |
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Keywords: | Down syndrome Portal vein Vascular malformations Therapeutic embolization |
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