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J Pediatr Intensive Care 2018; 07(01): 054-058
DOI: 10.1055/s-0037-1603822
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Transarterial and Transhepatic Endovascular Intervention to Alleviate Portal Hypertension Secondary to Arterioportal Fistula in a Trisomy 21 Infant

Mutlu Uysal Yazici
1   Department of Pediatric Intensive Care, Hacettepe University, School of Medicine, Ankara, Turkey
,
Barbaros Cil
2   Department of Radiology, Hacettepe University, School of Medicine, Ankara, Turkey
,
Benan Bayrakci
1   Department of Pediatric Intensive Care, Hacettepe University, School of Medicine, Ankara, Turkey
,
Necati Sasmaz
3   Department of Pediatric Gastroenterology, Hacettepe University, School of Medicine, Ankara, Turkey
,
Gokhan Baysoy
3   Department of Pediatric Gastroenterology, Hacettepe University, School of Medicine, Ankara, Turkey
,
Figen Gurakan
3   Department of Pediatric Gastroenterology, Hacettepe University, School of Medicine, Ankara, Turkey
› Author Affiliations
Further Information

Publication History

28 December 2016

10 May 2017

Publication Date:
21 June 2017 (online)

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Abstract

Individuals with trisomy 21 have an 80% risk reduction of vascular anomalies compared with general population. However, an association of trisomy 21 and portal vascular and arteriovenous anomalies has been defined in the literature. The primary hemodynamic abnormality in portal hypertension is increased resistance to portal blood flow. In various case reports in adults and pediatric age group patients, transarterial coil embolization of hepatoportal fistula was described. One of the authors of this article has previously reported successful treatment of congenital arterioportal fistula (APF) with percutaneous transhepatic liquid embolization in a patient who previously had transarterial coil embolization. To date, eight patients with trisomy 21 (Down syndrome) and congenital portosystemic shunts were reported of which four were treated with embolization. Here, we describe a 3-month-old infant with trisomy 21 and intrahepatic APF associated with extrahepatic portal hypertension and massive ascites. In the current report, a rare case of a patient with a diagnosis of trisomy 21 is discussed who was attempted to be treated with transarterial coil embolization and percutaneous transhepatic liquid embolization of the congenital APF in a single session.

Keywords

portal hypertension - arterioportal fistula - trisomy 21
 

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