Congenital Vascular Malformations of the Liver: An Association With Trisomy 21. Issue 6 (December 2016)

• Record Type: Journal Article
• Authors: Burdall, Oliver C.; Grammatikopoulos, Tassos; Sellars, Maria; Hadzic, Nedim; Davenport, Mark
• Is Part Of: Journal of pediatric gastroenterology and nutrition. Volume 63:Issue 6(2016)
• Publication Date: 2016-12
• Subjects: Down syndrome -- hepatic arteriovenous malformation -- patent ductus venosus -- portocaval shunt -- trisomy 21; Children -- Nutrition -- Periodicals; Pediatric gastroenterology -- Periodicals; Infants -- Nutrition -- Periodicals; Nutrition disorders in children -- Periodicals; Child Nutrition -- Periodicals; Digestive System -- growth & development -- Periodicals; Gastrointestinal Diseases -- Periodicals; Infant Nutrition -- Periodicals; Nutrition Disorders -- Periodicals; Child; 618.923
• DOI: 10.1097/MPG.0000000000001405
• Languages: English
• ISSNs: 0277-2116

Abstract

Abstract : ABSTRACT: A link between congenital vascular malformation (CVM) of the liver and trisomy 21 has been suggested. We reviewed all children with trisomy 21 referred for investigation to a specialist pediatric hepatobiliary unit (1985–2015). Forty-five children with trisomy 21 were identified; 7 (15%) had a defined CVMs (4 girls). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection. Group (i) (n = 3): Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (n = 2). Group (ii) (n = 4): Involvement of all 3 vascular systems. Two infants had arterioportal hypertension caused by hepatic arteries feeding into a left portal vein aneurysm within the umbilical fissure. Two infants had more complex hepatic artery to hepatic vein shunts developing early cardiorespiratory failure with progressive jaundice. Our series shows a clear association between cardiac anomalies and CVM in children with trisomy 21.