https://www.ncbi.nlm.nih.gov/pubmed/27602703

Burdall OC, Grammatikopoulos T, Sellars M, Hadzic N, Davenport M.
J Pediatr Gastroenterol Nutr. 2016 Sep 6.

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 paediatric hepatobiliary unit (1985-2015).Forty-five children with Trisomy 21 were identified; 7 (15%) had a defined CVMs (4 female). 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 veno-venous anomaly. This included porto-caval shunt and patent ductus venosus (n = 2).Group (ii) (n = 4) Involvement of all three 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 cardio-respiratory failure with progressive jaundice.Our series shows a clear association between cardiac anomalies and CVM in children with Trisomy 21.