Volume 63, Issue 6 pp. e141-e146
Original Article: Hepatology

Congenital Vascular Malformations of the Liver

An Association With Trisomy 21

Oliver C. Burdall

Oliver C. Burdall

Department of Paediatric Surgery, Kings College Hospital, London, UK

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Tassos Grammatikopoulos

Tassos Grammatikopoulos

Department of Paediatric Hepatology, Kings College Hospital, London, UK

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Maria Sellars

Maria Sellars

Department of Radiology, Kings College Hospital, London, UK

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Nedim Hadzic

Nedim Hadzic

Department of Paediatric Hepatology, Kings College Hospital, London, UK

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Mark Davenport

Corresponding Author

Mark Davenport

Department of Paediatric Surgery, Kings College Hospital, London, UK

Address correspondence and reprint requests to Prof Mark Davenport, ChM, Department of Paediatric Surgery, Kings College Hospital, London, SE5 9RS, UK (e-mail: [email protected]).Search for more papers by this author
First published: 01 December 2016
Citations: 13

The authors report no conflicts of interest.

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.

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