Congenital Vascular Malformations of the Liver: An Association With Trisomy 21.
J Pediatr Gastroenterol Nutr
; 63(6): e141-e146, 2016 12.
Article
en En
| MEDLINE
| ID: mdl-27602703
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) (nâ=â3) Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (nâ=â2). Group (ii) (nâ=â4) 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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Banco de datos:
MEDLINE
Asunto principal:
Síndrome de Down
/
Malformaciones Vasculares
/
Cardiopatías Congénitas
/
Venas Hepáticas
/
Hígado
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Female
/
Humans
/
Infant
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Male
/
Newborn
Idioma:
En
Año:
2016
Tipo del documento:
Article