Agenesis of the venous duct: two cases of extrahepatic drainage of the umbilical vein and extrahepatic portosystemic shunt with a review of the literature.
Agenesis of the venous duct is a rare congenital anomaly resulting in abnormal drainage of the umbilical vein into the foetal venous circulation. The clinical presentation and prognosis is variable, and may depend on the specific drainage pathways of the umbilical vein. We present two foetuses with agenesis of the venous duct, both associated with a postnatal portosystemic shunt, but with markedly different postnatal clinical courses. We also review all previously reported cases to better characterise this foetal disorder and the prognosis.
Department of Pediatric Cardiology,Children's Hospital of Wisconsin/Medical College of Wisconsin,Milwaukee,Wisconsin,United States of America.
Rohit S
RS
Loomba
Department of Pediatric Cardiology,Children's Hospital of Wisconsin/Medical College of Wisconsin,Milwaukee,Wisconsin,United States of America.
Michele
M
Frommelt
Department of Pediatric Cardiology,Children's Hospital of Wisconsin/Medical College of Wisconsin,Milwaukee,Wisconsin,United States of America.
David
D
Moe
Department of Pediatric Cardiology,Children's Hospital of Wisconsin/Medical College of Wisconsin,Milwaukee,Wisconsin,United States of America.
Amanda J
AJ
Shillingford
D000015:Abnormalities, Multiple; D000328:Adult; D000792:Angiography; D004452:Echocardiography; D005260:Female; D006325:Heart Atria / Q000002:abnormalities*; D006503:Hepatic Veins / Q000002:abnormalities; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology; D007231:Infant, Newborn; D011169:Portal Vein / Q000002:abnormalities*; D011247:Pregnancy; D016216:Ultrasonography, Prenatal; D014471:Umbilical Veins / Q000002:abnormalities*; D054079:Vascular Malformations / Q000150:complications / Q000175:diagnosis*; D014682:Vena Cava, Inferior / Q000002:abnormalities*; D055815:Young Adult
ductus venosus