Evolution of congenital malformations of the umbilical-portal-hepatic venous system.
OBJECTIVE
The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life.


METHODS
All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution.


RESULTS
Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic.


CONCLUSIONS
Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants.
Service de chirurgie pédiatrique, Hôpital de La Timone, 13385 Marseille, France. aurelien.scalabre@gmail.com
Aurelien
A
Scalabre
Guillaume
G
Gorincour
Geraldine
G
Hery
Marc
M
Gamerre
Jean-Michel
JM
Guys
Pascal
P
de Lagausie
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000601:surgery / Q000628:therapy*; D000783:Aneurysm / Q000151:congenital / Q000196:embryology; D001163:Arteriovenous Anastomosis / Q000601:surgery; D005260:Female; D006345:Heart Septal Defects, Ventricular; D006498:Hepatectomy; D006503:Hepatic Veins / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000517:prevention & control; D007231:Infant, Newborn; D008026:Ligation; D008297:Male; D011168:Portal System / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000196:embryology / Q000601:surgery; D011169:Portal Vein / Q000002:abnormalities* / Q000196:embryology; D011172:Portography; D012075:Remission, Spontaneous; D018608:Ultrasonography, Doppler; D016216:Ultrasonography, Prenatal; D014472:Umbilicus / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000196:embryology / Q000601:surgery; D014551:Urinary Tract / Q000002:abnormalities; D014682:Vena Cava, Inferior / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D020246:Venous Thrombosis / Q000151:congenital / Q000209:etiology / Q000601:surgery; D015017:Yolk Sac / Q000098:blood supply