Evolution of congenital malformations of the umbilical-portal-hepatic venous system

Area of Science:

• Medical Imaging
• Pediatric Surgery
• Vascular Malformations

Background:

• Congenital malformations of the umbilical-portal-hepatic venous system are rare.
• Prenatal diagnosis is uncommon, and postnatal prognosis is not well-established.
• These anomalies involve the network of veins connecting the umbilical vein, portal vein, and hepatic veins.

Purpose of the Study:

• To describe the clinical and radiological evolution of eight cases of congenital umbilical-portal-hepatic venous system malformations.
• To highlight the importance of postnatal follow-up for these rare conditions.

Main Methods:

• Systematic review of congenital malformations of the portal and hepatic venous system diagnosed prenatally or within the first month of life.
• Analysis of clinical features, imaging findings (primarily ultrasonography), and anatomical details.
• Focus on clinical and radiological evolution over time.

Main Results:

• Eight cases were studied, with 50% diagnosed prenatally.
• Reported malformations included four portosystemic shunts (three resolved spontaneously, one surgically treated), an asymptomatic anatomical variation, a vitelline vein aneurysm causing portal vein thrombosis (surgically treated), a complex venous malformation (surgically treated), and an asymptomatic persistent right umbilical vein.
• Varied clinical presentations and outcomes were observed, ranging from asymptomatic to requiring surgical intervention.

Conclusions:

• Prenatal diagnosis of these venous malformations is infrequent.
• Postnatal prognosis and management require careful clinical, biological, and radiological follow-up.
• Ultrasonography is crucial for distinguishing pathological malformations from normal anatomical variants.