Congenital portosystemic shunt: our experience

Area of Science:

• Vascular Surgery
• Pediatric Surgery
• Radiology

Background:

• Congenital portosystemic venous malformations (CPSVMs) are rare vascular anomalies.
• These shunts involve abnormal drainage of portal blood into systemic veins.
• CPSVMs exhibit significant clinical variability.

Purpose of the Study:

• To present two case reports of congenital extrahepatic portosystemic shunts (Type II).
• To illustrate the diverse clinical presentations and diagnostic challenges of CPSVMs.
• To emphasize the importance of a multidisciplinary approach in managing these rare conditions.

Main Methods:

• Case report 1: Endovascular embolization of a portocaval fistula.
• Case report 2: Diagnosis via routine ultrasonography in a patient with Down's syndrome.
• Both cases involved radiographic confirmation of the portosystemic shunt.

Main Results:

• Patient 1 presented with nonspecific symptoms secondary to hypoglycemia, successfully treated with embolization.
• Patient 2, asymptomatic, was diagnosed incidentally and managed conservatively.
• The cases highlight the wide spectrum of clinical manifestations.

Conclusions:

• Congenital portosystemic shunts demonstrate extreme clinical heterogeneity.
• A multidisciplinary approach is crucial for effective management.
• Radiographic diagnosis is essential following clinical evaluation for suspected CPSVMs.