Congenital portosystemic shunt: our experience

Abstract

Introduction. Congenital portosystemic venous malformations are rare abnormalities in which the portal blood drains into a systemic vein and which are characterized by extreme clinical variability. Case Presentations. The authors present two case reports of a congenital extrahepatic portosystemic shunt (Type II). In the first patient, apparently nonspecific symptoms, such as headache and fatigue, proved to be secondary to hypoglycemic episodes related to the presence of a portosystemic shunt, later confirmed on imaging. During portal vein angiography, endovascular embolization of the portocaval fistula achieved occlusion of the anomalous venous tract. In the second patient, affected by Down's syndrome, the diagnosis of a portosystemic malformation was made by routine ultrasonography, performed to rule out concurrent congenital anomalies. Because of the absence of symptoms, we chose to observe this patient. Conclusions. These two case reports demonstrate the clinical heterogeneity of this malformation and the need for a multidisciplinary approach. As part of a proper workup, clinical evaluation must always be followed by radiographic diagnosis.

Figure 1

Contrast-enhanced CT images. Axial projection (a); sagittal-oblique projection (b). The arrows in (a) and (b) show a shunt between the posterior wall of the portal vein (PV), just before its intrahepatic hilar division, and the inferior cava vein (ICV); the intrahepatic portal branches appear reduced and filiform (arrowhead in (a)).

Figure 2

Axial contrast-enhanced CT images. The arrow shows the shunt between the portal vein (PV) and the inferior cava vein (ICV); at the hepatic hilum, the PV appears enlarged with only one intrahepatic portal branch (arrowhead).