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Case Reports in Pediatrics
Volume 2015 (2015), Article ID 691618, 5 pages
Case Report

Congenital Portosystemic Shunt: Our Experience

1Unit of Clinical Pediatrics, Department of Medical and Pediatric Sciences, University of Catania, Via Santa Sofia, 95123 Catania, Italy
2Radiodiagnostic and Oncological Radiotherapy Unit, University Hospital “Policlinico-Vittorio Emanuele”, Via Santa Sofia, 95123 Catania, Italy

Received 7 November 2014; Revised 9 January 2015; Accepted 12 January 2015

Academic Editor: Josef Sykora

Copyright © 2015 Tiziana Timpanaro et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.