Define and classify the portosystemic shunts, based on their anatomy and embryology, and the characteristics of each different type. Describe the findings of CPSS using different imaging techniques, combined with clinical data. Explain the value of each technique and the role of the radiologist in the diagnosis and management of the patient.

Congenital portosystemic shunts (CPSS) are abnormal communications between the portomesenteric flow and the systemic venous system due to an anatomical anomaly, bypassing the hepatic acini [1]. They can be associated with other malformations: cardiac and heterotaxia being the most common (Fig. 2) [2]. CPSS are a very different condition than acquired portosystemic shunts, that arise due to hypertension in the portosystemic flow (in this case, the portal system drains into the IVC to palliate the negative effects of hypertension in its vascular bed) [2,3]. Classification...

Ultrasound (US) is the first line of diagnosis, as it is a noninvasive and radiation-free alternative. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) confirm the diagnosis and provide vascular information not available in a US examination. Finally, angiography can clarify cases in which the diagnosis is uncertain, especially angiography with occlusion test, and treat the shunt through an occlusive device placed in the shunt. Closing the shunt through interventional radiology is preferred whenever possible (Fig. 25). Imaging evaluation of CPSS: US (Fig. 21): In...

CPSS are abnormal communications between the portomesenteric and systemic circulations, which essentially makes them vascular malformations. The radiologist plays a key role in diagnosis, management, and follow-up, so the importance of suspecting it in children who present with nonspecific liver dysfunction is crucial to comfortably manage and diagnose it.

L. Riaza Martin; Barcelona/ES - nothing to disclose M. J. Moreno; Barcelona/ES - nothing to disclose E. Vazquez Mendez; Barcelona/ES - nothing to disclose I. Diez Miranda; Barcelona/ES - nothing to disclose L. Riera Soler; Barcelona/ES - nothing to disclose A. Coma; Barcelona/ES - nothing to disclose

Gallego C, Miralles M, Marín C, Muyor P, González G, García-Hidalgo E. Congenital Hepatic Shunts. RadioGraphics. 2004 May;24(3):755–72. The International Registry of Congenital Portosystemic Shunt members, Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, et al. Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol. 2018 Aug;43(8):2023–36. Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E. Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management. Semin Liver Dis. 2013 Feb 8;32(04):273–87. Kanazawa H, Nosaka S, Miyazaki O, Sakamoto S, Fukuda A,...