AI Article Synopsis
- Congenital portosystemic shunts (CPSS) are uncommon abnormalities that create a direct connection between the portal venous system and the systemic circulation.
- They are often discovered through prenatal screenings, during instances of neonatal cholestasis, or through serious complications like pulmonary hypertension and encephalopathy.
- Treatment typically involves interventional radiology to close the shunt, particularly if complications arise, it's extrahepatic, or it persists beyond two years, using a two-step procedure.
Article Abstract
Congenital portosystemic shunts (CPSS) are rare malformations, which connect the portal venous system and the systemic circulation. The disorder is discovered at prenatal screening, neonatal cholestasis as an incidental finding or by systemic complications such as pulmonary hypertension, encephalopathies or liver nodules. CPSS are associated to cardiac malformations and several syndromes. Intervention radiology plays a key role in treatment by closure of the shunt, which is indicated, if the patient has complications, the shunt is extrahepatic, or it is still persistent at two years of age, using a two-step approach, as summarised and discussed in this review.
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