Author of 1 Presentation
SS 11.1 - First report of the International Registry for Congenital Portosystemic Shunts
Abstract
Purpose
To describe patients followed by centres participating in the first International Registry for Congenital Portosystemic Shunts (IRCPSS).
Material and methods
Retrospective analysis of patients with CPSS from 15 centres. Porto-hepatic shunts were defined as intrahepatic (IH-CPSS) and others were defined as extrahepatic (EH-CPSS).
Results
242 patients were analysed with 122 IH-CPSS, 120 EH-CPSS. 24% were identified pre-natally with 75% IH-CPSS. 189 (76%) patients were diagnosed post-natally at a mean age of 39.1mo (0-200) for IH-CPSS and 61.9mo (0-192) for EH-CPSS. Both forms were equally frequent when diagnosed post-natally. Among them, symptoms were equally frequent among IH-CPSS (57%) or EH-CPSS (61%) CPSS. When comparing IH-CPSS and EH-CPSS, IH-CPSS were more likely to have neonatal hypoglycemia (9.8%vs 5%) and cholestasis (22.9% vs 15%) and EH-CPSS were more likely to have cardiopulmonary (33.3% vs 10.6%), neurocognitive (31.6% vs 9%) complications and liver nodules (39% vs 18%). Patients with EH-CPSS were more likely to have several symptoms than patients with IH-CPSS. Closure: 38.5% of IH-CPSS closed spontaneously vs 3% of EH-CPSS. When persistent, IH-CPSS were mostly treated using embolization and EH-CPSS using surgery with about 40% preventive closure. 8 patients had liver transplantation.
Conclusion
Prenatal diagnosis shows higher prevalence of IH-CPSS. Frequent spontaneous closure of IH-CPSS explains the equal frequency of both forms on post-natally diagnosed group. CPSS should be sought in infants with hypoglycemia or cholestasis and in children and adults with liver nodules, cardiopulmonary symptoms or neurocognitive deficits. IRCPSS will be helpful for further studies.
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Author of 1 Presentation
SS 11.1 - First report of the International Registry for Congenital Portosystemic Shunts (ID 460)
Abstract
Purpose
To describe patients followed by centres participating in the first International Registry for Congenital Portosystemic Shunts (IRCPSS).
Material and methods
Retrospective analysis of patients with CPSS from 15 centres. Porto-hepatic shunts were defined as intrahepatic (IH-CPSS) and others were defined as extrahepatic (EH-CPSS).
Results
242 patients were analysed with 122 IH-CPSS, 120 EH-CPSS. 24% were identified pre-natally with 75% IH-CPSS. 189 (76%) patients were diagnosed post-natally at a mean age of 39.1mo (0-200) for IH-CPSS and 61.9mo (0-192) for EH-CPSS. Both forms were equally frequent when diagnosed post-natally. Among them, symptoms were equally frequent among IH-CPSS (57%) or EH-CPSS (61%) CPSS. When comparing IH-CPSS and EH-CPSS, IH-CPSS were more likely to have neonatal hypoglycemia (9.8%vs 5%) and cholestasis (22.9% vs 15%) and EH-CPSS were more likely to have cardiopulmonary (33.3% vs 10.6%), neurocognitive (31.6% vs 9%) complications and liver nodules (39% vs 18%). Patients with EH-CPSS were more likely to have several symptoms than patients with IH-CPSS. Closure: 38.5% of IH-CPSS closed spontaneously vs 3% of EH-CPSS. When persistent, IH-CPSS were mostly treated using embolization and EH-CPSS using surgery with about 40% preventive closure. 8 patients had liver transplantation.
Conclusion
Prenatal diagnosis shows higher prevalence of IH-CPSS. Frequent spontaneous closure of IH-CPSS explains the equal frequency of both forms on post-natally diagnosed group. CPSS should be sought in infants with hypoglycemia or cholestasis and in children and adults with liver nodules, cardiopulmonary symptoms or neurocognitive deficits. IRCPSS will be helpful for further studies.