Michelle M Shnayder1, Mario Dervishi2, Alexandria Jo3, Benjamin Pomerantz3. 1. Department of Radiology; Division of Vascular and Interventional Radiology, University of Michigan Health System, 1500 E. Medical Center Dr, B1D502, 48109-5030, Ann Arbor, MI, USA. shnayder@med.umich.edu. 2. American University of the Caribbean School of Medicine, Cupecoy, St. Maarten. 3. Department of Radiology; Division of Vascular and Interventional Radiology, University of Michigan Health System, 1500 E. Medical Center Dr, B1D502, 48109-5030, Ann Arbor, MI, USA.
Abstract
BACKGROUND: Congenital portosystemic shunts are embryological malformations in which portal venous flow is diverted to the systemic circulation. High morbidity and mortality are seen in patients with concurrent hepatic encephalopathy, hepatopulmonary syndrome, and pulmonary hypertension. Endovascular therapy, in the correct patient population, offers a less invasive method of treatment with rapid relief of symptoms. CASE PRESENTATION: In this report, we discuss the treatment of a two-year-old male with abnormal chorea-like movements, altered mental status, anisocoria and hyperammonemia diagnosed with an intrahepatic congenital portosystemic shunt between the inferior vena cava and right portal vein. Given the patient's amenable anatomy and shunt type, embolization was performed with an 18 mm Amplatzer patent foramen ovale occlusion device. CONCLUSIONS: Portosystemic shunts are a rare congenital abnormality without universal treatment guidelines. An Amplatzer PFO occlusion device can provide a novel method of shunt closure given appropriate shunt type, size and anatomy.
BACKGROUND: Congenital portosystemic shunts are embryological malformations in which portal venous flow is diverted to the systemic circulation. High morbidity and mortality are seen in patients with concurrent hepatic encephalopathy, hepatopulmonary syndrome, and pulmonary hypertension. Endovascular therapy, in the correct patient population, offers a less invasive method of treatment with rapid relief of symptoms. CASE PRESENTATION: In this report, we discuss the treatment of a two-year-old male with abnormal chorea-like movements, altered mental status, anisocoria and hyperammonemia diagnosed with an intrahepatic congenital portosystemic shunt between the inferior vena cava and right portal vein. Given the patient's amenable anatomy and shunt type, embolization was performed with an 18 mm Amplatzer patent foramen ovale occlusion device. CONCLUSIONS: Portosystemic shunts are a rare congenital abnormality without universal treatment guidelines. An Amplatzer PFO occlusion device can provide a novel method of shunt closure given appropriate shunt type, size and anatomy.
Authors: O Bernard; S Franchi-Abella; S Branchereau; D Pariente; F Gauthier; E Jacquemin Journal: Semin Liver Dis Date: 2013-02-08 Impact factor: 6.115
Authors: Christiane Sokollik; Robert H J Bandsma; Juan C Gana; Meta van den Heuvel; Simon C Ling Journal: J Pediatr Gastroenterol Nutr Date: 2013-06 Impact factor: 2.839