Abstract
BackgroundCongenital 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 presentationIn 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.ConclusionsPortosystemic 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.
Highlights
Congenital Portosystemic Shunts (CPSS) are rare developmental malformations that occur in 1 in 50,000 births. [1] Some patients remain asymptomatic and the presence of an aberrant malformation is incidentally identified on imaging
An Amplatzer Patent Foramen Ovale (PFO) occlusion device can provide a novel method of shunt closure given appropriate shunt type, size and anatomy
Case report A two-year-old male with history of Trisomy 21, atrial septal defect per echocardiogram, and autism spectrum disorder presented to the emergency department with abnormal chorea-like movements, altered mental status, anisocoria
Summary
Portosystemic shunts are a rare congenital abnormality without universal treatment guidelines.
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