Congenital portosystemic shunt occlusion with an Amplatzer PFO occlusion device: a case report
Michelle M Shnayder1, Mario Dervishi2, Alexandria Jo3
1Department 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.
Insights
Congenital portosystemic shunts, rare vascular malformations, can be treated with endovascular embolization. An Amplatzer device successfully closed a shunt in a pediatric patient, offering a less invasive treatment option.
Area of Science:
- Vascular Surgery
- Pediatric Gastroenterology
- Interventional Radiology
Background:
- Congenital portosystemic shunts are rare embryological vascular malformations.
- These shunts divert portal venous flow, leading to significant morbidity and mortality.
- Complications include hepatic encephalopathy, hepatopulmonary syndrome, and pulmonary hypertension.
Purpose of the Study:
- To report the successful endovascular treatment of a pediatric patient with a congenital portosystemic shunt.
- To highlight a novel application of an Amplatzer patent foramen ovale occlusion device for shunt closure.
Main Methods:
- A two-year-old male presented with neurological symptoms and hyperammonemia.
- Diagnosis of an intrahepatic portosystemic shunt between the inferior vena cava and right portal vein was confirmed.
- Embolization was performed using an 18 mm Amplatzer patent foramen ovale occlusion device.
Main Results:
- Successful closure of the congenital portosystemic shunt was achieved.
- The endovascular approach provided rapid symptom relief.
- The Amplatzer device was effective for this specific shunt anatomy and type.
Conclusions:
- Congenital portosystemic shunts are rare and lack standardized treatment guidelines.
- Endovascular therapy offers a less invasive treatment option for selected patients.
- An Amplatzer PFO occlusion device presents a viable, novel method for shunt closure in appropriate cases.
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.
