Treatment Option for Abernethy Malformation-Two Cases Report and Review of the Literature

Frontiers in Pediatrics
Yuese LinLing Zhu

Abstract

Background: Abernethy malformation is a rare vascular anomaly of the portal venous system, which is also known as congenital portosystemic shunts (CPSS). The clinical manifestations of this anomaly can be serious, including hepatopulmonary syndrome(HPS), which can lead to significant hypoxemia and cyanosis. Case Presentation: This study reports two cases of patients with Abernethy Malformation. Case 1 was a 6-year-old boy whose blood oxygen saturation was 78%. Case 2 was a 6-year-old girl who had a history of open heart surgery and residual cardiac left to right shunt, whose blood oxygen saturation was 83%. These two children had unexplained cyanosis and were diagnosed with pulmonary arteriovenous fistula by contrast echocardiography with agitated saline. A selective retrograde catheter angiography confirmed the presence of a portosystemic shunt. Case 1 was a type I Abernethy malformation and did not receive any specific treatment and could only wait for liver transplantation. Case 2 was with type II Abernethy and underwent transcatheter closure of the CPSS. A 20mm-diameter, 14mm-long Vascular Plug (SHSMA Inc, Shanghai, China) was used to occlude the shunt. Results: In case 1, the boy developed deteriorating cyanosis and dyspne...Continue Reading

References

Mar 1, 1997·Journal of Pediatric Surgery·E R Howard, M Davenport
Jul 6, 2010·Journal of Pediatric Gastroenterology and Nutrition·Stéphanie Franchi-AbellaOlivier Bernard
Feb 5, 2011·Journal of Pediatric Surgery·Timothy B LautzRiccardo A Superina
May 17, 2011·Radiographics : a Review Publication of the Radiological Society of North America, Inc·Eduardo Alonso-GamarraManuel López-Santamaría
Jul 9, 2011·Pediatric Surgery International·Matthew PassalacquaHooman Yarmohammadi
Nov 30, 2012·Journal of Gastroenterology and Hepatology·Josephine A Grace, Peter W Angus
Nov 20, 2013·Catheterization and Cardiovascular Interventions : Official Journal of the Society for Cardiac Angiography & Interventions·Subramanian VenkateshwaranSivasubramonian Sivasankaran
Dec 31, 2015·Catheterization and Cardiovascular Interventions : Official Journal of the Society for Cardiac Angiography & Interventions·Walter KnirschOliver Kretschmar

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