You Are Too Sweet: Endovascular Embolization of Congenital Porto Systemic Shunt
Year: 2021
Type: conference-paper
Study design: Case Report
Abstract: Background: Congenital porto systemic shunt (CPSS) can present with hyperammonemia, hepatopulmonary syndrome, and porto-pulmonary hypertension. CPSS can be intrahepatic or extrahepatic. Severe persistent hypoglycemia is one of the rare manifestations of CPSS which is hypothesized due to decreased clearance of insulin in the liver. Closure of CPSS should be considered for all symptomatic extrahepatic or intrahepatic shunt. Materials and Methods: A 12-day-old neonate (weighing 3.6 kg) presented to us with complaints of seizures, persistent hyperinsulinemic hypoglycemia, unresponsive to IV diazoxide, hydrocortisone, and octreotide. On evaluation, she had congenital porto-systemic shunt (patent ductus venosus) associated with dilated right atrium, right ventricle, severe pulmonary hypertension, and severe tricuspid regurgitation. Due to severe persistent hypoglycemia and associated severe pulmonary hypertension, endovascular embolization of CPSS was suggested. Under general anesthesia, right jugular vein access was secured with 5-F long sheath. A buddy wire was placed in the infrarenal IVC for stability of sheath since the distal end of porto-systemic shunt was close to cavoatrial junction. Under ultrasound and fluoroscopy guidance, the shunt was cannulated using triaxial system of 4-F multipurpose catheter-progreat microcatheter–microwire and obliterated using Amplatzer IV vascular plug. Results: Following the procedure, serum insulin level decreased and the hypoglycemic episodes reduced, requiring minimal medications. Follow-up echo showed resolution of pulmonary hypertension. On further evaluation, the neonate had underlying genetic mutation (ABCC8) positive for familial hyperinsulinemic hypoglycemia which contributed as a synergistic factor Conclusion: Neonates with persistent and treatment resistant hyperinsulinemic hypoglycemia should be screened for CPSS as a causative or synergistic factor which can be treated by endovascular embolization. Buddy wire in the IVC offers stability of sheath during placement of vascular plug and USG guidance during placement of vascular plug offers optimal positioning. Publication History Article published online: 14 December 2021 © 2021. The Pan Arab Interventional Radiology Society. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/) Thieme Medical and Scientific Publishers Pvt. Ltd. A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
Authors: Karthikeyan M. Athiyappan, Mathew Cherian, Pankaj Mehta, Santhosh Poyyamoli, Nikhil H. Reddy, Akhil Monga
Institution: Kovai Medical Center and Hospital
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4210384111