Rare Case of Abdominal Aortic and Multiple Visceral Aneurysms in a Pediatric Patient With PIK3CA Mutation and Vasculitis

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  • Gomes, Vivian Carla
  • Parodi, Federico Ezequiel
    • Affiliation: School of Medicine, Department of Surgery
  • Wood, Jacob C.
    • Affiliation: School of Medicine, Department of Surgery
  • Motta, Fernando
    • Affiliation: School of Medicine, Department of Surgery
  • Farber, Mark A.
    • Affiliation: School of Medicine, Department of Surgery
Abstract
  • Abdominal aortic aneurysms (AAA) are most commonly observed in elderly male patients and are particularly rare in children. Among the pediatric population, they are usually diagnosed in the context of connective tissue disorders, genetic mutations, or vasculitis. The same is true of visceral arteries aneurysms. This case report describes the staged management of an 11-year-old patient presenting PIK3CA mutation and a 5.8 cm infrarenal AAA associated with bilateral common iliac arteries and multiple visceral aneurysms, the largest observed in the superior mesenteric artery (SMA = 3.2 cm). After careful evaluation, decision was made to first approach the most life-threatening lesion (the infrarenal AAA due to the large diameter) and the remaining aneurysms in secondary procedures, with special attention to the SMA aneurysm. The patient underwent a staged repair, with the first phase consisting of an aortobi-iliac graft with the distal anastomosis made at the left common iliac artery and right external iliac artery. The right hypogastric artery was ligated. The second procedure consisted of SMA aneurysm repair with a plication technique, as 7 branches were visualized coming off the aneurysm sac. Postoperative pathology analysis of the aortic and SMA aneurysms sac revealed vasculitis with a mixed inflammatory pattern and a COL3A1 gene heterozygote variant. He is currently in his 18th month after the last surgical intervention, receiving immunomodulatory therapy, with a planned follow-up by the interdisciplinary team to monitor the medications’ side effects and the diameter of the remaining visceral aneurysms.
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  • Article
Rights statement
  • In Copyright
Rights holder
  • SAGE Publications
License
  • Attribution-NonCommercial 4.0 International
Journal title
  • Vascular and Endovascular Surgery
Journal volume
  • 58
Journal issue
  • 4
Page start
  • 387
Page end
  • 391
ISSN
  • 1538-5744
  • 1938-9116
Copyright date
  • 2023
Publisher
  • SAGE Publications

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