Conservative management by embolization of a ruptured renal arterio-venous malformation (AVM) in Hereditary Hemorrhagic Telangiectasia (HHT)

Romain L'Huillier; Gaële Pagnoux; Sophie Dupuis-Girod; Nicolas Stacoffe

doi:10.1186/s42155-024-00444-8

Conservative management by embolization of a ruptured renal arterio-venous malformation (AVM) in Hereditary Hemorrhagic Telangiectasia (HHT)

CVIR Endovasc. 2024 Mar 16;7(1):30. doi: 10.1186/s42155-024-00444-8.

Authors

Romain L'Huillier^{1

2

3}, Gaële Pagnoux⁴, Sophie Dupuis-Girod^{5

6}, Nicolas Stacoffe⁷

Affiliations

¹ Department of Diagnostic and Interventional Radiology, Hospices Civils de Lyon, University of Lyon, Pavillon B, Hôpital Edouard Herriot, 5 Place D'Arsonval, Lyon, 69003, France. romain.lhuillier@chu-lyon.fr.
² LabTAU - Inserm U1032, Lyon, 69003, France. romain.lhuillier@chu-lyon.fr.
³ The French Comprehensive Liver Center, Hospices Civils de Lyon, University of Lyon, Lyon, 69004, France. romain.lhuillier@chu-lyon.fr.
⁴ Department of Diagnostic and Interventional Radiology, Hospices Civils de Lyon, University of Lyon, Pavillon B, Hôpital Edouard Herriot, 5 Place D'Arsonval, Lyon, 69003, France.
⁵ Genetics Department and National, Hospices Civils de Lyon, HHT Reference Center Hôpital Femme-Mère-Enfant, Bron, 69677, France.
⁶ Laboratory Biology of Cancer and Infection, Inserm, CEA, Université Grenoble Alpes, Grenoble, France.
⁷ Department of Diagnostic and Interventional Radiology, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, University of Lyon, Pierre-Bénite, 69495, France.

Abstract

Background: Renal arteriovenous malformation (AVM) in Hereditary Hemorrhagic Telangiectasia (HHT) is uncommon and only few cases have been described, mainly with surgical management because of uncontrolled hematuria.

Case presentation: We managed a 70-year-old patient with HHT who presented with hematuria and left flank pain. Computed Tomography and ultrasound showed left renal AVM of 18 mm with clotting in the urinary tract. An external ureteral catheter was placed during 3 days to allow rinsing and facilitate elimination of clots. Given the patient's hemodynamic stability, a non-surgical management was chosen. Treatment of the AVM was performed by trans-arterial embolization using micro-coils and ethylene-vinyl alcohol copolymer.

Conclusions: Our case study shows a conservative management by embolization of ruptured left renal AVM revealed by hematuria in a 70-year-old patient with HHT.

Keywords: Arterio-Venous Malformation; Embolization; Hematuria; Hereditary Hemorrhagic Telangiectasia; Rendu-Osler-Weber disease.