Dissection of Iliac Artery in a Patient With Autosomal Dominant Polycystic Kidney Disease

AORTA

article

Audrey Courtois¹  Betty V. Nusgens¹  Philippe Delvenne²  Michel Meurisse³  Jean-Olivier Defraigne⁴  Alain C. Colige¹  Natzi Sakalihasan⁴ 

[1] Laboratory of Connective Tissues Biology, University of Liège;Department of Anatomopathology, University of Liège;Department of Abdominal, University of Liège;Department of Cardiovascular and Thoracic Surgery, University of Liège

关键词: Autosomal dominant polycystic kidney disease;    Arterial dissection;    Elastic lamellae;   

DOI  :  10.12945/j.aorta.2013.12.012

来源: Thieme

【 摘 要 】

Autosomal dominant polycystic kidney disease (ADPKD) is a risk factor for several cardiovascular disorders such as intracranial aneurysm or aortic dissection, preferentially occurring at the thoracic or abdominal level. A 47-year-old man suffering from ADPKD had renal transplantation. Sixteen hours after surgery, he presented with left leg pain. Clinical and ultrasound examination revealed thrombosis of the external left iliac artery. Therefore, we decided to perform intra-arterial angiography to evaluate the possibility of an endovascular treatment. Aorto-femorography showed an obstruction of the external left iliac artery that was found during emergency surgery, consecutive to a dissection, which occurred following the surgery for kidney transplantation. The resected segment of the dissected vessel was analyzed by histology. Collagen fibers organization and density in the adventitia and smooth muscle cells density in the media were similar in the dissected and a normal artery from a healthy donor. By contrast, an almost complete disappearance and fragmentation of elastic lamellae were observed in the media of the dissected artery, most likely responsible for the weakening of the arterial wall and its dissection. Association between ADPKD and single dissection of the iliac artery has been rarely reported. Relationship between inactivation of polycystin/PKD genes and elastic fibers degradation through elevated TGFβ signaling and matrix metalloproteinase 2 (MMP2) elastolytic activity, as recently reported in ADPKD, would be worth investigating.

【 授权许可】

Unknown