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

Abstract

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.

Key Words

Autosomal dominant polycystic kidney disease - Arterial dissection - Elastic lamellae

• References

• 1 Wang D, Strandgaard S. The pathogenesis of hypertension in autosomal dominant polycystic kidney disease. J Hypertens 1997; 15: 925-933 . 10.1097/00004872-199715090-00002
• 2 Bichet D, Peters D, Patel AJ, Delmas P, Honore E. Cardiovascular polycystins: Insights from autosomal dominant polycystic kidney disease and transgenic animal models. Trends Cardiovasc Med 2006; 16: 292-298 . 10.1016/j.tcm.2006.07.002
• 3 Barker SG, Burnand KG. Retrograde iliac artery dissection in Marfan's syndrome. A case report. J Cardiovasc Surg (Torino) 1989; 30: 953-954
• 4 Cattan S, Mariette X, Labrousse F, Brouet JC. Iliac artery dissection in alpha 1-antitrypsin deficiency. Lancet 1994; 343: 1371-1372 . 10.1016/S0140-6736(94)92513-5
• 5 Chapman AB, Rubinstein D, Hughes R, Stears JC, Earnest MP, Johnson AM. , et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. N Engl J Med 1992; 327: 916-920 . 10.1056/NEJM199209243271303
• 6 Griffin MD, Torres VE, Grande JP, Kumar R. Vascular expression of polycystin. J Am Soc Nephrol 1997; 8: 616-626 , 1997.
• 7 Hassane S, Leonhard WN, van der Wal A, Hawinkels LJ, Lantinga-van Leeuwen IS, ten Dijke P. , et al. Elevated TGFbeta-Smad signalling in experimental Pkd1 models and human patients with polycystic kidney disease. J Pathol 2010; 222: 21-31 . 10.1002/path.2734
• 8 Sakalihasan N, Delvenne P, Nusgens BV, Limet R, Lapiere CM. Activated forms of MMP2 and MMP9 in abdominal aortic aneurysms. J Vasc Surg 1996; 24: 127-133 . 10.1016/S0741-5214(96)70153-2