Successful Kidney Transplantation in a Patient with Marfan’s Syndrome with a History of Aortic Dissection Operation and a Dissection Flap Up to the Iliac Bifurcation Level

Marfan’s syndrome is a collagen tissue disease characterized by aortic aneurysm and dissection. In our study, a case who was diagnosed with Marfan’s syndrome had a dissection flap up to the iliac bifurcation level and underwent successful renal transplantation; is presented. A 27-year-old male patient had a history of operations due to 2 aortic dissections. In the physical examination, he had hyperflexible joints, arachnodactyly, a pansystolic murmur in the mitral focus, and severe myopia. Echocardiography revealed enlargement of the aortic root, and a dissection flap extending from the abdominal aorta to the iliac bifurcation was detected in tomographic angiography. Genetic analysis revealed Fibrillin-1 gene exon 66 region c.8384T > C (p.Ile2795Thr) missense heterozygous mutation. The patient, who was diagnosed with Marfan’s syndrome, underwent kidney transplantation from his healthy and non-mutated sibling. There were no vascular complications in the 1-year-period after transplantation, and he is followed up with normal kidney function. In our study, it has been shown that successful kidney transplantation can be performed in a patient with Marfan’s syndrome who has a dissection flap up to the iliac bifurcation level.

Cite this article as: Taner Yılmaz V, Kısaoğlu A, Avanaz A, et al. Successful kidney transplantation in a patient with Marfan’s syndrome with a history of aortic dissection operation and a dissection flap up to the iliac bifurcation level. Turk J Nephrol. 2023;32(1):98-101.