Undescended testis with congenital unilateral absence of the vas deferens

Congenital absence of the vas deferens (CAVD) is a condition in which the vasa deferentia, fail to form properly prior to birth. It may either be unilateral (CUAVD) or bilateral (CBAVD). Congenital absence of vas deferens is responsible for up to 2% of male infertility. The disease is considered to be a primary genital form of cystic fibrosis. It contributes towards 6% of obstructive azoospermia cases. Upto 80% of patients with CAVD have mutations accociated with Cystic Fibrosis Transmembrane Conductance (CFTR) gene. Upper urinary tract malformations are noted in up to 20% of cases. This category of patients have a normal frequency of CFTR mutations and considered a distinct clinical entity. We hereby report a rare case of CUAVD with ipsilateral renal agenesis with cryptorchidism. The Sri Lanka Journal of Surgery 2014; 32(3): 40-41