Congenital absence of the vasa deferentia presenting with infertility.

Congenital absence of both vasa deferentia is not an infrequent cause of sterility. Between April 1975 and December 1981, 11 men out of a total of 749 presenting with infertility were diagnosed as having congenital absence of both vasa deferentia. Subsequent clinical investigations showed that FSH levels were within the normal range (2-10 mIU/ml), blood karyotype (XY) was normal, and testicular histology demonstrated normal spermatogenesis. Seminal volume was markedly reduced in nine patients (range 0.25-1.0 ml). In three out of four patients tested, seminal fructose was found to be completely absent. Of the 11 patients, eight subsequently had exploratory surgery. In four men, the whole epididymis was present on both sides, while the other four had varying parts of one or both epididymides absent. In six of the eight patients explored surgically, no trace of the vasa deferentia could be found, while one other patient had thin fibrous cords in the anatomical site of the vasa deferentia. A possible cause for the abnormality and the importance of seminal fructose estimation are discussed.