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A 33-year-old male presented to the Chiba University Hospital with the main complaint of right flank pain. Bilateral vas deferens were not palpable. Hormonal examination revealed hypergonadotropic hypogonadism and cytogenetic studies a 47, XXY karyotype. The significance of the association of this karyotype with the absence of vas deferens is discussed. Hideki Fuse, MD, Department of Urology, School of Medicine, Chiba University, Chiba (Japan) Introduction Abnormality of the external genitalia or internal genital duct is rare in Klinefelter’s syndrome. Leiba et al. [1] first reported a case of Klinefelter’s syndrome with bilateral absence of the vas deferens. Thereafter no other case of Klinefelter’s syndrome with this anomaly has been reported. Herein, a case with these two malconditions is presented. smaller increase to 110.1 mlU/ml at 30 min after an injection of 100 μg luteinizing hormonereleasing hormone. Lymphocyte cultures followed by G and Q banding showed a chromosome complement of 47, XXY (fig. 1). Semen analysis showed azoospermia. Intravenous pyelography revealed normal findings. From the above findings, the diagnosis of Klinefelter’s syndrome with bilateral absence of the vas deferens was made and testicular biopsy and examination of vas deferens were performed. Bilateral normal epididymis were found but the vas deferens was absent in both sides. Histology of both testes obtained by biopsy revealed hyalinization of the seminiferous tubules and proliferation of Ley-dig cells (fig. 2). Case Report A 33-year-old male presented to the Chiba University Hospital with the main complaint of right flank pain. At the patient’s birth, his father and mother were 30 and 27 years old, respectively. Pregnancy and delivery were normal and there was no disease in his past history. He was 167 cm tall and 67 kg in weight. His arm span was 173.5 cm. He had pubic hair of male type. The D ow nl oa de d by : 54 .7 0. 40 .1 1 11 /1 /2 01 7 12 :3 0: 34 A M volumes of the testes was 3 ml each. Bilateral vas deferens were not palpable. Rectal examination showed a small sized prostate gland. There was no gynecomastia. Hematological and liver function tests and urinalysis were within normal range. The basal levels of luteinizing hormone (LH), follicle stimulating hormone (FSH) and testosterone in blood were 56.0 mlU/ml, 49.7 mlU/ml and 285 ng/dl, respectively, which correspond to hypergonadotropic hypogonadism. After administration of human chorionic gonadotropin (hCG, 4,000 IU) for 3 days, the testosterone level in blood rose from 285 to 300 ng/dl. LH rose to 290.0 mlU/ml at 30 min and FSH showed a Discussion It has been reported that of all males investigated for infertility, 0.3–1.5% have no vas deferens [2–5] and 5–10% of the men with azoospermia have this anomaly [6, 7], which means that it is not a rare cause of male infertility. Michelson [3] postulated that absence of the vas deferens must be the result of a disturbance of the outgrowth of the Wolffian duct, which is controlled by testosterone secreted from embryonic testes. Then hormonal disorders in gestation may cause this anomaly, but abnormality of internal genital duct in Klinefelter’s syndrome is very rare [1], which revealed most of the 182 Fuse/Shiseki/Shimazaki/Katayama