International Urology and Nephrology 29 (4). pp. 403-410 (1997) Renal Agenesis, Ureteral Ectopia into Seminal Vesicle, Vas Deferens Agenesis and Hemivertebra: An Incomplete Form of Caudal Regression Syndrome? E ARAGONA, L. D'URso, C. VALOTTO, C. IV[ILANI, A. C A L A B R O Institute ofUrology,University ofPadua,Italy (Accepted January I0, 1997) The association of seminal vesicle cyst and upper urinary tract malformation is well known in the literature [1]. More rarely, urogenital malformations are associated with vertebral [2] or anorectal anomalies [3]. A 35-year-old infertile man with unilateral renal and deferential agenesis, seminal vesicle cyst and hemivertebra is reported. This complex malformative syndrome has been reported previously by Sheih et al. [4] and, to our knowledge, this is the third case described in the literature. Case report A 35-year-old married man was admitted for evaluation of infertility. The patient was asymptomatic and the ejaculation was preserved. Physical ex- amination revealed an enlarged fight epididymis and the absence of the left vas. On digital rectal examination a painless soft mass the size of an olive was palpable above the left prostatic lobe. Laboratory investigations were unremarkable except for sperm count which revealed azoospermia associated with hypoposia (less than 1.5 ml); FSH, LH and testosterone were all within normal limits. Bilateral testicular fine needle biopsy showed normal spermatogenesis. Ultrasound, CT scan and MRI revealed agenesis of the right kidney and cystic enlargement of the ipsilateral seminal vesicle with ectopic opening of a blind-ending ureter; the left seminal vesicle was hypoplastic and cystic (Figs 1A, 1B, 2). Transperineal sonographically-guided right vesiculography (Fig. 3) and right deferentovesiculography (DVG) (Fig. 4) showed normal epididymis and vas deferens with enlarged seminal vesicle and ectopic ureteral stump, about 10 cm long; the right ejaculatory duct is not visible, probably because of the obstruction of its outlet. A hemivertebra of L3-L4 was also noted. Repeated attempts to puncture the left seminal vesicle were unsuccessful. Puncture of the right seminal vesicle allowed to obtain about 5 ml of a dense, brownish liquid, whose examination showed the presence of rare sperms (1-2 per microscopic field) with poor vitality. VSP, Utrecht Akad~miai Kiadf, Budapest