Archivio Italiano di Urologia e Andrologia 2019; 91, 158 CASE REPORT Minimally invasive management of a symptomatic case of Zinner’s syndrome: Laparoscopic seminal vesiculectomy and ipsilateral nephroureterectomy Emanuele Corongiu 1, Pietro Grande 2, Valerio Olivieri 3, Giorgio Pagliarella 1, Flavio Forte 1 1 Department of Urology, M.G. Vannini Hospital, Rome, Italy; 2 Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Pitié Salpétière, Urology Department, Paris, France; 3 Department of Urology, Ivrea Hospital - ASL TO 4, Ivrea, Italy. Introduction: Zinner syndrome is a rare developmental anomaly of the Wolffian (mesonephric) duct which is characterized by a triad of obstruc- tion of the ejaculatory duct, the ipsilateral seminal vesicle cyst, and the ipsilateral renal agenesis. Usually is totally asympto- matic, however it can also determine symptoms such as lower urinary tract symptoms, perineal pain, ejaculatory disorders such as painful ejaculation or hematospermia, and infertility. Case report: We present a case of a 51 years old men with a 3-year history of lower urinary tract symptoms, perineal pain, obstructed defecation, recurrent urinary tract infections and infertility. CT scan showed a voluminous cystic neoformation of the left seminal vesicle, hypoplasia of the left kidney and ipsi- lateral ureteronephrosis. The mass was removed using laparo- scopic “en block” seminal vesiculectomy with associated ipsilat- eral nephroureterectomy. No post-operative complications occurred. At 2-month post-operative control the patient report- ed an improvement of urinary and rectal symptoms.. KEY WORDS: Seminal vesicle cyst; Zinner’s syndrome; Nephroureterectomy; Seminal vesciculectomy; Laparoscopy. Submitted 2018; Accepted 2019 Summary No conflict of interest declared. infections and infertility. Uroflowmetry, showed reduced maximal flow (9 ml/s), irregular flow pattern and a con- sistent postvoid residue (150 ml). The evaluation was completed by an abdominal ultrasound which showed a voluminous cystic formation of the left seminal vesicles together with agenesis of the left kidney. Accordingly, CT scan with excretory phase, revealed a small pelvis com- pletely occupied by a voluminous cystic formation dislo- cating the rectum and the urinary bladder and causing right ureteronephrosis, with ureteral outlet at the level of the cystic mass (Figure 1). Additionally, hypoplasia of the left kidney was noticed (Figure 1). Based on these data, a minimally-invasive surgical approach was planned, as pre- viously reported as a safe and feasible option (3). A laparoscopic left nefroureterectomy and cystic mass ablation was subsequently planned. The patient was placed in Trendelenburg position: a 12 mm optical Trocar was inserted 1 cm above the umbilical fold. Pneumoperitoneum was obtained and three other trocars (two of 5 and one of 10 mm) were placed. The Douglas fold was completely obliterated by the engorged left seminal vesicle underlying the parietal pelvic peritoneum. The extra-peritoneal space was accessed and the seminal tangle carefully dissected, tak- ing care to avoid direct damages to rectal walls and moreover to the Hovealque plexus located at both later- al rectal sides. Special attention was taken to retract lat- erally the sacro-recto-genito-pubic ligaments (Delbet bands) in which prostatic and vesical vessels roots are contained. The right ductus deferens was identified and spared, while the left one was necessarily transacted at the point in which it seemed to fuse with the left ureter on the engorged dome of the seminal vesicle, configur- ing a disturbance of the paramesonephric Wolffian duct development. Once the seminal vesicle removed, the patient was moved in right flank position to perform nephroureterectomy. One more 5 mm Trocar was insert- ed at the cross between the pararectal and infracostal left lines. Via detaching the mesosigma and gaining the Jonnesco space, 3-5 cm above the left common iliac ves- sels, the aplastic kidney with its small vascular pedicle was found and it was possible to remove the left kidney, the dilated ureter and the cystic mass "en block", with an DOI: 10.4081/aiua.2019.1.58 INTRODUCTION Zinner syndrome is a very rare condition, first described in 1914 (1), with less than 200 cases reported world- wide, characterized by unilateral renal agenesis, ipsilat- eral seminal vesicle cyst and ejaculatory duct obstruc- tion. The association between upper urinary tract abnor- malities and seminal vesicle malformation are based on the shared origin of the ureteral buds and seminal vesi- cles from the mesonephric (Wolffian) duct (2). In most cases, these anomalies are completely asymptomatic and the diagnosis is often incidental. However, the association with lower urinary tract symptoms, perineal pain, ejacula- tory disorders such as painful ejaculation or hematosper- mia, and infertility may help unveil the syndrome. CASE REPORT We report the case of a 51-year old Caucasian man, pre- senting with lower urinary tract symptoms (LUTS), per- ineal pain, obstructed defecation, recurrent urinary tract Corongiu2_Stesura Seveso 25/03/19 17:24 Pagina 58 59Archivio Italiano di Urologia e Andrologia 2019; 91, 1 Nephroureterectomy e seminal vesciculectomy in Zinner’s syndrome Ta D operative time of about 2 hours (Figure 2). The post- operative course was without complications and the patient was discharged on the 4th post-operative day. CONCLUSIONS Final pathological analysis revealed a small polycystic left kidney in the absence of residual parenchyma. The cystic mass (measured at 33 cm in diameter) was compatible with a polycystic and ectopic seminal vesicle (Figure 2). The patient was subsequently followed-up in outpatient setting, reporting a progressive and continu- ous improvement LUTS and rectal symptoms. Approximately 60 days after the surgical procedure, the patient underwent a new uroflowmetric check which showed a more regular pattern and significantly improved urinary indices (Qmax 15 ml/S) and a reduction of post-void volume (60 cc). REFERENCES 1. Zinner A. Ein fall von intravesikaler samenblasenzyste. Wein Med Wochenschr. 1914; 64:605-609. 2. Kao CC, Wu CJ, Sun GH, et al. Congenital seminal vesicle cyst associated with ipsilateral renal agenesis mimicking bladder outlet obstruction: a case report and review of the literature. Kaohsiung J Med Sci. 2010; 26:30-4. 3. Kiremit MC, Acar O, Sag AA, et al. Minimally invasive manage- ment of Zinner’s syndrome with same-session robot-assisted seminal vesiculectomy and ipsilateral nephroureterectomy using a single geometry of trocars, J Endourol Case Rep. 4: 1, 186-189. Figure 1. CT scan. Correspondence Emanuele Corongiu, MD (Corresponding Author) emanuele.corongiu@libero.it Giorgio Pagliarella, MD Flavio Forte, MD G.M. Vannini Hospital, Department of Urology Via di Acqua Bullicante 4, 00177, Rome (Italy) Pietro Grande, MD Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Pitié Salpétière, Urology Department, Paris (France) Valerio Olivieri, MD Department of Urology, Ivrea Hospital - ASL TO 4, Ivrea (Italy) Figure 2. Hypoplasia of the left kidney and voluminous seminal cystic formation. Corongiu2_Stesura Seveso 25/03/19 17:24 Pagina 59