Classifying seminal vesicle cysts in the diagnosis and treatment of Zinner syndrome: A report of six cases and review of available literature.

This study aims to emphasise the importance of imaging in the diagnosis and treatment decision-making in Zinner syndrome and provide a classification for seminal vesicle cysts. The data of six patients with Zinner syndrome in a single institution were collected. All patients underwent a contrast-enhanced computed tomography (CT) exam. Among these patients, five patients also underwent an magnetic resonance imaging (MRI). These results were combined with the review of available literature to classify the seminal vesicle cysts. Among these patients, two patients had urinary urgency and frequency, while four patients had no urinary symptoms. No reproductive-system symptoms were revealed. The imaging revealed left-sided involvement in two patients and right-sided involvement in four patients. The associated features included ipsilateral renal agenesis, seminal vesicle cyst or agenesis, and ejaculatory duct obstruction. Either an ipsilateral ureterocele or an ipsilateral small testis was noted. The seminal vesicle cysts demonstrated varying attenuation or intensity in the imaging. Imaging (CT and especially MRI) can be critical in the noninvasive diagnosis of Zinner syndrome and in allowing aberrant anatomy to be displayed for possible surgery. The proposed seminal vesicle cyst imaging classification could potentially contribute to clinical decision-making.

Magnetic resonance imaging and clinical findings in seminal vesicle pathologies.

PURPOSE: Congenital and acquired pathologies of the seminal vesicles (SV) are rare diseases. The diagnosis of SV anomalies is frequently delayed or wrong due to the rarity of these diseases and the lack of adequate evaluation of SV pathology. For this reason, we aimed to comprehensively evaluate SV pathologies and accompanying genitourinary system abnormalities. MATERIALS AND METHODS: Between March 2012 and December 2015, 1455 male patients with different provisional diagnosis underwent MRI. Congenital and acquired pathology of the SV was identified in 42 of these patients. The patients were categorized according to their SV pathologies. The patients were analyzed in terms of genitourinary system findings associated with SV pathologies. RESULTS: SV pathologies were accompanied by other genitourinary system findings. Congenital SV pathologies were bilateral or predominantly in the left SV. Patients with bilateral SV hypoplasia were diagnosed at an earlier age compared to patients with unilateral SV agenesis. There was a significant association between abnormal signal intensity in the SV and benign prostate hypertrophy (BPH) and patient age. CONCLUSION: SV pathologies are rare diseases of the genitourinary system. The association between seminal vesicle pathology and other genitourinary system diseases requires complete genitourinary system evaluation that includes the seminal vesicles.

Minimally Invasive Approach for Treatment of Seminal Vesicle Cyst Associated with Ipsilateral Renal Agenesis.

OBJECTIVES: We reviewed the clinical presentation and approach for the treatment of symptomatic seminal vesicle cyst associated with ipsilateral renal agenesis while presenting our experience with a series of 5 patients treated with minimally invasive surgery for Zinner syndrome. MATERIALS AND METHODS: Between the years 2008 and 2016, we operated on 5 patients who presented with symptomatic seminal vesicle cyst and ipsilateral renal agenesis. Patients' charts and medical records were reviewed and compared with past published minimally invasive series. RESULTS: Four patients were treated in a laparoscopic approach and one was treated with robotic-assisted seminal vesicle cyst excision. In all cases, the cystic complex was drained and excised with marsupialization of the remaining cyst walls to prevent cystic recurrence. The mean operating time was 3:47 h and mean hospitalization time was 7 days (4-14). The mean follow-up period was 3.2 years (range 1.7-4.8 years). All patients reported a resolution of symptoms during postoperative follow-up. CONCLUSIONS: Seminal vesicle cyst with ipsilateral renal agenesis should be suspected in young male patients presenting with pelvic cystic masses. Treatment is reserved for symptomatic patients and the preferred approach is minimally invasive surgery. This approach is feasible and effective while providing advantages both for the patient and the surgeon.

Hypogonadotropic hypogonadism with Zinner syndrome: a coincidence or a consequence?

This case report describes a man in his 20s presenting with bilateral crypto-orchidism, micropenis and underdeveloped secondary sexual characteristics. The patient also exhibited hyposmia, eunuchoid stature and gynecomastia. Biochemical investigations revealed low levels of testosterone, luteinising hormone and follicle-stimulating hormone. Hence, he was diagnosed with Kallmann syndrome. Imaging studies showed an absent right kidney and cystic dilatation of the distal ureteric bud, seminal vesicle and absent/hypoplastic ejaculatory duct. The association of hypogonadotropic hypogonadism with Zinner syndrome, a rare condition characterised by renal agenesis, seminal vesicle cyst and ejaculatory duct obstruction, was noted.

Zinner's Syndrome: Case report of a Developmental Anomaly of the Mesonephric Duct.

Zinner's syndrome is a rare congenital malformation characterized by the association of unilateral renal agenesis with ipsilateral seminal vesicle cyst and ejaculatory duct obstruction. Most patients are asymptomatic until the third or fourth decade of life when the syndrome is associated with dysuria, perineal pain, infertility, and painful ejaculation. In this report, we present the common imaging findings of this rare developmental anomaly involving the mesonephric duct in a 48-year-old male patient experiencing pelvic pain, recurrent dysuria, and pollakiuria.

Urogenital sinus cyst in a 21-year-old man.

We report a case of a rare congenital malformation, a urogenital sinus cyst in a young patient with non-specific symptoms for several months. A 21-year-old male presented with vague and intermittent abdominal pain. Ultrasound scan showed a retrovesical hypoechoic collection (approximately 6 cm) and left renal agenesis, compatible with a possible congenital malformation. These findings were confirmed by MRI. A laparoscopic excision of the cyst was performed with no complications. Pathology report confirmed a urogenital sinus cyst. The postoperative recovery was uneventful and the patient was discharged within 48 h. Urogenital sinus cysts are rare entities with few cases described in the literature. Imaging techniques such as ultrasound, CT or MRI may help with diagnosis. Therapeutic modalities range from observation to needle aspiration or surgical removal. In our case, we chose a laparoscopic approach to minimize morbidity and achieve an early recovery.

Zinner Syndrome with Ectopic Ureter Remnant.

INTRODUCTION: Zinner syndrome is a rare congenital abnormality defined by a clinical triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction. CASE PRESENTATION: Most patients are asymptomatic, but if the cystic dilatation of the seminal vesicle becomes significant, it can result in urinary symptoms such as dysuria and urinary retention. This rare developmental anomaly related to mesonephric duct can also present with other abnormalities. CONCLUSION: Here, we report our experience of Zinner syndrome with bladder outlet obstruction and an ectopic ureter remnant.

Unilateral renal agenesis with absent ductus deferens, epididymis and seminal vesicle: incidental finding in a 22-year-old patient with maldevelopment of the mesonephric duct.

Unilateral renal agenesis with an absence of the seminal vesicle, epididymis and ductus deferens is rare and is the result of a developmental disorder of the mesonephric or Wolffian duct. We report the case of a 22-year-old man who presented with testicular pain on the left side of 3 weeks' duration. During the clinical investigation of the scrotum a nonpalpable ductus deferens on the left side was found incidentally. As a result of the urological ultrasound the diagnosis of renal, epididymal, seminal vesicle and ductus deferens agenesis on the left was confirmed. As a vascular variety the CT demonstrated 2 renal veins and 2 renal arteries on the right originating from the superior mesenteric artery together with the right hepatic artery. The testicular artery was placed on both sides. Further diagnostic investigations including a spermiogram, hormone analysis and kidney function tests were normal. Congenital urogenital malformations can be found in various combinations even in adults. Unilateral absence of the vas deferens during clinical examination should alert the clinician to an underlying renal, seminal vesicle and epididymal anomaly; further urological investigation is mandatory. A genetic investigation of the CFTR gene is not necessary in the absence of both ductus deferentes with renal agenesis.

A wide spectrum of rare clinical variants of Zinner syndrome.

Congenital malformations of the seminal vesicles (SVs) are rare and are associated with abnormalities of the ipsilateral urinary tracts as embryologically both the ureteral buds and SVs arise from the mesonephric ducts. The triad of SV cysts, ipsilateral renal agenesis and ejaculatory duct obstruction is known as the Zinner syndrome. We, herein, present three very rare presentations of Zinner syndrome. Case 1 presented with haematuria, and was found to have a large SV cyst with stones and underwent a robotic cyst excision. Case 2 presented with primary infertility, and was found to have a variant of Zinner syndrome. Case 3 was a known case of chronic kidney disease on maintenance haemodialysis who presented with fever and oliguria. He was found to have Zinner syndrome and underwent aspiration of SV abscess. To the best of our knowledge, such varying presentations of Zinner syndrome have been rarely reported thus far.

Novel application of da Vinci robotic system in patients of Zinners syndrome--case report and review of literature.

Seminal vesicle cysts combined with ipsilateral renal agenesis, ectopic ureter and giant right ampullary cyst of vas deferens represent a rare urological anomaly, Zinners syndrome. In symptomatic patients' seminal vesiculectomy along with enbloc excision of the ipsilateral ampullary cyst, ectopic ureter and dysplastic, renal tissue is the preferred treatment option. We report robotic assisted removal of a large seminal vesicle cyst with ipsilateral renal agenesis, ectopic ureter and a giant right ampullary cyst of vas deferens in a 34-year-old male. We reviewed the literature about this rare urological anomaly and novel usage of da Vinci surgical robotic surgical system (DSRS) (Intuitive Surgical, Sunnyvale, California) in performing this procedure.

Imaging of the seminal vesicle and vas deferens.

The seminal vesicle (SV) and vas deferens (VD) are ancillary but essential urogenital organs. Understanding their embryologic features and anatomy can be helpful in evaluating various disorders of these organs. Recently, cross-sectional imaging modalities, including ultrasonography, computed tomography, and magnetic resonance (MR) imaging, have been increasingly used for evaluation of the SV and VD. The development of these organs is closely related to that of urinary organs, including the kidneys and ureters. Frequently, unilateral SV agenesis is associated with renal agenesis, and bilateral SV or VD agenesis is associated with mutations of the cystic fibrosis gene. Congenital SV cysts are commonly associated with ipsilateral renal agenesis or dysgenesis. These congenital anomalies can be well evaluated with MR imaging. Inflammation, post-radiation therapy changes, and amyloidosis of the SV appear as diffuse wall thickening and may mimic tumor invasion by prostate cancer. Primary neoplasms involving the SV and VD are extremely rare, whereas secondary neoplasms are much more common. Carcinoma from the prostate, bladder, or rectum can directly invade the SV and VD. Typical MR imaging findings of such invasion include a low-signal-intensity mass on T2-weighted images or soft-tissue thickening in the SV or VD along with loss of normal architecture.

Wolffian duct derivative anomalies: technical considerations when encountered during robot-assisted radical prostatectomy.

BACKGROUND: Although malformations of the genitourinary tract are typically identified during childhood, they can remain silent until incidental detection in evaluation and treatment of other pathologies during adulthood. The advent of the minimally invasive era in urologic surgery has given rise to unique challenges in the surgical management of anomalies of the genitourinary tract. OBJECTIVE: This article reviews the embryology of anomalies of Wolffian duct (WD) derivatives with specific attention to the seminal vesicles, vas deferens, ureter, and kidneys. This is followed by a discussion of the history of the laparoscopic approach to WD derivative anomalies. Finally, we present two cases to describe technical considerations when managing these anomalies when encountered during robotic-assisted radical prostatectomy. DESIGN, SETTING, AND PARTICIPANTS: The University of Chicago Robotic Laparoscopic Radical Prostatectomy (RLRP) database was reviewed for cases where anomalies of WD derivatives were encountered. We describe how modifications in technique allowed for completion of the procedure without difficulty. MEASUREMENTS: None RESULTS AND LIMITATIONS: Of the 1230 RLRP procedures performed at our institution by three surgeons, only two cases (0.16%) have been noted to have a WD anomaly. These cases were able to be completed without difficulty by making simple modifications in technique. CONCLUSIONS: Although uncommon, it is important for the urologist to be familiar with the origin and surgical management of WD anomalies, particularly when detected incidentally during surgery. Simple modifications in technique allow for completion of RLRP without difficulty.

[Left renal atrophy and ureteral abouchement in seminal vesicle. Laparoscopic surgical treatment]. / Atrofia renal izquierda y abocamiento ureteral a vesícula seminal. Tratamiento quirúrgico laparoscópico.

Renal atrophy with an ectopic drainage of the ureter is an uncommon malformation. Zinder described in 1914 a cystic dilation of the seminal vesicle in association with an ipsilateral renal agenesis. Usually, these patients present lower urinary tract symptoms with perineal discomfort and fever. The most accurate diagnosis is given by the MRI, while CT and ultrasonography are less precise. We present a case of a patient who goes into hospital because of left lumbar pain and urinary sepsis data, whose CT detects an ectopic drainage of the left uereter in the seminal vesicle. He underwent a successful laparoscopic surgical treatment.

[Prostate cancer and left ectopic ureter opening to seminal vesicle with left renal agenesis: a case report].

We report a case of prostate cancer and left ectopic ureter opening to seminal vesicle with left renal agenesis. A 62-year-old man was admitted to our hospital for treatment of prostate cancer with cyst formation. On the rectal examination, a cystic tumor was palpable on the left side of prostate. The left kidney was not detected by intravenous pyelography and ultrasonography. Magnetic resonance imaging revealed a retrovesical cystic lesion in the left side. Total prostatectomy and left ureterectomy were performed under the diagnosis of clinical T1cN0M0 prostate cancer and left ectopic ureter opening to seminal vesicle with left renal agenesis. The pathological findings showed well differentiated adenocarcinoma, Gleason score 3 + 3, and left ectopic ureter entering into the seminal vesicle and left renal agenesis. The patient was well 39 months after the total prostatectomy and left ureterectomy without evidence of recurrence. There have been no cases of the association of this urogenital anomaly, such as ectopic ureter opening to seminal vesicle with renal agenesis and prostate cancer and the combined management of both. Our case seems to be first case in the Japanese literature.

Minimally invasive management of a symptomatic case of Zinner's syndrome: Laparoscopic seminal vesiculectomy and ipsilateral nephroureterectomy.

INTRODUCTION: Zinner syndrome is a rare developmental anomaly of the Wolffian (mesonephric) duct which is characterized by a triad of obstruction of the ejaculatory duct, the ipsilateral seminal vesicle cyst, and the ipsilateral renal agenesis. Usually is totally asymptomatic, 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 ipsilateral ureteronephrosis. The mass was removed using laparoscopic "en block" seminal vesiculectomy with associated ipsilateral nephroureterectomy. No post-operative complications occurred. At 2-month post-operative control the patient reported an improvement of urinary and rectal symptoms.

Incidental finding of Zinner syndrome in a Greek military recruit: a case report of a rare clinical entity.

BACKGROUND: Zinner syndrome represents a rare congenital malformation of the urinary tract. It comprises a constellation of Wolffian duct anomalies and is almost exclusively encountered as a classic triad of seminal vesicle cysts, ejaculatory duct obstruction and renal agenesis. Patients can be either asymptomatic or symptomatic. Recently, minimally invasive surgical techniques have emerged, superseding traditional surgery for select symptomatic cases. Our case highlights the finding of a rare clinical syndrome that was incidentally detected during a routine mass screening of military recruits in the Greek Armed Forces. CASE PRESENTATION: Herein, we present a case of a 19-year-old male who reported having a solitary right kidney when examined in a military training center of Northern Greece. No additional clinical information was available; thus, referral to a tertiary urology department for further investigation ensued. Imaging studies, namely, computed tomography and magnetic resonance imaging, revealed left renal aplasia, multiple left seminal vesicle cysts, and ejaculatory duct obstruction. Laboratory values and urinalysis were within normal range. Semen analysis was significant for cryptozoospermia. Our patient remained asymptomatic during the entire hospitalization. Long-term follow-up was recommended. Nevertheless, he declined further investigation and sought treatment in a private practice setting. CONCLUSIONS: This article aims to present the incidental diagnosis of a rare syndrome in a military setting. Population screening conducted in the armed forces permits the identification of undiagnosed diseases that warrant further investigation. To the best of our knowledge, this was the first report of Zinner syndrome in a military recruit and the second case cited of a Greek patient in the published literature. Regular follow-up is the key to timely intervention in conservatively managed cases.

Primary seminal vesicle adenocarcinoma: a lethal yet cryptic malignancy with review of literature.

The rarity of primary seminal vesical adenocarcinoma (PSVA) coupled with mostly late and advanced presentation with high mortality makes it an unanticipated malignancy with poor prognosis. Although there has been sporadic reporting of cases, the dearth of literature makes standardised care a challenge. The detection has incorporated immunohistochemistry for establishing the site of origin as well as the differentiation of primary from metastatic cancer. Surgical management with seminal vesiculectomy continues to be the mainstay of treatment, but difficult anatomy and delayed intervention do lead to an increased chance of residual disease that may warrant further adjuvant chemoradiation. We present a case report where PSVA developed in a patient with Zinner syndrome-an observation that is extremely rare with a literature review of PSVA including the various aspects of management including contemporary diagnosis techniques.

Zinner and Mayer-Rokitansky-Küster-Hauser syndromes: when unilateral renal agenesis meets genital anomalies.

Congenital unilateral renal agenesis is a relatively frequent condition at birth diagnosed mostly incidentally. Despite the excellent prognosis, unilateral renal agenesis is associated with an increased risk of other structural abnormalities, including genital malformations. The authors present two cases of asymptomatic adolescents with known congenital unilateral renal agenesis and associated genital malformations solely diagnosed during puberty-a man with Zinner syndrome and a female with Mayer-Rokitansky-Küster-Hauser syndrome.

Correlation between CFTR gene mutations in Iranian men with congenital absence of the vas deferens and anatomical genital phenotype.

Congenital bilateral absence of the vas deferens (CBAVD) and congenital unilateral absence of the vas deferens (CUAVD) are 2 causes of male sterility; these phenotypes are found in 1%-2% of men investigated for infertility and approximately 10% of men with azoospermia. To study the correlation between genital phenotype and cystic fibrosis genotype in men lacking at least 1 vas deferens, we evaluated the role of different CFTR gene mutations in the morphologic genital phenotype of 119 infertile men with bilateral or unilateral absence of the vas deferens (112 CBAVD and 7 CUAVD patients). Renal, scrotal, and transrectal ultrasonography were systematically performed. CFTR mutations and (TG)m(T)n polymorphism were analyzed, and epididymal and seminal vesicular abnormalities and testicular volume were compared among men with 2, 1, or no CFTR gene mutation, with or without the 5T allele. Our results showed that patients with CBAVD and renal agenesis have the same reproductive tract abnormalities as those with CUAVD, and reproductive tract abnormalities were independent of the subtypes of CFTR genotype in patients with absence of the vas deferens and CFTR gene mutations. Seminal vesicles did not differ between patients with or without CFTR gene mutation, but epididymal abnormalities were more frequent in CBAVD men without the mutation. Low testicular volume was observed in CBAVD men without the CFTR and IVS8-5T mutations, so we can hypothesize that a testicular factor (genetic or environmental) rather than CFTR gene mutations plays a role in determining the phenotype. Further studies using common diagnostic criteria are required to confirm our observations.