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 syndrome in pediatric age group: An underdiagnosed entity.

INTRODUCTION: Zinner Syndrome (ZS), a rare congenital malformation of the mesonephric duct, combines seminal vesicle cyst (SVC) with ipsilateral upper urinary tract abnormalities. Typically asymptomatic in childhood, ZS manifests between 2nd to 4th decades with bladder symptoms, perineal pain and infertility. Diagnostic confirmation with additional imaging is needed when either renal or seminal abnormalities are identified. MATERIALS AND METHODS: A retrospective study spanning 22 years identified 20 pediatric ZS cases through clinical analytics. Demographic, clinical, and radiological data were analyzed, including presenting complaints, imaging modalities (ultrasound, CT, MRI), and surgical findings. The study was HIPAA-compliant and IRB-approved. RESULTS: Among 20 cases (mean age: 7.3 years), clinical presentations included asymptomatic cases, urinary symptoms, and abdominal pain. Imaging revealed renal anomalies (agenesis, multicystic dysplastic kidney) and seminal vesicle abnormalities. Surgical interventions (n = 12) addressed symptomatic cases, often involving robotic or laparoscopic procedures. DISCUSSION: ZS, though rare, presents with varied clinical features, necessitating a multidisciplinary approach. Early diagnosis is facilitated by prenatal identification of renal abnormalities. Surgical intervention is reserved for symptomatic cases, with techniques such as vesiculectomy and resection of remnant structures employed. CONCLUSION: This study highlights ZS's diverse clinical and radiological spectrum, emphasizing the need for vigilance in detecting overlapping entities. Timely identification, utilizing advanced imaging techniques, is crucial for accurate diagnosis and appropriate management of Zinner Syndrome in the pediatric population.

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.

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.

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.

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.

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.

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.

Ultrassonografia no auxílio do diagnóstico de vesiculite seminal em touros de corte / Ultrasonography in the diagnosis of seminal vesiculites in beef bulls

A vesiculite é um processo inflamatório das glândulas vesiculares, podendo ser unilateral ou bilateral, que acomete reprodutores. O objetivo do presente trabalho foi avaliar a utilização da ultrassonografia como meio de diagnóstico precoce das alterações das vesículas seminais em touros. O presente trabalho foi realizado no município de Videira, Santa Catarina. Analisou-se um total 42 reprodutores, com média de idade de 15 meses, das raças Aberdeen Angus e Polled Hereford em semi-confinamento. Foi realizado exame clinico do estado geral dos animais e exame andrológico dos reprodutores. As amostras de sêmen para a realização do exame das características físicas do ejaculado foram obtidas por eletroejaculação. Através da palpação retal, realizou-se a avaliação das glândulas vesiculares por ultrassonografia. A análise estatística dos dados foi realizada por meio de análise de variância (ANOVA) para comparação entre médias com nível de significância de 5%. A presença de vesiculite foi observada em 31 animais (73,8%) dos 42 analisados. Dos 31 animais portadores 11 animais (35,5%) apresentaram vesiculite bilateral e 20 apresentaram vesiculite unilateral (64,5%; P<0,05). Animais com perímetro escrotal maior tendem a desenvolver vesiculite unilateral, tal fato pode ser explicado pela precocidade sexual agravado pela sodomia entre os animais. O uso do ultrassom auxilia de forma preventiva a detecção de animais portadores de vesiculite em reprodutores.(AU)

The vesiculite is an inflammatory process of the vesicular glands, unilateral or bilateral, that affects bulls. The purpose of this study was to evaluate the use of ultrasound as a complementary method for early detection of changes in seminal vesicles in bulls. This study was conducted in the municipality of Videira, Santa Catarina. We evaluated 42 bulls, with an average age of 15 months, Aberdeen Angus and Polled Hereford breeds and in semi-confinement. Clinical animal examination and andrological exam were performed in all animals. Semen samples were obtained by electroejaculation and physical characteristics of the ejaculate were performed. Rectal palpation was performed by ultrasonography to evaluate vesicular glands changes. Statistical analysis of the data was performed using analysis of variance (ANOVA) for comparison between means with significance level of 5%. The presence of vesiculitis was observed in 31 (73.8%) from the 42 analyzed bulls. Of the 31 animals, 11 animals (35.5%) presented bilateral vesiculitis and 20 showed unilateral vesiculitis (64.5%; P<0.05). Animals with a larger scrotal perimeter tend to develop unilateral vesiculitis, which can be explained by the sexual precocity observed by sodomy among animals. In this way, the use of ultrasound helps preventively to detect animals with vesiculitis in breeding animals.(AU)

Magnetic resonance imaging and clinical findings in seminal vesicle pathologies

ABSTRACT 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.

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.

Multicystic seminal vesicle with ipsilateral renal agenesis: two cases of Zinner syndrome.

The association of ipsilateral renal agenesis and cystic seminal vesicle is a rare congenital syndrome described by Zinner in 1914. The cases of two patients with this syndrome are presented, one of them associated with infertility, the other with cryptorchidism and testicular pain. A brief review of the literature is undertaken, regarding the main clinical and imaging implications, and the developmental anomalies that are involved in this unusual congenital anomaly are analyzed.

ß-CateninC429S mice exhibit sterility consequent to spatiotemporally sustained Wnt signalling in the internal genitalia.

Wnt/ß-catenin signalling regulates numerous developmental and homeostatic processes. Ctnnb1 (also known as ß-catenin) is the only protein that transmits signals from various Wnt ligands to downstream genes. In this study, we report that our newly established mouse strain, which harbours a Cys429 to Ser missense mutation in the ß-catenin gene, exhibited specific organ defects in contrast to mice with broadly functioning Wnt/ß-catenin signalling. Both homozygous mutant males and females produced normal gametes but were infertile because of abnormal seminal vesicle and vaginal morphogenesis. An ins-TOPGAL transgenic reporter spatiotemporally sustained Wnt/ß-catenin signalling during the corresponding organogenesis. Therefore, ß-catenin(C429S) should provide new insights into ß-catenin as a universal component of Wnt/ß-catenin signal transduction.

Seminal vesicle hypoplasia with contralateral renal agenesis.

Renal agenesis is a rare condition of unknown etiology frequently seen together with ipsilateral seminal vesicle and vas deferens anomalies because of common embryologic development. However, no cases of contralateral seminal vesicle hypoplasia accompanying renal agenesis have previously been reported. We describe a case of contralateral seminal vesicle hypoplasia accompanying renal agenesis incidentally detected in a 27-year-old presenting to the urology clinic with pelvic pain.