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Zinner Syndrome (ZS) is a rare congenital genitourinary abnormality defined by seminal vesicle cysts, ejaculatory duct obstruction, and unilateral renal dysplasia or agenesis. Patients can be asymptomatic, while others experience pain, urinary or ejaculatory symptoms and infertility. A patient that presented with painless gross hematuria was found to have a large pelvic cystic structure, an absent left kidney, multiple fluid collections in the region of the left seminal vesicle and right hydronephrosis. Hydronephrosis is atypical in ZS. This patient eventually developed right flank and pelvic pain treated with robotic-assisted laparoscopic excision of the pelvic cystic structure and extravesical ureteral reimplantation.
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Introduction: This report describes a case with an ectopic ejaculatory duct opening into the bladder trigone in Zinner syndrome, congenital unilateral renal agenesis, and an ipsilateral seminal vesicle cyst. Case presentation: The patient was identified when no left kidney was detected in the fetal period. Abdominal ultrasonography and pelvic plain MRI at 6 months old revealed a 10-mm cystic lesion on the dorsal aspect of the bladder. Cysto-urethroscopy at 1 year old revealed a rather short posterior urethra and right and left inferior crests extending from the posterior urethra beyond the bladder neck. The ejaculatory duct opening was identified on the bladder trigone. Conclusion: Anatomical abnormality of the ejaculatory duct may represent a cause of infertility and ejaculatory dysfunction in Zinner syndrome. Endoscopic evaluation should be performed for this rare anomaly, even in children.
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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.
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Glândulas Seminais , Humanos , Estudos Retrospectivos , Masculino , Criança , Glândulas Seminais/anormalidades , Glândulas Seminais/diagnóstico por imagem , Pré-Escolar , Síndrome , Feminino , Adolescente , Anormalidades Múltiplas/diagnóstico , Ductos Mesonéfricos/anormalidades , Cistos/diagnóstico , Cistos/cirurgia , LactenteRESUMO
Zinner syndrome is a rare congenital urological entity, secondary to an alteration in embryogenesis between 4th and 13th weeks of gestation, specifically because of abnormalities in the development of the distal mesonephric duct. It is characterized by the triad of unilateral renal agenesis, cystic dilatation of the ipsilateral seminal vesicle and ipsilateral ejaculatory duct obstruction. The aim of this article is to provide the reader with all the necessary information to be able to suspect the presence of this syndrome, reviewing its physiopathology, clinical manifestations and the imaging techniques that enable its diagnosis, emphasizing those radiological findings by MRI that should lead us to think about it. This work is illustrated with representative radiological images of cases belonging to our institution, including patients with different variants of Zinner syndrome. We also include an overview of the embryology of the male urogenital system, to remember the role of the mesonephric duct and the ureteral bud in the formation of the different urogenital structures, as well as a differential diagnosis that allows us to differentiate seminal vesicle cysts from other pelvic cystic lesions.
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OBJECTIVE: This study aims to describe the MRI findings of six patients with ectopic ureters in a tertiary care institute. METHODS: A retrospective analysis was conducted on six patients presenting to the Department of Radiodiagnosis at Sawai Man Singh (SMS) Hospital, Jaipur, India, with ectopic ureters. Data were collected from the 3 Tesla (3T) Philips MRI scanner (Koninklijke Philips N.V., Amsterdam, Netherlands) from 2021 to 2023. RESULTS: The mean age was 21.6 years, with an equal male-to-female ratio (1:1). Most patients presented with urinary incontinence (50%, n = 3), followed by abdominal pain. Only 16.6% (n = 1) were associated with a duplex collecting system. In males, the most common site was the seminal vesicle, observed in 66.6% (n = 2), followed by the urethra in 33.4% (n = 1) of cases. One patient with an ectopic opening into the seminal vesicle had Zinner syndrome. In females, the ectopic site was found to be the vagina in all three patients. CONCLUSION: Ectopic ureter is a rare anomaly of the urinary system, often associated with other urinary system anomalies and a few syndromes. Clinical presentations range from the patient being asymptomatic to renal failure; therefore, a high index of suspicion and appropriate imaging are necessary for early diagnosis and timely treatment.
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Zinner syndrome, a rare congenital anomaly affecting males, is characterized by atresia of the ejaculatory duct, seminal vesicle cysts, and ipsilateral renal agenesis. This case report details a 2-year-old boy successfully treated with laparoscopic excision of a dilated vas deferens and seminal vesicle cyst. The rarity of Zinner syndrome in pediatric patients underscores the importance of understanding its diagnosis and minimally invasive surgical management.
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Zinner syndrome comprises a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction, which can be accompanied by additional abnormalities of the genitourinary tract in some cases. Patients may be asymptomatic or present with urinary, reproductive, and/or local pain symptoms. Diagnosis is most commonly achieved via MRI. Here, we present the case of an 18-year-old male previously diagnosed with unilateral renal agenesis, who presented with testicular and penile pain, along with urinary urgency and frequency. MRI of the abdomen and pelvis revealed all three components of Zinner syndrome as well as an ectopic ureter emptying into the seminal vesicle. Our case adds to the existing limited literature on this rare syndrome and broadens the understanding of how this syndrome can present both clinically and radiologically.
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We report the case of a 24-year-old male presenting with obstructive renal failure, characterised by imaging evidence of a cystic lesion contingent upon the seminal vesicle and concurrent renal agenesis. Initial management involved urinary diversion, followed by outpatient monitoring and subsequent recurrence. Subsequent diagnostic assessments led to the identification of Zinner's syndrome, accompanied by retroperitoneal fibrosis. We present the clinical course, diagnostic methodology and the efficacious implementation of medical-surgical therapeutic interventions, yielding favourable outcomes. LEARNING POINTS: The value of the Internal Medicine team in the assessment of low prevalence diseases.The importance of multidisciplinary teams.Integration of the internists in the surgical teams.
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Introduction: Zinner syndrome (ZS) is the association of seminal vesicle cysts, ipsilateral ejaculatory duct obstruction, and ipsilateral renal agenesis. This condition is very rare in children and both diagnosis and treatment may be challenging. We reviewed the clinical presentation and treatment describing our experience with a series of three patients. Methods: From January 2016 to January 2021, three patients (patients 1, 2, and 3) with symptomatic ZS, aged 2, 15, and 17 years, respectively, were diagnosed and treated. All three patients were symptomatic, manifesting pelvic pain and dysuria. The diagnosis was made by physical examination, ultrasonography, and abdominopelvic MRI. Patient 1 underwent open surgery, while for patients 2 and 3, laparoscopic excision was performed. Results: The renal agenesis regarded the left side in patients 1 and 3, and the right side in patient 2. In all cases, the cystic complex was excised. The mean operating time was 4â h and the mean hospitalization time was 5 days (range 4-6 days). The mean follow-up period was 5 years (range 2-5 years). Patients 1 and 3 showed a complete resolution of the symptoms during postoperative follow-up. In patient 2, clinical symptoms relapsed because of the persistence of a 9â mm cyst requiring a redo laparoscopic excision. Conclusions: Seminal vesicle cyst with ipsilateral renal agenesis, even if rare in pediatric age, should be suspected in young male patients presenting with pelvic cystic masses, pelvic pain, dysuria, and ipsilateral renal absence. Conservative management should be reversed to asymptomatic patients. Surgical treatment is mandatory in symptomatic cases and the preferred approach is minimally invasive surgery to magnify the operating field to spare anatomical structures, primarily the contralateral vas deferens. Radicality is crucial to avoid the persistence of symptoms and the need for reintervention.
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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.
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Doenças dos Genitais Masculinos , Rim , Masculino , Humanos , Pessoa de Meia-Idade , Rim/diagnóstico por imagem , Rim/anormalidades , Disuria , Glândulas Seminais/diagnóstico por imagem , Glândulas Seminais/anormalidades , Ductos Mesonéfricos/diagnóstico por imagem , Ductos Mesonéfricos/anormalidades , SíndromeRESUMO
Triazolinediones are an important class of derivatization agents that have found application in various research disciplines. Their unique reactivity often allows precise and selective tagging of relevant molecular scaffolds to facilitate structural elucidation, tracking in biological systems, and stabilization of labile compounds. Recent research efforts mainly focused on the development of novel fluorescent and ionizable or isotopically labeled tags improving the quantification and identification of the parent molecule by suitable analytical methods. However, these concepts often lack the ability to improve properties facilitating the analysis by nuclear magnetic resonance (NMR) spectroscopy. We herein describe the first synthesis of 13 C and 15 N labeled [3,5-13 C2 ,4-15 N]4-phenyl-1,2,4-triazoline-3,5-dione utilizing the Cookson/Zinner-Deucker synthesis of urazoles. The introduced isotopic labels are ideally suited to support the structural elucidation of unknown and complex derivatization mixtures by NMR, thereby exploiting the increased sensitivity of detecting long-range JHC and additional JCC and JCN couplings within the derivatized compounds of interest.
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Triazóis , Indicadores e Reagentes , Triazóis/química , Espectroscopia de Ressonância MagnéticaRESUMO
Zinner Syndrome is a rare congenital anomaly. It is considered a rare cause of male infertility and can cause a range of clinical manifestations that may lead to significant morbidity. The diagnosis of Zinner Syndrome requires a high index of suspicion, combined with a detailed clinical evaluation and imaging studies. Ultrasonography, computed tomography, and MRI are the imaging modalities of choice for the diagnosis of this condition. Radiological evaluation also plays a crucial role in the management of Zinner Syndrome. In symptomatic cases, surgical intervention may be necessary, and radiology is essential for surgical planning and postoperative monitoring. In this case report, we describe an uncommon case of a 35-year-old patient with vague scrotal pain and discuss the clinical presentation, diagnosis, and management of this rare condition. Prompt and accurate diagnosis is important to prevent the potential morbidity associated with this condition, such as recurrent epididymitis, urinary tract infections, and infertility.
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BACKGROUND: Zinner's syndrome is a rare congenital malformation of the seminal vesicle and ipsilateral upper urinary tract caused by mesonephric duct developmental anomaly during early embryogenesis. This study aimed to demonstrate the significance of magnetic resonance imaging (MRI) in distinguishing pelvic cysts in males, given that MRI is the gold standard exam for confirming the diagnosis and managing therapy. CASE REPORT: A 21-year-old male patient with a solitary kidney who had been diagnosed since birth presented with abdominal pain. Transabdominal and transrectal ultrasonography (US), computed tomography (CT), and MRI were performed. The contrast-enhanced MRI of the pelvis showed a tubular fluid-filled, macrolobulated lesion measuring 6 x 6 x 4 cm, mildly high signal intensity in the T2-weighted images, and slightly high signal intensity in the T1-weighted images, without contrast enhancement. The left kidney was hypoplasic. Imaging findings led to the diagnosis of Zinner's syndrome, and conservative treatment was planned. DISCUSSION: Zinner's syndrome is characterized by a triad consisting of unilateral renal agenesis or hypoplasia, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction. MRI is the modality of choice for an impeccable depiction of the anatomy of the male genital tract, for demonstrating the seminal vesicles and evaluating anomalies of the mesonephric duct. It is also useful in distinguishing seminal vesicle cysts from other cystic pelvic masses. CONCLUSION: Zinner's syndrome should be considered when diagnosing cystic pelvic masses in males with renal agenesis or hypoplasia. Because of its high soft tissue contrast resolution, MRI is the gold standard modality for confirming the diagnosis and assessing the cyst's origin and contents.
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Background: Zinner syndrome is a rare congenital anomaly featuring a unilateral seminal vesicle cyst and ipsilateral renal agenesis. While the majority of affected patients are asymptomatic and followed with conservative management, others have symptoms such as micturition, ejaculatory difficulties, and/or pain, thus may require treatment. These patients often undergo an invasive procedure as first-line treatment, such as transurethral resection of the ejaculatory duct, or aspiration and drainage, which reduces pressure within the seminal vesicle cyst, or surgical resection of the seminal vesicle. Reported here is a patient with ejaculation pain and pelvic discomfort associated with Zinner syndrome who was successfully treated in a non-invasive manner with silodosin, an α1-adrenoceptor antagonist. Case Description: A 37-year-old Japanese male had ejaculation pain and pelvic discomfort associated with Zinner syndrome. Two months of treatment with silodosin, an α1-blocker, resulted in complete pain relief. Thereafter, conservative management with regular follow-up examinations has been conducted for five years, without recurrence of ejaculation pain or other symptoms associated with Zinner syndrome. Conclusions: This is the first known published case report of a patient with Zinner syndrome treated with silodosin who was completely relieved from ejaculation pain. The effect of α1-adrenoceptor antagonists to inhibit seminal vesicle contraction, as well as cause relaxation of smooth muscles of the urethra and prostate may contribute to reduce pain associated with ejaculation. We concluded that silodosin treatment should be attempted in affected patients before considering surgical treatment.
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Zinner syndrome is a rare developmental anomaly of the distal Wolffian duct. It is characterized by a triad of unilateral renal agenesis, cysts in the ipsilateral seminal vesicle, and ipsilateral obstruction of the ejaculatory duct. While some patients are asymptomatic and diagnosed incidentally, other patients may present with symptoms related to obstructed ejaculatory ducts and seminal vesicle cysts. We report a unique case of a 32-year-old male who presented with pelvic pain for three days.
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This is the case of a 45-year-old male who presented with dysuria and gross hematuria. Ultrasonography and computed tomography revealed an enormous pelvic cystic lesion and a right nephromegaly but an invisible left kidney. Using the imaging findings, the patient was diagnosed with Zinner syndrome. Transperitoneal laparoscopic excision of the cyst was performed for symptom relief. At post-treatment, the symptoms disappeared, and sexual function remained. Seminal vesicle cysts have been commonly reported in previous studies but rarely in this case. Furthermore, most previous studies have documented surgical excision of <10 cm cysts. However, a â¼12.5-cm seminal vesicle cyst was excised with the laparoscopic method without any perioperative complication in this case.
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Zinner's syndrome is a rare congenital disorder presenting with unilateral renal agenesis or dysgenesis, ipsilateral seminal vesicle cysts, and ejaculatory duct obstruction. Treatment of this syndrome can be conservative or surgical. In this case report, we describe the case of a 72-year-old patient who was diagnosed with Zinner's syndrome and underwent laparoscopic radical prostatectomy for prostate cancer treatment. The peculiarity of our case was that the patient's ureter emptied ectopically into the left seminal vesicle, which was notably enlarged and multicystic in appearance. Although many minimally invasive procedures have been reported for treating symptomatic Zinner's syndrome, to our knowledge, this is the first reported case of prostate cancer in a patient with Zinner's syndrome who was treated with laparoscopic radical prostatectomy. Laparoscopic radical prostatectomy can be safely and efficiently performed in patients with Zinner's syndrome and synchronous prostate cancer by urological surgeons with extensive experience in laparoscopy in high-volume centers.