Zinner Syndrome Research Articles

VJY09 Is the robotic assisted surgery the optimal management of Zinner syndrome? Description of a rare entity and description of our technique

VJY09 Is the robotic assisted surgery the optimal management of Zinner syndrome? Description of a rare entity and description of our technique

Unraveling Zinner Syndrome: Insights from a distinct case

Unraveling Zinner Syndrome: Insights from a distinct case

Pediatric Zinner syndrome variants: Case series with newer insights into pathogenesis in early childhood

Zinner Syndrome (ZS) is a rare congenital disorder characterized by seminal vesicle cysts (SVC) and ipsilateral upper urinary tract abnormalities, mainly due to developmental anomalies of the mesonephric duct. This series highlights our institutional experience with pediatric ZS, with a review of the current literature offering insights into its etiopathogenesis in early childhood. A retrospective case review of pediatric ZS diagnosed at our institution from 2019 to 2023, alongside a comprehensive literature review. Four pediatric ZS cases were identified, a neonate, an infant and two older (pre-pubertal) children, presenting with recurrent epididymo-orchitis and/or UTIs. The two older children had duplex systems, both undergoing curative upper moiety heminephrectomy; the infant underwent nephroureterectomy and the neonate is under observation, asymptomatic for past 18 months. When Zinner identified the link between unilateral renal agenesis and ipsilateral SVC in 1914, the condition's embryological basis was attributed to incorrect ureteric bud migration from the mesonephric duct, failing to stimulate the metanephros, leading to renal agenesis/dysplasia and this disruption was hypothesized to obstruct seminal vesicle drainage, causing cyst formation. Another theory suggests anomalous development of the distal mesonephric duct leading to ejaculatory duct atresia/stenosis which results in cystic enlargement of the seminal vesicles which, in turn, leads to aberrant ureteral budding, resulting in renal malformations. It is our belief that the SVCs, that are typically problematic in adolescence/adulthood due to secretion accumulation, sometimes manifest in childhood due to urinary reflux into the seminal vesicles, leading to epididymo-orchitis or UTIs. This contrasts with adult pathogenesis, where ejaculatory duct obstruction predominates. Hence, treatment leans towards a conservative approach for asymptomatic cases, with surgery reserved for symptomatic children. The scope of this case series is limited by the rare nature of ZS in prepubertal children (41 published cases in English literature), preventing a comprehensive understanding of its untreated natural history and restricting the formulation of generalized recommendations. The variability in presentation of ZS in children necessitates a tailored approach. Unlike adults, where ejaculatory duct obstruction is the common cause, pediatric ZS symptoms mainly stem from urethra-cystic reflux, leading to recurrent infections.

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

IntroductionThis 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 presentationThe 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.ConclusionAnatomical 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.

Zinner syndrome: a radiological journey through a little known condition.

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.

Zinner syndrome in pediatric age group: An underdiagnosed entity

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. 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. 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. 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. 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 syndrome in a case of male infertility: a case report

Zinner syndrome is a rare congenital malformation formed by the developmental anomaly of distal portion of the wolffian duct. It is an unusual cause of infertility and dysuria among young males. It is associated with ipsilateral renal agenesis, seminal vesical cyst, and ejaculatory duct obstruction. We herein report a rare case of a Zinner syndrome, in a patient of primary infertility. He also presented with complaints of chronic pelvic pain and dysuria. Any young patients of male infertility or unspecified pelvic complaints should be evaluated for Zinner syndrome and surgical intervention is the mainstay treatment for symptomatic patients.

Ectopic Ureter: Spectrum of Magnetic Resonance Imaging Findings.

This study aims to describe the MRI findings of six patients with ectopic ureters in a tertiary care institute. 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. 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. 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.

Systematic Review of Zinner syndrome

Zinner syndrome is characterized as a pathology of congenital origin in which there is a unilateral absence of the kidney, presence of cysts and obstruction in the ejaculatory ducts. Being diagnosed in the stage of sexual activity of patients, it can cause problems in the person's sexuality or reproduction. The objective of this work was to carry out a systematic review of clinical cases reported in the literature between 2013 and 2023. 42 reports of clinical cases with Zinner syndrome were evaluated, with a total of 49 patients with a mean age of 22.5 years, the 77.55 % of the patients presented cysts, absence of the right kidney in 44.90 % of the patients, and 91.84 % presented pain. The most common treatment for this pathology was laparoscopy (16.33 %) and in the reviewed cases there were no complications or deaths. Identifying the different presentations of this syndrome could improve the speed of diagnosis and guarantee effective management, thereby improving the quality of life of patients.

Zinner syndrome: A rarer variant of a rare syndrome

Zinner syndrome, characterized by the combination of absent unilateral kidney, obstructed ejaculatory duct on the same side, and cysts in the seminal vesicle, represents a rare congenital malformation of the mesonephric duct. Its symptoms are often vague, ranging from urinary discomfort to painful ejaculation and infertility, making it difficult to diagnose without imaging. In this report, we discuss a less common form of Zinner syndrome identified through diagnostic imaging.

Incidental Zinner Syndrome in Nigeria: A Case Report

Zinner syndrome (ZS) is a rare urogenital condition characterised by the triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction, resulting from malformation during early embryogenesis of the mesonephric (Wolffian) duct. The authors present a 35-year-old male who was being evaluated for chronic hepatitis B virus infection. He was referred to the urology outpatient clinic on account of incidental ultrasound finding of solitary right kidney. General physical examination revealed a healthy-looking young male with a flat abdomen and no palpable enlarged organs. Digital rectal examination revealed normal sized prostate with no palpable pararectal masses. MRI of the pelvis revealed a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction. The clinical diagnosis was asymptomatic ZS. He is on yearly follow-up at the urology outpatient clinic for lower urinary tract symptoms, pelvic pain, painful ejaculation, features of infertility, and pelvic ultrasound. If any of these symptoms occur, he will be treated with an α-adrenergic receptor blocker, drainage of the seminal vesicle cyst, and appropriate treatment for infertility. He is also on active surveillance for viral hepatitis by the gastroenterology team. In conclusion, prompt referral and comprehensive radiological imaging investigations of patients with unilateral agenesis of the kidney will lead to increased identification and report of patients with ZS. There is paucity of literature reports on ZS in the authors’ environment, and this case report, to the best of the authors’ knowledge, is the first from Nigeria.

Zinner syndrome in pediatric age: Clinical image

Zinner Syndrome (ZS) is a rare genitourinary malformation characterized by the triad of unilateral seminal vesicle cyst (QVS), ipsilateral Multicystic Displastic Kidney (MCDK), and obstruction of the ejaculatory duct. This condition is rare in pediatric age and both diagnosis and treatment are challenging.

Zinner syndrome in pediatric patients: rare disease leading to challenging management.

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. 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. 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 4 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 9 mm cyst requiring a redo laparoscopic excision. 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.

Durable response to docetaxel in a case of primary seminal vesicle adenocarcinoma with zinner syndrome.

Primary adenocarcinoma of the seminal vesicles is a rare tumor. Congenital seminal vesicle cysts are often associated with unilateral renal dysgenesis or agenesis (Zinner syndrome). Zinner syndrome is a rare congenital malformation represented by a group of characteristics: ipsilateral renal agenesis, ejaculatory duct obstruction, and seminal vesicle cyst. There have been 214 cases in the literature with abdominal pain and lower urinary tract symptoms as the most common presentation. Most cases are diagnosed incidentally. We report a case of a 39-year-old male presenting with recurrent hematuria for 12 years, diagnosed as metastatic primary clear cell adenocarcinoma of seminal vesicle with Zinner syndrome. He was managed symptomatically with radiation therapy, hormonal therapy, and palliative chemotherapy. He is living with a well-controlled disease at present.

Zinner's Syndrome and Retroperitoneal Fibrosis: An Unknown Association.

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.

Presentation, imaging and fertility findings in 13 cases of confirmed Zinner syndrome

Presentation, imaging and fertility findings in 13 cases of confirmed Zinner syndrome

Zinner Syndrome: Left Unilateral Renal Dysgenesis with Seminal Vesicle Cyst and Ipsilateral Testicular Atrophy Associated with Azoospermia - Case Report

Seminal vesicular cysts associated with ipsilateral renal agenesis or hypoplasia are a rare urological abnormality. This combination of urinary and genital abnormalities occurs due to the closely related embryological origin of these structures from the distal mesonephric duct [Wolff]. This case illustrates the discovery of this malformation in adulthood following chronic pelvic pain associated with infertility. The diagnostic approach consisted of a clinical examination with the performance of a set of imaging examinations including a suprapubic ultrasound, a scanner and an MRI. While the therapeutic management was limited to an ultrasound-guided evacuating puncture of the cyst of the seminal vesicle with a bacteriological and histological analysis of the puncture liquid. Given the complexity of the case, our surgical treatment is mainly aimed at relieving pain by evacuating puncture and offering medically assisted procreation for her infertility. We report the case of a young patient with Zinner's syndrome associated with infertility.

Zinner Syndrome: Case Series and Literature Review

Zinner syndrome is a rare congenital anomaly of the mesonephric duct, characterized by a triad of symptoms: seminal vesicle cysts, ipsilateral renal agenesis and ejaculatory duct obstruction. This leads to a severe complication — oligozoospermia/azospermia, which can subsequently cause infertility. The widespread use of medical imaging increases the probability of incidental detection. Namely, magnetic resonance imaging (MRI) is the imaging modality of choice for making a diagnosis. Study purpose: to optimize patient routing in Zinner syndrome, as well as to minimize the risk of misdiagnosis or missed pathology, by providing strong and weak points for each modality. Materials and methods: we present two clinical cases of Zinner syndrome. The first one is a complicated course in a 25-year-old patient, and the second one is accidentally discovered in a 27-year-old patient. The patients underwent a comprehensive diagnostic panel, including: ultrasound (US), computed tomography (CT), magnetic resonance imaging (MRI). The results obtained were analyzed in the light of available literature data. Results: in most cases, Zinner syndrome is an incidental finding during. The diagnosis based on these imaging methods and the correct patient routing allowed us to make a timely and correct diagnosis, followed by decisions on further treatment tactics. Conclusion: Zinner syndrome is a rare disease and is often diagnosed based on imaging findings only. A radiologist and clinician need to know about the diagnostic criteria for this syndrome in order to successfully diagnose and determine the optimal treatment tactics.

Zinner's syndrome: clinical case of a rare pathology

Zinner’s syndrome is a rare congenital urogenital anomaly characterised by the triad of seminal vesicle cyst, ipsilateral renal aplasia, and seminal duct obstruction. According to the published data, the incidence of seminal vesicle cysts with ipsilateral renal agenesis is 0.0046%, but the true incidence appears to be higher. The article presents a rare clinical case of patient K, 17 years old, followed since birth with a solitary right kidney. The patient was repeatedly examined (ultrasound, cystography, urography, abdominal MRI, pelvic CT). The boy was admitted to our clinic with the diagnosis of "Solitary right kidney" and pelvic cystic formation detected according to the last CT scan. MRI examination of the small pelvis, cystoscopy, diagnostic laparoscopy, puncture of the cystic mass were performed. The diagnosis of "Zinner's syndrome" was made. The size of the cyst did not change during follow-up.

Screening for Zinner Syndrome in Patients With a Congenitally Solitary Kidney: Lessons Learned.

We determined if serial screening ultrasounds are beneficial in evaluating for the development of Zinner syndrome in males with a congenital solitary kidney. All patients included had their congenital solitary kidney diagnosed at <20 years of age and had to be ≥20 at their last visit. Individuals were seen annually, with pelvic ultrasounds to screen for mesonephric duct cysts obtained at birth and every year of age, divisible by 5. At a median follow-up of 38 years of age (range 20-57), 17% (20/121) developed Zinner syndrome, with 60% (12/20) developing clinical symptoms. The yield for screening ultrasound studies was significantly higher in patients ≤20 years of age at 3.5% (12/340), compared to 0.33% (1/296) in patients >20 years of age (P = .004). Serial ultrasounds reveal the onset of lower urinary tract and cyst-related pain symptoms are associated with the growth of the seminal vesicle cyst to ≥5 cm (P = .0198). Of symptomatic patients, 75% (8/12) had abnormal uroflows. Complete urodynamic studies revealed findings consistent with bladder outlet obstruction in 38% (3/8), equivocal for obstruction in 24% (2/8), and detrusor underactivity in 38% (3/8). Cyst excision effectively resolved voiding symptoms that were obstructive in etiology but failed to resolve symptoms in patients with detrusor underactivity. Serial ultrasound evaluations reveal that cyst growth to ≥5 cm in size is highly related to the onset of clinical symptoms, with the resolution of voiding symptoms by cyst excision directly associated with urodynamic findings.