Zinner syndrome in children: clinical presentation, imaging findings, diagnosis, and outcome.

BACKGROUND: Zinner syndrome (ZS), the association of congenital seminal vesicle cyst (SVC) and ipsilateral kidney anomalies, is rarely diagnosed in childhood. This study aimed to assess presentation, imaging findings, management, and outcome of pediatric ZS. METHODS: Sixteen children with ZS were diagnosed and managed at our hospital from 2003 to 2021. We reviewed the medical records to collect data on initial symptoms, results of imaging studies, complications, operation, and follow-up. RESULTS: Ultrasound was used in all 16 cases as initial diagnostic tool. Fourteen patients were asymptomatic at diagnosis: these were transferred from obstetricians or pediatricians for evaluation of the prenatally or postnatally detected ultrasonic kidney anomalies. SVCs were incidentally noted on ultrasonography. The other two cases initially presented with urinary tract infection (UTI). Kidney anomalies included multicystic dysplastic kidney in 3 and kidney agenesis in 13 patients. Eleven (68.7%) patients had ipsilateral ectopic ureters entering SVC. Four (36.4%) patients had a reflux from urethra into SVC (urethro-cystic reflux) on voiding cystourethrography. Ten (62.5%) patients remained asymptomatic over a mean of 58 months (range, 7-216 months), two patients developed lower urinary tract dysfunction, and five patients had UTIs. Two boys needed SVC removal, and SVC had disappeared in two patients after 2.5-4 years of follow-up. CONCLUSIONS: Unilateral kidney hypodysplasia with ectopic ureter inserting into the ipsilateral SVC is a characteristic sign for diagnosis of ZS. In our case series, ZS was mainly asymptomatic. Urethro-cystic reflux was associated with UTIs in young infants. SVC removal was rarely required. A higher resolution version of the Graphical abstract is available as Supplementary information.

Zinner Syndrome with Papillary Neoplasm: A Rare Case Report.

One of the uncommon urogenital malformations in males, which presents late in the second or third decades of life, is the congenital malformation of the seminal vesicle. Zinner syndrome is a rare condition comprising a triad of unilateral renal agenesis, ipsilateral seminal vesicle obstruction, and ipsilateral ejaculatory duct obstruction. Very few cases in the literature describe the malignant change in seminal vesicle cyst in Zinner syndrome. To the best of our knowledge, this is the first report of a papillary neoplasm of the seminal vesicle cyst developed in a patient with Zinner syndrome.

[Surgical treatment of Zinner syndrome].

Zinners syndrome (SC) is a rare congenital disease characterized by ejaculatory duct obstruction, seminal vesicle cyst in combination with ipsilateral renal agenesis. This syndrome is due to development arrest of the Wolffian duct (mesonephros). Before the onset of sexual activity, the disease is asymptomatic. The main symptoms are nonspecific, including dysuria, urinary frequency, perineal and scrotal pain after ejaculation. A clinical case with the presentation of our own experience of surgical robot-assisted treatment of a patient with Zinners syndrome is presented in the article.

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.

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.

[Zinner syndrome:A case report and review of the literature].

OBJECTIVE: To explore the clinical diagnosis and treatment of seminal vesicle cyst (SVC) associated with ipsilateral renal agenesis (Zinner syndrome) in order to promote the understanding of the disease. METHODS: We retrospectively analyzed the clinical data about 1 case ofZinner syndrome diagnosed and treated in our hospital and reviewed the literature related to this disease in domestic and foreign authoritative databases. RESULTS: The patient was a 23-year-old male, diagnosed with Zinner syndrome, treated bytransrectal aspiration of SVC, and discharged from hospital 3 days postoperatively. Follow-upat 6 months after discharge found that the patient no longer felt perineal discomfort in the endstage of urination, but transrectal ultrasonography of the prostate revealedthe samevolume of fluid in the left seminal vesicles as before,which indicated recurrence. CONCLUSIONS: SVC associated with ipsilateral renal agenesis can be considered asZinner syndrome. Transrectal aspiration of SVCcan relieve the local symptoms of the patient but relapse may easilyoccur. Therefore it is not recommended as the first-choice treatment of the disease.

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.

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.

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.

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

INTRODUCTION: 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. MATERIAL AND METHODS: A retrospective case review of pediatric ZS diagnosed at our institution from 2019 to 2023, alongside a comprehensive literature review. RESULTS: 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. DISCUSSION: 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. CONCLUSION: 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 variant of Zinner syndrome with ectopic ureteral insertion into the seminal vesicle.

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.

Laparoscopic excision of huge seminal vesicle cyst in Zinner syndrome.

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 ∼12.5-cm seminal vesicle cyst was excised with the laparoscopic method without any perioperative complication in this case.

Zinner syndrome: A first case report from Ethiopia.

A triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction has been called Zinner syndrome since its first description in 1914 by Zinner. It is a very rare congenital abnormality of the male genitourinary tract due to abnormal embryologic development of the Wolffian ducts. There have been several case reports from different parts of the world about the clinical and imaging findings of these anomaly. In this case report, we present the first case report of Zinner syndrome in a 28-year-old male Ethiopian patient.

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's syndrome: Masquerading as pyonephrotic ectopic kidney.

Zinner's syndrome is a rare developmental anomaly of Wolffian duct, comprising a triad of seminal vesicle cyst, ipsilateral renal agenesis and ejaculatory duct obstruction, first described by Zinner in 1914. Several aberrations have been reported like renal dysplasia, ectopic ureteric orifice in one of the derivatives of Wolffian duct. Usually it presents in second to fourth decade of life with symptoms of urinary bladder irritation/obstruction, cyst distension, ejaculatory duct obstruction. The diagnosis is principally based on imaging studies, usually confirmed by MRI. Treatment is based upon the persistent symptoms or complications related to it. Excision of cyst is gold standard.

Zinner's syndrome: A rare diagnosis of congenital seminal vesicle cyst and renal agenesis on basis of radiological imaging and its management.

INTRODUCTION AND IMPORTANCE: Seminal vesical cyst (SVC) together with ipsilateral renal agenesis known as Zinner's syndrome is a rare congenital malformation mostly asymptomatic and is detected in second to fourth decade of life presenting with symptoms of bowel and bladder neck irritation/obstruction. Diagnosis is based mainly on various imaging techniques with MRI being the confirmatory. Recent management includes laparoscopic excision of the SVC but conventional aspiration of the cystic fluid together with explorative open excision of the cyst is still regarded useful. CASE PRESENTATION: A 32 years old male presented with urgency and frequency of micturition, constipation, post ejaculatory pain and USG, CT, MRI findings suggestive of right renal agenesis and bilateral seminal vesicle cyst. CLINICAL DISCUSSION: As other studies show, our patient is a sexually active male with characteristic symptoms of bladder and bowel obstruction. USG, CT, MRI were used for diagnosis co-relating other studies. Intervention was done by aspiration of the cystic fluid and open surgical excision of the cyst. CONCLUSION: Zinner's Syndrome is uncommon cause of symptoms of bowel and bladder obstruction in young men; whose diagnosis is mostly based on imaging techniques.

Transperitoneal Laparoscopic Unroofing versus Fenestration Under Seminal Vesiculoscopy for Seminal Vesicle Cyst, a Multi-Institutional Retrospective Cohort Study.

Background: To illustrate the transperitoneal laparoscopic unroofing (TLU) and compare the efficacy and safety of TLU to fenestration under seminal vesiculoscopy (FUSV) in treating symptomatic seminal vesicle cyst (SVC). Methods: We retrospectively reviewed all patients with symptomatic SVC who underwent TLU or FUSV between 2008 and 2020 at 3 institutions in Hunan. The two groups were evaluated with reference to radiological failure-free survival (R-FFS), fertility outcome, symptoms, and complications at a median 33.5-month follow-up. Results: Of the 98 males, 58 (59.2%) received TLU, and 40 (40.8%) underwent FUSV. Baseline characteristics were comparable. Semen analysis, prostatitis-like symptoms, and the maximum diameter of cyst were partially improved after both surgeries at 12-month follow-up. The TLU groups suggested a higher incidence rate of fertility for SVC patients with comorbid infertility compared with the FUSV group (82.4% vs 70.3%, p = 0.041), as well as better R-FFS of cysts at five-year follow-up (Log rank test, p = 0.021). In addition, the number of patients with NIH-CPSI (National Institutes of Health Chronic Prostatitis Symptom Index) scores higher than 15 decreased more significantly after TLU (p = 0.004). Except for hematospermia within 3 months, no significant difference in adverse events was observed in the two groups during the long-term follow-up. Conclusion: TLU was superior for patients with large and symptomatic SVC to FUSV, with more relieved symptoms, better R-FFS of cysts and fertility outcomes. Registration Number of Clinical Trial: ChiCTR2100053850 in Chinese Clinical Trial Registry Platform (ChiCTR).

Zinner Syndrome: Ipsilateral Renal Agenesis and Seminal Vesicle Cyst Presenting With Bony Metastasis.

Zinner syndrome is a rare congenital triad of mesonephric duct abnormality encompassing unilateral renal agenesis or dysgenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction. Literature has reported 214 cases, with the most common presentation being lower urinary tract symptoms and abdominal pain. Most cases are incidentally diagnosed, and MRI has been the choice of radiological diagnosis. We report the case of an 81-year-old male who presented with a three-month history of a fungating elbow lesion, elbow pain, and weight loss. Imaging revealed an ipsilateral seminal vesicle cyst, absent kidney, and ejaculatory duct obstruction, i.e., Zinner syndrome with bone metastasis. A bone biopsy revealed a urothelial primary, and cyst aspiration and cytology revealed spermatozoa and malignant cells representing an adenocarcinoma. This patient was managed with symptom control, radiotherapy to the elbow, and palliative chemotherapy, but later succumbed to the condition.

Zinner Syndrome.

Zinner syndrome is a developmental anomaly of the urogenital tract. This condition is defined by the triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction. The syndrome is due to malformation of the mesonephric duct during embryogenesis. The condition used to be rare but is now frequently encountered due to the advent of MRI and CT. MRI confirms the diagnosis by revealing the seminal vesicle cyst and its contents, and the ejaculatory duct obstruction, while CT confirms renal agenesis. We report the case of a young patient with Zinner syndrome. LEARNING POINTS: Zinner syndrome consists of the triad of renal agenesis, seminal vesicle cyst and ejaculatory duct obstruction.Any insult during embryogenesis of the mesonephric duct in men can result in Zinner syndrome.Pelvic MRI is the gold standard to confirm the diagnosis of Zinner syndrome.

A rare case of Zinner syndrome: Triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ejaculatory duct obstruction.

A rare triad of Wolffian duct anomaly known as Zinner syndrome includes unilateral renal agenesis with ipsilateral seminal vesicle cyst and ejaculatory duct obstruction. It is often diagnosed in third and fourth decades of life. Patient presents with dysuria, perineal pain, infertility and painful ejaculation. The aim of this case report is to show the importance of the radiological imaging on diagnosis of Zinner syndrome. MRI being the modality of the choice for the confirmation of the diagnosis is vital in further management of the syndrome.

Zinner Syndrome: A Case Report of a Rare Etiology of Infertility.

Infertility is a common medical issue with different etiologies. It can be related to female factors, male factors, or factors related to both partners. We report the case of a 35-year-old male patient who presented with primary infertility for 10 years. He was otherwise healthy with unremarkable past medical history. Physical examination revealed normal external genitalia with both testes were normal in size. Basic semen analysis revealed decreased sperm volume and sperm count. Abdominal ultrasound examination revealed an absent right kidney. Subsequently, the patient under a computed tomography scan that confirmed the right renal agenesis and demonstrated a well-defined right seminal vesicle cyst. Such findings were consistent with the diagnosis of Zinner syndrome. He underwent aspiration of the cyst that resulted in improvement in the sperm parameter of basic semen analysis. The case demonstrated a rare etiology of male infertility that was successfully managed conservatively. Despite its rarity, physicians should consider the developmental anomalies of the genitourinary system when encountering patients with infertility.