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

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 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. 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.

Zinner Syndrome: Radiologic Diagnosis in a Rare Case.

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.

Study on the effect of completely preserving the ejaculatory duct during prostatectomy on reducing postoperative retrograde ejaculation in benign prostatic hyperplasia patients.

Introduction: Preserving the sexual function of benign prostatic hyperplasia (BPH) patients and reducing the incidence of postoperative retrograde ejaculation are critical for BPH patients with sexual needs. Aim: To explore the effect of complete preservation of the seminal tract during transurethral prostatectomy (TURP) on reducing retrograde ejaculation in BPH patients. Material and methods: BPH patients meeting the inclusion criteria were randomly divided into the Control group (traditional TURP) and the Experimental group (complete reserved ejaculatory duct) in a ratio of 1 : 1. Finally, data of 64 BPH patients - 34 in the Control group and 30 in the Experimental group - were analyzed. We measured the preoperative and postoperative maximum urinary flow rate (Qmax), International Prostate Symptom Score (IPSS), Quality of Life (QOL) score, semen volume, and ejaculation function. Results: Compared with pre-operation values, patients in the two groups exhibited increased Qmax and decreased IPSS and QOL scores after the operation. However, there was no significant difference in Qmax, IPSS, or QOL between the Control and Experimental groups after the operation. The two groups of patients had a significant reduction in postoperative ejaculation. Compared with the Control group, the semen volume of patients was higher, and the incidence of retrograde ejaculation was lower in the Experimental group. Conclusions: Prostatectomy with complete preservation of the seminal tract is not different from conventional electrosurgical resection in improving urination symptoms, while the incidence of retrograde ejaculation is significantly lower.

The Male Reproductive System of the Kissing Bug, Rhodnius prolixus Stål, 1859 (Hemiptera: Reduviidae: Triatominae): Arrangements of the Muscles and the Myoactivity of the Selected Neuropeptides.

The gross anatomy of the male reproductive structures and their associated musculature are described in the blood-gorging vector of Chagas disease, Rhodnius prolixus. The male reproductive system is composed of muscular tissues each performing contractions that aid in the movement of sperm out of the testis into the vas deferens, seminal vesicle and then into the ejaculatory duct, along with proteins and lipids from the transparent and opaque accessory glands. Phalloidin staining shows the various patterns of muscle fiber layers, from thin circular to more complex crisscross patterns, implying subtle differences in the form of the contractions and movement of each of the structures, allowing for waves of contractions or twisting patterns. The transcripts for the receptors for proctolin, myosuppressin (RhoprMS) and for the extended FMRFamides are expressed in the various regions of the reproductive system, and the nerve processes extending over the reproductive structures are positive for FMRFamide-like immunoreactivity, as are neurosecretory cells lying on the nerves. Proctolin and AKDNFIRFamide are strong stimulators for the frequency of the contractions, and RhoprMS can inhibit the proctolin-induced contractions. Taken together, this work implicates these two families of peptides in coordinating the male reproductive structures for the successful transfer of sperm and the associated accessory gland fluid to the female during copulation.

Loss of Adgra3 causes obstructive azoospermia with high penetrance in male mice.

The adhesion receptor ADGRA3 (GPR125) is a known spermatogonial stem cell marker, but its impact on male reproduction and fertility has not been examined. Using a mouse model lacking Adgra3 (Adgra3-/- ), we show that 55% of the male mice are infertile from puberty despite having normal spermatogenesis and epididymal sperm count. Instead, male mice lacking Adgra3 exhibited decreased estrogen receptor alpha expression and transient dilation of the epididymis. Combined with an increased estradiol production, this indicates a post-pubertal hormonal imbalance and fluid retention. Dye injection revealed a blockage between the ejaculatory duct and the urethra, which is rare in mice suffering from infertility, thereby mimicking the etiologies of obstructive azoospermia found in human male infertility. To summarize, male reproductive tract development is dependent on ADGRA3 function that in concert with estrogen signaling may influence fluid handling during sperm maturation and storage.

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.

Challenging clinical presentation of Zinner syndrome.

Zinner syndrome is a rare congenital malformation of the mesonephric duct comprising of seminal vesicle cyst, ipsilateral renal agenesis, and ejaculatory duct obstruction. Clinical presentation varies with perineal pain, painful ejaculation, hematospermia and infertility common presenting complaints. Here, we present a case of Zinner syndrome in a 35-year-old male with a rare clinical presentation of only abdominal discomfort. The purpose of this case report is to highlight the challenging clinical presentation of Zinner syndrome and the use of imaging modalities in diagnosing the condition.

[Seminal vesicoscopy in the treatment of seminal abnormalities associated with ejaculatory duct obstruction：Advances in studies].

Obstructive azoospermia is a kind of common clinical disease, which often happens in epididymis, vas deferens and ejaculatory duct obstruction and accounts for male infertility. Azoospermia, which is caused by obstruction of the ejaculatory duct, is relatively rare. but the development of endoscopic technology now allows the disease to be resolved by surgery. In the past, transurethral resection of ejaculatory duct (TURED) has been used to treat patients with ejaculatory duct obstruction and azoosperm-free disease. But with the further study of TURED operation in recent years, as well as in patients with postoperative follow-up results showed that the urine - tube reflux and retrograde ejaculation ejaculation the incidence of iatrogenic injury is higherduring the TURED.therefore, in recent years, some experts committed to seeking a less damage and better effect of the surgical method. With the continuous development of endoscopic technology, seminal vesiculoscopy has come into being, which also creates conditions for further understanding the structure and function of the seminal vesicle and distal seminal duct, accurate diagnosis and minimally invasive treatment of the currently discovered diseases. In this article, we summarized the semen abnormalities associated with seminal vesiculoscopy in the treatment of ejaculatory duct obstruction, and also reviewed the surgical methods, clinical application and research progress of seminal vesiculoscopy.

[Seminal vesiculoscopy-assisted thulium laser ablation for the treatment of ejaculatory duct obstruction: An analysis of clinical outcomes].

OBJECTIVE: To evaluate the clinical outcomes of seminal vesiculoscopy-assisted thulium laser ablation (SVS-TLA) in the treatment of oligoasthenozoospermia or azoospermia induced by ejaculatory duct obstruction (EDO). METHODS: We retrospectively analyzed the clinical data on 42 cases of EDO-induced oligoasthenozoospermia or azoospermia in our Clinic of Andrology from April 2018 to January 2020, all definitely diagnosed and treated by SVS-TLA. We followed up the patients regularly after operation, obtained their routine semen parameters at 3, 6 and 9 months postoperatively, examined them by t-test and compared them with the baseline. RESULTS: Operations were successfully completed in all the 42 cases, with an average surgery time of 52.7 minutes. Compared with the routine semen parameters collected 2 weeks before surgery, the semen volume, sperm concentration and total sperm motility of the patients were all significantly improved at 3, 6 and 9 months postoperatively (P < 0.01). Sperm were found in 40 cases at 3 months and in the other 2 cases at 6 and 9 months after surgery. Postoperative complications were observed in 7 cases, including epididymitis, perineal or testicular pain, and hematuria, which all disappeared after corresponding symptomatic treatment. No such serious complications as retrograde ejaculation, rectal injury, urethral stricture or urinary incontinence occurred in any of the cases after operation. CONCLUSION: SVS-TLA is a safe and effective option for the treatment of EDO, which can significantly improve the semen quality of the patient without causing serious postoperative complications.

A rare abnormality of ejaculatory duct opening in the bladder trigone in a 33-year-old male associated with primary infertility: Case report and literature review.

Genitourinary anomalies constitute a large proportion of congenital malformations. However seminal tract anomalies, particularly ejaculatory duct (ED) anomalies are very rare and little information exists on the topic. We are reporting a very rare case of bilateral ectopic EDs opening in the bladder trigone in a 33-year-old gentleman presenting for evaluation for primary infertility. The patient's semen analysis showed low-ejaculate-volume, fructose negative, acidic pH and azoospermia. His hormonal profile was normal. Cystoscopy revealed an empty posterior urethra, and the verumontanum and the openings of the EDs could not be identified in the posterior urethra. The ED openings were found inside the bladder trigone. Vasography combined with cystoscopy confirmed the opening of the ED in the trigone following Intra-vasal injection of methylene blue. Our patient had a successful intracytoplasmic sperm injection using testicular spermatozoa that resulted in a healthy baby boy. We also did a formal literature review through PUBMED, MEDLINE and Google Scholar with the search term (ectopic ED). Search results were filtered to exclude vas deferens ectopia. Our literature search revealed five studies comprising 24 patients with ectopic EDs. Mean age at diagnosis was 29.88 ± 12.88 years. The most common presenting symptom was hemospermia. The ectopic EDs most commonly opened in a midline cyst (21 cases), bladder trigone (1 case), or bladder neck (1 case). The most common management used for symptomatic patients with ectopic EDs opening in the midline cyst was through transurethral fenestration. In conclusion, ectopic ED openings in the bladder trigone are very rare. Management varies by case depending on the presentation, anatomy of underlying anomaly, associated complication/s and desire for fertility.

Zinner Syndrome.

Zinner syndrome is a less common birth anomaly of the Wolffian duct consisting of unilateral kidney absence, ipsilateral ejaculatory duct obstruction, and seminal vesicle cyst. A failure of embryogenesis of the ureteric bud between the fourth and 13th week of gestation results in Zinner syndrome. Conservative treatment is recommended for asymptomatic patients, whereas invasive treatment is reserved for symptomatic patients and for those who have failed conservative treatment. In this case report, we describe the non-specific presentation of lower abdominal pain and dysuria, as well as episodes of hematuria and new-onset hypertension, in a male patient, who was otherwise deemed healthy, with no other previous medical or surgical history. An imaging study and laboratory investigations were performed, and the patient was detected to have left renal agenesis and hypointense/hyperintense cysts in the left seminal vesicle of the left kidney. The findings supported the diagnosis of Zinner syndrome. The patient did not present with any symptoms or findings that would suggest infertility at the time of the study. Zinner syndrome is a rare cause of painful micturition and hematuria in males and can be diagnosed using ultrasound (USS), computer tomography (CT), and magnetic resonance imaging (MRI) techniques. Zinner syndrome should be considered as a differential diagnosis in male patients with unilateral renal agenesis and cystic pelvic masses. Patients who are asymptomatic typically undergo conservative treatment and are followed up to prevent infertility. For patients with symptomatic cysts who fail to respond to conservative treatment or whose cysts are larger than 5 cm in diameter, surgical intervention is recommended (open or laparoscopic surgery and ejaculatory duct balloon dilatation).

Ultrastructure of Ejaculatory Ducts of Cerapanorpa nanwutaina and Furcatopanorpa longihypovalva (Mecoptera: Panorpidae).

The ultrastructure of the ejaculatory duct was investigated in the scorpionflies Cerapanorpa nanwutaina (Chou 1981) and Furcatopanorpa longihypovalva (Hua & Cai, 2009) (Mecoptera: Panorpidae) using light and transmission electron microscopy. The ejaculatory ducts of both species comprise a median duct and an accessory sac. The median duct consists of a basal lamina, a mono-layered epithelium, a subcuticular cavity, and an inner cuticle. The accessory sac contains a single layer of epithelium and a basal lamina. A muscular layer is present in the accessory sac of C. nanwutaina and in the median duct of F. longihypovalva. The epithelia in the median duct and the accessory sac are well developed, their cells containing numerous cisterns of rough endoplasmic reticulum, mitochondria, and microvilli. The secretions of the median duct are first extruded into the subcuticular cavity and then into the lumen through an inner cuticle, while the secretions of the accessory sac are discharged directly into the lumen. The ejaculatory duct of F. longihypovalva is longer and has thicker epithelium with more cell organelles and secretions than that of C. nanwutaina.

Efficacy analysis of 26 cases of ejaculatory duct obstruction treated by prostatic utricle neck endoscopy.

Objective: To evaluate the safety and efficacy of transvesical incision in the treatment of ejaculatory duct obstruction. Methods: The clinical data of 26 male infertile patients with ejaculatory duct obstruction were retrospectively analysed at the First Affiliated Hospital of Zhengzhou University from June 2020 to August 2021. All patients were treated with seminal vesicle neck incision for ejaculatory duct obstruction. The general clinical characteristics, intraoperative conditions and postoperative effects on the patients were recorded, and the therapeutic effect was evaluated. Results: The ejaculatory duct was found through fenestration, and the seminal vesicle gland was smoothly entered in 25 patients (96.2%). Among them, 22 cases underwent bilateral endoscopy and three underwent unilateral endoscopy. Sperm appeared in 23 cases (88.5%) 3 months after surgery. The sperm concentration and motility postoperatively at 6 months were higher than that at 3 months postoperatively. No postoperative complications, such as epididymitis or retrograde ejaculation, occurred. Conclusion: Searching for the ejaculatory duct via the neck of the prostatic utricle, assisted by a low-energy holmium laser, is a new method for the treatment of ejaculatory duct obstruction. Microscopic vision is clear using this approach and the postoperative complications are few, which has high value for clinical application.

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.

Establishment of an animal model of ejaculatory duct obstruction.

This study aimed to establish animal models with different degrees of ejaculatory duct obstruction. Forty-eight male rats aged 14-15 weeks were randomly divided into three groups (n = 16): control, complete ejaculatory duct obstruction (tied around the lower seminal vesicle gland and ductus deferens with a 2-0 silk ligature), and partial ejaculatory duct obstruction (padded with a wire guide). Mortality, complications, seminal vesicle morphology and histopathology were compared in the three groups at 4 and 8 weeks postoperatively. In the complete ejaculatory duct obstruction group, seminal vesicle weight decreased gradually with increased obstruction time compared with those of the control group (p < 0.05); moreover, stone-like material was occasionally observed. In the partial ejaculatory duct obstruction group, there was an increase followed by a decrease in seminal vesicle weight in the postoperative period compared with that of the control group (p < 0.05). Histopathological lesions of seminal vesicles were observed in the complete and partial ejaculatory duct obstruction groups (8 weeks postoperatively). We successfully established animal models of complete and partial ejaculatory duct obstruction, which provide an easy-to-use tool for studying seminal vesicle changes after ejaculatory duct obstruction.

Zinner Syndrome: A Case Report of Rare Urogenital Anomaly.

Zinner Syndrome is a rare urogenital tract anomaly with unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction. The syndrome occurs due to an insult to the development of the paramesonephric duct during embryogenesis. Patients may present late due to dysejaculation as a result of obstruction of the ejaculatory duct or commonly remain asymptomatic. Here, we present such a case presenting with dysejaculation which was diagnosed with Zinner syndrome on ultrasound.

Long-Term Success Durability of Transurethral Resection of Ejaculatory Duct in Treating Infertile Men with Ejaculatory Duct Obstruction.

Objectives: The current study aimed to evaluate the long-term outcomes of TURED (transurethral resection of the ejaculatory duct) in treating male infertility due to ejaculatory duct obstruction (EDO) to the short-term follow-up. Methods: From 2000 to 2010, we retrospectively reviewed 30 men with infertility resulting from EDO who underwent TURED. Information on preoperative evaluation of patients was collected from the medical records. Standardized semen analysis was performed 6 weeks, 3 months, and 12 months postoperatively, as well as after a 7-year follow-up. Results: The average age of patients was 35.5 ± 2.6. All patients had low ejaculate volume and normal range of the mean serum hormone levels (gonadotropins and testosterone). Transrectal ultrasonography (TRUS) assessment revealed midline cysts in 46.7% (n = 14 cases), a seminal vesicle dilatation in 83.3% (25/30 cases), and ejaculatory duct dilatation in 76.7% (23/30 patients). In addition, two patients (6.6%) underwent MRI pelvis to confirm equivocal EDO by TRUS. After short-term and long-term follow-up, semen parameters improved significantly in 90% of cases vs 63.3% of cases, respectively, with statistically insignificant differences in all parameters (p ≥ 0.1). Based on subgroup analysis, semen parameters improved in all partial EDO and cystic EDO cases vs complete EDO and noncystic EDO, respectively. Spontaneous pregnancies were achieved in 10 (33.3%) vs 8 (26.6%) cases after short and long term, respectively. Regarding post-TURED complications, we reported 23.3% (n = 7). Conclusion: TURED is an effective treatment for EDO. It significantly improves all semen parameters and improves the symptoms related to EDO after short and long-term follow-up.

Ejaculatory duct cyst causing bilateral obstruction and subsequent infertility: A rare case report from Indonesia.

An ejaculatory duct cyst is a rare and challenging problem for a urologist. Diagnosing these cases may be difficult as prior case reports showed that the condition has various clinical presentations from asymptomatic to major complications, such as recurrent urinary tract infections and infertility. We aimed to report a 26-year-old married man with seemingly unknown infertility, which was confirmed to be due to an ejaculatory duct cyst obstructing both ejaculatory ducts.

Ejaculatory duct reflux revealed by chronic scrotal swelling in an adult.

Ejaculatory duct reflux is rare and few investigations have focused on this entity, which is usually described in children. This study reports a new case of unilateral ejaculatory reflux in a 32-year-old patient, with a history of urethroplasty at the age of 5 for hypospadias, who presented for right chronic scrotal swelling. Urethrocystscopy showed an anterior urethral stricture and a gaping opening of the right ejaculatory duct. CT scan with opacification through the right ejaculatory duct, showed a dilated right seminal vesicle, associated to a reflux in the right deferent vas and epididymis, which was dilated explaining the scrotal swelling.