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: 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.

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.

Multiple vas deferens with polyorchidism and many congenital malformations in a symptomatic 11-year-old male patient: a rare case report.

BACKGROUND: Ductus deferens may manifest in a variety of anomalies such as its absence, duplication, ectopy, or diverticulum. Ectopic seminal tract opening has two main types, ectopic ejaculatory duct opening, and ectopic vas deferens opening. Generally, ductus deferens anomalies affect approximately 0.05% of the population. Patients may be asymptomatic or complaining of urinary tract infections and/or epididymitis. Most of these cases are associated with renal dysplasia. To confirm the diagnosis Cystourethroscopy catheterization and retrograde urethrogram should be performed, but the definitive diagnosis is done by vasography. The definitive treatment is complete surgical resection of the pathological urogenital connection. This case is commonly discovered while exploring other findings such as testicular torsion and inguinal hernia. CASE PRESENTATION: We report a rare case of an 11-year-old male who presented with gross hematuria and numerous congenital malformations including a left polydactyly clubfoot, polyorchidism, with several surgical procedures, and left kidney dysgenesis. Surgery was performed for a left inguinal hernia, during which a third undescended testicle was discovered incidentally and was eradicated. A retrograde urethrogram was performed to establish the diagnosis. A fistula- that is connected with the left ureter- was resected. The histopathologic findings confirmed the diagnosis of true duplication of the Vas deferens, with communication between the ureter and the vas deferens. By follow-up, the kidney function tests were within normal limits. CONCLUSIONS: This case report aims to highlight the early diagnosis and management of the duplicated vas deferens and the associated congenital malformations to improve the prognosis and kidney function and to avoid long-term complications.

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.

A 30-year retrospective study of rare ectopic seminal tract opening cases.

Ectopic seminal tract opening is a rare congenital malformation. Until recently, there has been a lack of comprehensive reporting on the condition. The purpose of this retrospective study is to summarize the experience of diagnosis and treatment of this condition based on 28 clinical practice cases throughout the past 30 years. We conducted auxiliary examinations on such patients including routine tests, imaging examinations, and endoscopy. Among these 28 cases, there were ectopic opening of vas deferens into enlarged prostatic utricles (6 cases); ejaculatory ducts into enlarged prostatic utricles, Müllerian ducts cysts, and urethras (18 cases, 2 cases, and 1 case, respectively); and ectopic opening of the unilateral vas deferens and the contralateral ejaculatory duct into enlarged prostatic utricle (1 case). The size of the enlarged prostatic utricle, the type of ectopic seminal tract opening, and the opening's location effectively assisted in the selection of clinical treatment methods, including transurethral fenestration of the utricle, transurethral cold-knife incision, open operation, laparoscopic operation, and conservative treatment. Satisfactory effect was achieved during follow-up. In conclusion, a definite diagnosis and personalized treatment are especially important for patients with ectopic seminal tract opening.

Clinical Consultation Guide on Imaging in Male Infertility and Sexual dysfunction.

Imaging can benefit clinicians in evaluating men with infertility or sexual dysfunction by giving an overview of a patient's overall clinical condition before undertaking an invasive procedure. An understanding of the limitations and advantages of image modalities used in clinical practice will ensure that clinicians can optimize patient care with imaging when necessary. PATIENT SUMMARY: The objective of this article was to review the current literature on imaging modalities used for the diagnosis and management of male infertility and sexual dysfunction. An understanding of the advantages and limitations of these imaging modalities will ensure that clinicians can optimize patient care with imaging when necessary.

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.

[Congenital bilateral ejaculatory duct absence complicated with seminal vesicle cyst: a case report and review of the literature].

OBJECTIVE: To report a case of simple congenital bilateral ejaculatory duct absence (EDA) complicated with seminal vesicle cyst and review the relevant literature in order to improve the diagnosis and treatment of the disease. METHODS: We retrospectively reviewed the clinical data of a case of bilateral congenital EDA complicated with seminal vesicle cyst, reviewed the relevant literature at home and abroad, and comprehensively analyzed the embryonic development, diagnosis, and treatment of congenital EDA. RESULTS: The patient was a 23-year-old man, present at the clinic for infertility after married for a year. Vasography and other imaging examinations confirmed simple congenital bilateral EDA complicated with seminal vesicle cyst. Pathologic biopsy showed normal spermatogenic function of the testes. CONCLUSION: Congenital EDA originates from embryonic developmental defect of the mesonephric duct, and it can be confirmed by vasography. Transurethral incision of the ejaculatory duct and intracytoplasmic sperm injection can be employed for the treatment of bilateral EDA.

[Congenital obstruction of the ejaculatory ducts: diagnosis and resolution of two successful cases]. / Obstrução congénita dos canais ejaculadores: Diagnóstico e resolução em dois casos de sucesso.

The ejaculatory ducts obstruction is an uncommon finding in the study of infertility male factor. We present two cases referred to our department for conjugal infertility, with proven male factor (low ejaculate volume and azoospermia). On physical examination there was no abnormality in both patients. Imaging studies demonstrated intra-prostatic cystic structure, causing ejaculatory ducts dilation. We performed transurethral resection of the ejaculatory ducts. Semen analysis 3 months postoperatively showed 1 case of normalization and 1 case of improvement in sperm count parameters. The couple 1 couple has 2 children, without medical assisted reproductive techniques. The couple 2 had one pregnancy after Intracytoplasmic Sperm Injection, from the ejaculate. This diagnosis depends on a strong clinical suspicion and is of high importance, since this disease has effective surgical treatment. The authors aim to highlight the relevance of the assessment andrological when studying marital infertility.

A obstrução dos canais ejaculadores é um achado infrequente no estudo do factor masculino em casais inférteis. Apresentamos dois casos referenciados à nossa consulta por infertilidade conjugal primária, com factor masculino comprovado (ejaculado de baixo volume e azoospermia). Ao exame objectivo masculino não havia alterações em ambos os pacientes. Os exames de imagem demonstraram estrutura quística intra-prostática, condicionando dilatação dos canais ejaculadores. Foram submetidos a ressecção transuretral dos canais ejaculadores. O espermograma 3 meses pós-operatório demonstrou 1 caso de normalização e 1 caso de melhoria dos parâmetros espermáticos. O casal 1 tem 2 filhos, sem necessidade de técnicas de reprodução medicamente assistida. O casal 2 apresentou gravidez após Injecção Intracitoplasmática de Espermatozóides do ejaculado. Este diagnóstico depende de uma suspeita clínica forte e é de elevada importância, pois esta patologia tem tratamento cirúrgico eficaz. Assim, os autores pretendem realçar a relevância da avaliação andrológica aquando do estudo da infertilidade conjugal.

Ejaculatory ducts opening into accessory urethral channel with hypospadias and absent verumontanum: a rare association.

Ejaculatory ducts draining into accessory urethral channel opening into perineum is rare. This is a case report of a 27-year-old male who had hypospadias surgery at 3 years of age, presenting with discharge of semen through the perineal opening from adolescence. Cystoscopy and dye study showed that it was a short channel communicating with both ejaculatory ducts. Cystoscopy of the native urethra revealed that the vermontanum had not developed. The mucous lined accessory urethra was anastomosed to the bulbar urethra. Urethrogram done after one year showed that the accessory urethra was draining well into the bulbar urethra. Such type of accessory urethral channel communicating with ejaculatory ducts and associated with hypospadias and absent vermontanum has not been reported so far.

Hybrid method of transurethral resection of ejaculatory ducts using holmium:yttriumaluminium garnet laser on complete ejaculatory duct obstruction.

A 32-year old single man presented with azoospermia and low semen volume which was noted one and half a year ago. Transrectal ultrasonography and seminal vesiculography were performed to evaluate ejaculatory duct obstruction, and transurethral resection of the ejaculatory duct was performed using a hybrid technique of holmium:yttriumaluminium garnet laser with monopolar transurethral resection to overcome the narrow prostatic urethra. To our knowledge, this is the first report on the successful outcome of a hybrid technique applied for transurethral resection of the ejaculatory duct.

Ectopic opening of cystic dilatation of the ejaculatory duct into enlarged prostatic utricle.

A 28-year-old man was referred to our department for the management of recurrent hemospermia during the past 5 years. Genital examination and hormonal levels were normal. Semen analysis showed no change in volume and pH; however, hemospermia and asthenozoospermia were observed. Ultrasonography and computed tomography scan revealed the presence of a cystic lesion with calcification in the terminal part of seminal vesicles adjoining the prostate gland. The following vasography and endoscopic retrograde urethrography demonstrated 2 communicating cystic dilatations arising from the verumontanum. The diagnosis of cystic dilatation of the ejaculatory duct opening into an enlarged prostatic utricle was reached. Transurethral unroofing of the cyst was separately performed with a successful outcome. The characteristic of the 2 cystic dilatations was confirmed by pathologic examination. To the best of our knowledge, this is the first case of ectopic cystic dilatation of the ejaculatory duct opening into an enlarged prostatic utricle.

Urethro-ejaculatory duct reflux in children: an updated review.

AIM: Reflux of urine into the ejaculatory ducts of children may result in recurrent orchitis and sterility. Urethro-ejaculatory duct reflux (UER) is an uncommonly reported condition in children. The diagnosis of this condition can be made using a micturating cystourethrogram (MCUG) to demonstrate the reflux of contrast into any of the ejaculatory ducts. The aim of this study was to look at the incidence of UER in the broader pediatric population and review the management options. METHODS: A retrospective study was made of all MCUGs done in boys up to 13 years of age in all service departments caring for children in a teaching hospital. Those studies demonstrating UER were correlated with the hospital and departmental clinical notes. RESULTS: Over a 6 1/2 year period, 360 MCUGs were done in boys, and UER was demonstrated in 54 boys treated in seven departments. Twenty-three patients had recognized causes of UER. Eighteen patients were found to have other urological pathologies, and seven patients had neurological or complex VACTERL pathologies. The remaining six patients had no underlying pathology but presented with orchitis or torsion. Reflux into the seminal vesicles was the most frequent finding (74%), but reflux into the ductus deferens was the most debilitating. Orchitis was seen in a total of nine patients, and biopsies showed severe destruction of testicular tissue in these patients. Management of patients with recognized UER consisted of attempted correction of the underlying pathology and prolonged antibiotics. Where this failed in 6 patients, vasectomy was done in 4, and injection of a bulking gel into the verumontanum was done in 2 patients to prevent ongoing orchitis and pain. CONCLUSION: UER is more common then originally thought. All boys who present with uro-genital symptoms should have a MCUG as part of the investigative work-up and be scrutinized for UER. Management should aim at correcting any underlying anomaly and providing prolonged antibiotics, but vasectomy and the injection of a bulking agent should be considered. The indications for these newer forms of treatment are not clearly defined.

The ejaculatory duct ectopically invading the bladder with multiple congenital malformations of the homolateral urogenital system: a report of a rare case and an embryological review.

We report a rare case of a left ejaculatory duct that allotropically protrudes towards or invades the left vesicle triangular area with its dead end. The patient simultaneously exhibited multiple congenital malformations of the homolateral urogenital system, such as absence of the left kidney, dysplasia and allotopia of the left seminal vesicle, absence of the left ureterostoma, separation between the left testis and the epididymis tail, and maldevelopment of the left testis. According to all clinical and laboratory evidence, the case represented a new syndrome, which we named Wuyang's syndrome. It involved a rare phenomenon in embryonic development; the dysplastic proximal vas precursor, having intruded into a common mesonephric duct and accidentally encroaching on the ureteric bud position, resulted in the absence or dysplasia of the homolateral urinary tract and ectopic invasion of the bladder by the homolateral seminal tract.

Congenital anomalies of the mesonephric duct and fertility.

UNLABELLED: Agenesia of deferential duct and the others congenital malformations of mesonephronical duct are often encountering condition in andrological practice. This study presents the possibilities of reproductive medicine to restore the male fertility and concentrates on biological and clinical aspects of malformations of mesonephronic duct like factors for excretory male infertility. The investigations including 104 patients (mean age 30.25+/-1.91 years old) with congenital anomalies of mesonephronical duct with: Agenesia of mesonephronical derivates - case with unilateral agenesia of the deferent duct, kidney and left urether (n=1); Dysgenesia of the epididymis (n=5); Cysts of the epididymis (n=47); Agenesia of the deferential duct (n=48); Aplasia of the eja-culatorial duct (n=3). The following methods were used: open testicular biopsy by Vilar; objective findings in situ operation; bilateral epididymo - and deferentovesiculography; morphological specimens of the testicular and epididymal tissues; enzymohistochemical analysis of testicular specimens; the ejaculates and morphological analysis of spermatozoa were assessed for quality parameters by the standard protocol; enzymocytological research of spermatozoa in seminal fluid; biochemical analysis of seminal fluid fructose and citric acid concentrations; radioimmunoassay for measurements of blood FSH and testosterone concentration. RESULTS: Most often observed localization of the malformations of mesonephronic canal is bilateral at 54.81%. The testicular specimens proved preserved testicular architecture and spermatogenesis, and the epididymal slides showed dilatation of the epididymal tubules, which were overfilled with a lot of spermatozoa. The strongest expression of the enzymichistochemical NADH-TR reactions is in the Sertoli cells. We observed spermatozoa with disrup-ted configuration among which the abnormalities in sperm head and acrosome were predominated. The biochemical analysis of seminal fluid proves normal citric acid and low or lack of fructose concentration in seminal fluid. There were no breaches in endocrine regulation of the reproductive process. Patients with congenital anomalies of mesonephronic canal have preserving fertility in 31.73%. There are morpho-functional disorders of the testes, epididymis and seminal ducts in 68.27%. CONCLUSION: There are normal spermatogenesis but definite sterility. These results necessitate an application of plastic reconstructive operation for recovery of fertilizing ability of the patients, or their involvement in ICSI programme.

Clinical and radiological evaluation of ejaculatory duct obstruction.

Ejaculatory duct obstruction (EDO) is a rare but surgically correctable cause of male infertility. With the advent and increased use of transrectal ultrasonography and magnetic resonance imaging, abnormalities of the ejaculatory duct (ED) related to infertility have been diagnosed more frequently. Recently, with the increased awareness of functional obstruction of ED, reports have been focusing on the diagnosis of partial or functional EDO. We present 2 review of the ED pathologies, imaging modalities and treatment options.

Renal aplastic dysplasia and ipsilateral ectopic ureter obstructing the seminal via: a possible cause of male infertility.

Few cases of unilateral renal agenesis associated with ipsilateral seminal vesicle ectasia or cyst have been reported. Two cases of unilateral renal aplastic dysplasia and ipsilateral ectopic ureter opening in the ejaculatory ducts associated with infertility secondary to bilateral obstruction of the seminal via are reported. Clinical and physical assessment including transrectal ultrasound and magnetic resonance imaging are proposed as a comprehensive algorithm for the diagnostic evaluation of the pelvic cystic masses.

Rare case of ectopic duplicated megaureter with dysplastic upper kidney moiety opening into ipsilateral ejaculatory duct.

A rare case of left mesonephric duct malformation consisting of a duplicated ectopic megaureter opening into the ejaculatory duct and ipsilateral upper moiety cystic renal dysplasia is reported to increase awareness among urologists and radiologists of this entity. Magnetic resonance imaging has been shown to be an excellent diagnostic tool for tracking of the trajectory of the ectopic ureter, thereby obviating the need for other invasive diagnostic techniques and permitting surgical correction of the anomaly. The embryology, clinical features, and diagnostic and therapeutic aspects of this rare malformation are presented.

Müllerian duct remnant involving Wolffian system: a case report and literature review.

A 14-month-old boy with repeated left acute epididymitis was admitted to our department. Ultrasonography detected a midline round cystic mass in a retrovesical region. This was easily opacified by cystourethrography and seen to have a free communication with the posterior urethra. Since urethroscopy revealed a passable orifice in the center of the verumontanum, while a cystic-wall biopsy specimen showed squamous epithelium, we considered this cystic lesion to be an enlarged prostatic utricle. Vasography showed that the bilateral vasa was implanted directly into this cystic lesion, and was the possible cause of his left epididymitis. Ligature of the left vas deferens was performed to prevent left epididymitis. An enlarged prostatic utricle involving the vasa is a rare presentation.