Zinner syndrome: A unique triad of mesonephric duct abnormalities as an unusual cause of urinary symptoms in late adolescence

Ghonge, Nitin P; Aggarwal, Bharat B.; Sahu, Amit Kumar

doi:10.4103/0970-1591.70592

Cited by 43 publications

(43 citation statements)

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“…Dysuria, frequency of micturition, perineal pain, and painful ejaculation due to pressure of the seminal vesicle cysts are the presenting complaints [9]. Later oligozoospermia and even azoospermia develops, leading to infertility.…”

Section: Discussionmentioning

confidence: 99%

“…A convoluted tail-like connection of the cystic tubules to the seminal vesicle also pins the seminal vesicles as the site of origin. Cysts are rarely more than 5 cm in size, although giant cysts larger than 12 cm have been described and these can cause bowel and bladder obstruction [9]. MR is superior to CT as it demonstrates the anatomic relations in the pelvis better and can identify the ectopic ureteric orifices, which are missed on CT. Vasovesiculography is another confirmatory imaging technique where an iodinated contrast material is injected into the vas deferens ducts and serves to demonstrate seminal vesicle patency.…”

Section: Discussionmentioning

confidence: 99%

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Zinner syndrome—a rare developmental anomaly of the mesonephric duct diagnosed on magnetic resonance imaging

Mehra

Ranjan

Garga

2016

Radiology Case Reports

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Developmental anomalies of the urogenital tract are rare but often encountered. Zinner's syndrome is a rare congenital abnormality of mesonephric (Wolffian) duct consisting of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction due to developmental arrest in early embryogenesis affecting the caudal end of Mullerian duct and only approximately a 100 cases have been reported so far. Radiologic modalities such as intravenous pyelography, ultrasonography, vasovesiculography, contrast enhanced computed tomography, and magnetic resonance imaging are all helpful in diagnosis of this unusual entity. We present here an extremely rare developmental anomaly involving the Mullerian ducts, which would remain undiagnosed but for radiologic imaging. The patient presented with symptoms of lower urinary tract irritation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Zinner syndrome—a rare developmental anomaly of the mesonephric duct diagnosed on magnetic resonance imaging

Mehra

Ranjan

Garga

2016

Radiology Case Reports

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“…The uterus, however, may be single with two cervices or may be septated. Hence, the term OHVIRA – o bstructed h emi v agina, i psilateral r enal a genesis/ a nomaly (dysplasia, duplication or crossed fused ectopia) with a uterine anomaly better describes the clinical entity [6]. …”

Section: Discussionmentioning

confidence: 99%

Wolffian Origin of Vagina Unfolds the Embryopathogenesis of OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) Syndrome and Places OHVIRA as a Female Counterpart of Zinner Syndrome in Males

Aswani

Varma

Choudhary³

et al. 2016

Pol J Radiol

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SummaryBackgroundThe classical theory of Müllerian origin of upper vagina fails to explain complex urogenital malformations like OHVIRA syndrome; the Acien’s hypothesis, however, unravels the hidden embryopathogenesis. As per Acien, Wolffian (mesonephric) ducts instead of Müllerian ducts and sinovaginal bulbs, give rise to the vagina. The new hypothesis, however, retains the concept of origin of the ureters (with ureters inducing renal development) by the former and the uterus by Müllerian ducts. Thus, a failure of development of mesonephros/mesonephric duct gives rise to absent ureters and hence absent homolateral kidney; blind ending (obstructed) ipsilateral hemivagina and cessation of support to paramesonephric ducts which leads to unfused uterus (uterus didelphys). Hence, the new hypothesis explains all components of OHVIRA syndrome. On a parallel track, unilateral anomalous development of the mesonephros in males causes atresia of the homolateral ejaculatory duct that results in obstruction of the proximally placed seminal vesicle. Besides, there is absence of the ipsilateral kidney (Zinner syndrome).Case ReportIn this manuscript, we describe four cases of OHVIRA syndrome. Case 1 was a 34-year-old nulligravida, married since fourteen years, who presented with a 5-month history of pelvic inflammatory disease and dyspareunia. Regular menses in the patient and azoospermia in her husband delayed the diagnosis. Case 2 was a 14-year-old girl who presented with dysmenorrhea and lower abdominal pain since a few months. Case 3 was a 27-year-old female who presented with infertility and dysmenorrhea. Case 4 was a 15-year-old female who presented with a one-year history of dysmenorrhea and cyclic pelvic pain. In all cases, one of the uterine horns revealed collection due to a hemivaginal septum and an absent ipsilateral kidney; thus, establishing the diagnosis of OHVIRA syndrome. The case 4 additionally revealed homolateral vaginal agenesis.ConclusionsOn the basis of our 4 cases, we support the Acien’s hypothesis of Wolffian origin of vagina which explains the development of OHVIRA syndrome. Besides, we emphasize the need to suspect this syndrome in a female with a pelvic mass and absence of homolateral kidney. Finally, we believe that OHVIRA due to its Wolffian origin is a female equivalent of Zinner syndrome in males. Therefore, we propose OSVIRA (Obstructed Seminal Vesicle and Ipsilateral Renal Agenesis) as an acronym for Zinner syndrome analogous to OHVIRA.

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“…Anomalous development of the distal mesonephric duct results to atresia of the ejaculatory ducts and abnormal ureteral budding; the latter leads to renal agenesis or dysplasia, the former leads to obstruction and cystic dilatation of the seminal vesicles with development of seminal cysts. The development of seminal vesicle cysts is variable along the time life and this variability is not still well explained [3] . Actually seminal vesicle cysts could be congenital or acquired [4] .…”

Section: Discussionmentioning

confidence: 99%

Asymptomatic Zinner's Syndrome Diagnosed during the Sixth Decade of Life: Case Report and Review of the Literature

Cerruto¹,

Alberto²,

Tafuri³

et al. 2017

J Anesth Surg

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A 61 year-old male with a history of metabolic syndrome (hypertension, obesity, dyslipidemia), left kidney agenesia, compensatory hypertrophy and previous stone disease of the contralateral kidney, was sent by his general practitioner to our department because the incidental diagnosis of left seminal vesicle cyst lesion, not documented in the previous annual ultrasound control. He was father of 2 children and did not complain any lower urinary tract symptoms (LUTS) with a satisfied sexual life. He had a previous history negative for urinary tract infections (UTIs), dysuria, hematuria, ejaculatory pain, prostatitis, epididymitis. The examination revealed a patient in good general condition with body mass index of 29.07. The abdomen was soft with no palpable mass. Digital rectal examination was a normal prostate according with age. Laboratory tests including urine culture and PSA showed no abnormality. Transabdominal ultrasound scan (US) confirmed left kidney agenesia with compensatory hypertrophy and revealed for the first time (not doc- Copyrights 97 Case ReportOpen AccessUrology Clinic, Department of Surgery, Dentistry, Paediatrics and Gynaecology, University of Verona, Italy AbstractSeminal vesicle cysts combined with genitourinary anomalies, named since 1914 Zinner's syndrome, are rare. We present a case of asymptomatic 61-year-old married man, with known left kidney agenesia and a previous ultrasound history negative for vesicle cyst since one year before. Digital rectal examination was normal. Diagnostic imaging (both ultrasound and magnetic resonance imaging) showed left seminal vesicle cyst without any intravesical protrusion. In the absence of symptoms, a conservative treatment was decided, with a strict clinical and ultrasonographic follow-up. The diagnosis of Zinner's syndrome was made later, by ultrasound and magnetic resonance imaging, with the development of multilocular seminal vesicle cystic lesion, with a rapid onset (within one year). This malformation should be considered in the differential diagnosis of a pelvic cyst in male patients with renal agenesis. So far, the patient was asymptomatic and had never presented with dysuria, signs of bladder obstruction or urinary tract infection. He had neither any perineal discomfort nor pain; he described normal ejaculation without pain, no hematospermia, and had never experimented any urinary infection or prostatitis. He also had a normal sexual activity. In this particular case, in the absence of symptoms a conservative treatment was decided, with a strict clinical and transrectal US follow-up, at least until

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Zinner syndrome: A unique triad of mesonephric duct abnormalities as an unusual cause of urinary symptoms in late adolescence

Abstract: The present article reports a triad of right renal agenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction (Zinner syndrome) in a 19-year boy who presented with urinary symptoms. A detailed review of the relevant literature is also presented.

Cited by 43 publications

References 15 publications

Zinner syndrome—a rare developmental anomaly of the mesonephric duct diagnosed on magnetic resonance imaging

Zinner syndrome—a rare developmental anomaly of the mesonephric duct diagnosed on magnetic resonance imaging

Wolffian Origin of Vagina Unfolds the Embryopathogenesis of OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) Syndrome and Places OHVIRA as a Female Counterpart of Zinner Syndrome in Males

Asymptomatic Zinner's Syndrome Diagnosed during the Sixth Decade of Life: Case Report and Review of the Literature

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