Zinner syndrome is a Wolffian duct abnormality known with unilateral renal agenesis, ipsilateral seminal vesicle cyst and ejaculatory duct obstruction. HWWS is a rare mesonephric duct malformation with mullerian duct anomaly characterized by uterus didelphys, clogged blind hemi-vagina and ipsilateral renal agenesis (OHVIRA) syndrome. It was first reported by Wilson in 1925 (4).
Congenital anomalies of kidney and urinary tract are birth defects that affecting approximately 1% of live births (5). These anomalies include a wide range of malformations such as obstruction of ureteropelvic junction, kidney dysplasia, hydropic or ectopic and short ureters caused by deficiencies in embryonic kidney and lower urinary tract development. Although many gene sequences have been identified, the genetic causes of these malformations are still largely unknown (6). The morphogenesis of the urogenital system is affected by the nephric duct (ND), a common structure also known as the Wolffian duct (7). It is said by the authors that 1-2% of the cause of infertility in men has ND defects (8). Malformations that show a common etiology between the development of the urinary and genital system in the kidneys of patients with hymen impereforation of newborn girls are observed.
Zinner syndrome is based on the common origin of ureter buds and seminal vesicles from the Wolffian duct, associated with upper urinary tract abnormalities and seminal vesicle malformation (9). This syndrome occurs after an inadequate movement in the first trimester of embryogenesis. The ureteral bud originates from the dorsal aspect of the distal mesonephric canal and extends dorsocranially to meet and stimulate the transformation of the metanephric blastema to form the adult kidney. The mesonephric duct is divided into epididymis, paradidim, vas deferens, ejaculatory duct, seminal vesicle and hemitrigon under the influence of testosterone and anti-muller hormone (10). Alternatively, there will be abnormal ureteral budding, which leads to ipsilateral kidney agenesis or dysplasia and atresia of the ejaculatory duct and, consequently, to occlusion and cystic dilatation of the mesonephric duct (11).
HWWS is an anomaly it’s etiopathogenesis is not clearly explained and its true incidence is unknown. Development in embryological period is affected by genetic and environmental factors. HWWS also moves towards the paramesonephric system and methanephrosis (12).
The upper two-thirds of the uterus, fallopian tube, cervix, and vagina develop from paired paramesonephric ducts. Then, caudally, it laterally passes into the mesonephric duct, and finally, in the midline, it comes in close contact with the paramesonephric canal from the opposite side and merges to form the upper part of the uterus, cervix and vagina (13). If they do not fuse, two separate hemiuterins and hemicervices occur, resulting in mullerian anomalies associated with OHVIRA syndrome (14).
As in our cases; most patients are asymptomatic from two to four decades until high sexual and reproductive activity periods (15). The clinical picture is related to the size of the seminal vesicle cysts. Cysts smaller than 5 cm are usually diagnosed on abdominal or digital rectal examination. Symptoms occur after progressive dilatation of the seminal vesicles due to the accumulation of secretions after inadequate drainage secondary to the ejaculatory canal atresia (16). In our cases, complicated appearance was observed with hemorrhage due to these drainage disorders. Seminal vesicles are localized in the posterior of the bladder and therefore enlarged cysts may cause different symptoms due to bladder irritation, such as dysuria, recurrent urinary tract infections, infertility painful ejaculation epididymitis and prostatitis (17-18). Larger cysts (> 12cm) can cause bladder outlet obstruction, colon obstruction, or perianal pain.
Various imaging techniques can be used in the diagnostic study of these congenital malformations. These can be listed as intravenous urography, transrectal, ultrasonography, CT examination, cystoscopy and MRI. Intravenous urography can be used to assess collecting duct system abnormalities or absence and therefore may indicate kidney agenesis. Transrectal USG can be used to determine the size and location of the associated cystic mass and its association with the seminal vesicle or prostate. It may include symptoms suggestive of bleeding or infection but is limited to small field of vision. Pelvic USG can help in the differential diagnosis, to examine whether the kidneys are in normal location, and to accurately identify the relationship of the cystic mass in the pelvic area with adjacent structures.
CT examination can accurately demonstrate pelvic anatomy and atypical kidney structures, the presence of kidney or its association with associated pathology. MR imaging may be preferred due to its relatively better contrast resolution, seminal vesicles, pelvic structures, and collector system imaging. In both of our cases, MRI was performed following CT examination.
Treatment of Zinner syndrome; Surgical resection can be performed for symptomatic seminal vesicle cysts. Also, there is infertility and there is a desire for fertility, testicular sperm extraction can be performed. There are other methods such as transrectal or transurethral interventional procedures or transurethral resection of the seminal coliculus and vas deferens. In HWWS, a therapeutic laparoscopic evaluation or vaginal septum excision can be performed to remove obstruction.
As a result; Zinner syndrome and HWWS are congenital genitourinary anomalies accompanied by ipsilateral renal agenesis. The diagnosis of these patients, which can cause symptoms such as infertility, pelvic pain, and bladder irritation, can be selected as digital rectal examination, transrectal and abdominal USG, CT examination, and the more ideal imaging method MRI. Clinical suspicion and early diagnosis can contribute to directing treatment.
DISCUSSION
Zinner syndrome is a Wolffian duct abnormality known with unilateral renal agenesis, ipsilateral seminal vesicle cyst and ejaculatory duct obstruction. HWWS is a rare mesonephric duct malformation with mullerian duct anomaly characterized by uterus didelphys, clogged blind hemi-vagina and ipsilateral renal agenesis (OHVIRA) syndrome. It was first reported by Wilson in 1925 (4).
Congenital anomalies of kidney and urinary tract are birth defects that affecting approximately 1% of live births (5). These anomalies include a wide range of malformations such as obstruction of ureteropelvic junction, kidney dysplasia, hydropic or ectopic and short ureters caused by deficiencies in embryonic kidney and lower urinary tract development. Although many gene sequences have been identified, the genetic causes of these malformations are still largely unknown (6). The morphogenesis of the urogenital system is affected by the nephric duct (ND), a common structure also known as the Wolffian duct (7). It is said by the authors that 1-2% of the cause of infertility in men has ND defects (8). Malformations that show a common etiology between the development of the urinary and genital system in the kidneys of patients with hymen impereforation of newborn girls are observed.
Zinner syndrome is based on the common origin of ureter buds and seminal vesicles from the Wolffian duct, associated with upper urinary tract abnormalities and seminal vesicle malformation (9). This syndrome occurs after an inadequate movement in the first trimester of embryogenesis. The ureteral bud originates from the dorsal aspect of the distal mesonephric canal and extends dorsocranially to meet and stimulate the transformation of the metanephric blastema to form the adult kidney. The mesonephric duct is divided into epididymis, paradidim, vas deferens, ejaculatory duct, seminal vesicle and hemitrigon under the influence of testosterone and anti-muller hormone (10). Alternatively, there will be abnormal ureteral budding, which leads to ipsilateral kidney agenesis or dysplasia and atresia of the ejaculatory duct and, consequently, to occlusion and cystic dilatation of the mesonephric duct (11).
HWWS is an anomaly it’s etiopathogenesis is not clearly explained and its true incidence is unknown. Development in embryological period is affected by genetic and environmental factors. HWWS also moves towards the paramesonephric system and methanephrosis (12).
The upper two-thirds of the uterus, fallopian tube, cervix, and vagina develop from paired paramesonephric ducts. Then, caudally, it laterally passes into the mesonephric duct, and finally, in the midline, it comes in close contact with the paramesonephric canal from the opposite side and merges to form the upper part of the uterus, cervix and vagina (13). If they do not fuse, two separate hemiuterins and hemicervices occur, resulting in mullerian anomalies associated with OHVIRA syndrome (14).
As in our cases; most patients are asymptomatic from two to four decades until high sexual and reproductive activity periods (15). The clinical picture is related to the size of the seminal vesicle cysts. Cysts smaller than 5 cm are usually diagnosed on abdominal or digital rectal examination. Symptoms occur after progressive dilatation of the seminal vesicles due to the accumulation of secretions after inadequate drainage secondary to the ejaculatory canal atresia (16). In our cases, complicated appearance was observed with hemorrhage due to these drainage disorders. Seminal vesicles are localized in the posterior of the bladder and therefore enlarged cysts may cause different symptoms due to bladder irritation, such as dysuria, recurrent urinary tract infections, infertility painful ejaculation epididymitis and prostatitis (17-18). Larger cysts (> 12cm) can cause bladder outlet obstruction, colon obstruction, or perianal pain.
Various imaging techniques can be used in the diagnostic study of these congenital malformations. These can be listed as intravenous urography, transrectal, ultrasonography, CT examination, cystoscopy and MRI. Intravenous urography can be used to assess collecting duct system abnormalities or absence and therefore may indicate kidney agenesis. Transrectal USG can be used to determine the size and location of the associated cystic mass and its association with the seminal vesicle or prostate. It may include symptoms suggestive of bleeding or infection but is limited to small field of vision. Pelvic USG can help in the differential diagnosis, to examine whether the kidneys are in normal location, and to accurately identify the relationship of the cystic mass in the pelvic area with adjacent structures.
CT examination can accurately demonstrate pelvic anatomy and atypical kidney structures, the presence of kidney or its association with associated pathology. MR imaging may be preferred due to its relatively better contrast resolution, seminal vesicles, pelvic structures, and collector system imaging. In both of our cases, MRI was performed following CT examination.
Treatment of Zinner syndrome; Surgical resection can be performed for symptomatic seminal vesicle cysts. Also, there is infertility and there is a desire for fertility, testicular sperm extraction can be performed. There are other methods such as transrectal or transurethral interventional procedures or transurethral resection of the seminal coliculus and vas deferens. In HWWS, a therapeutic laparoscopic evaluation or vaginal septum excision can be performed to remove obstruction.
As a result; Zinner syndrome and HWWS are congenital genitourinary anomalies accompanied by ipsilateral renal agenesis. The diagnosis of these patients, which can cause symptoms such as infertility, pelvic pain, and bladder irritation, can be selected as digital rectal examination, transrectal and abdominal USG, CT examination, and the more ideal imaging method MRI. Clinical suspicion and early diagnosis can contribute to directing treatment.