Rare disease
CASE REPORT
Zinner syndrome: an uncommon cause of painful ejaculation Raghav Sundar,1 Gaurav Sundar2 1
National University Health System, Singapore, Singapore 2 Department of Radiology, Division of Vascular and Interventional Radiology, Christian Medical College, Vellore, India Correspondence to Dr Raghav Sundar, [email protected] Accepted 13 February 2015
SUMMARY Zinner syndrome refers to the triad of ipsilateral renal agenesis, seminal vesicle cysts and ejaculatory duct obstruction. Ipsilateral renal agenesis may be associated with seminal vesicle cysts in 70% of cases, but a remnant ureteral bud has been shown to coexist in only 27% of these cases. While some patients may remain asymptomatic and are discovered incidentally, others present with symptoms related to seminal vesicle cysts or ejaculatory duct obstruction: voiding or ejaculatory difficulty or pain. The diagnosis is made with imaging findings, and differentiation from other pelvic cysts requires a multimodality approach. In this report, we present typical imaging findings of a patient who presented with painful ejaculation where there was a congenital seminal vesicle cyst with ipsilateral renal agenesis associated with a remnant ureteral bud draining into the seminal vesicle cyst and also associated with a cyst of the prostatic utricle. We discuss the relevant embryological basis for this unusual combination of findings. BACKGROUND Zinner syndrome refers to the triad of ipsilateral renal agenesis, seminal vesicle cysts and ejaculatory duct obstruction. The common embryological origin of the mesonephric duct and the ureteral bud lead to associated anomalous development of the genital and urinary tracts. While some patients may remain asymptomatic and are discovered incidentally, others may present with symptoms related to seminal vesicle cysts, voiding or ejaculatory difficulty or pain or prostatitis. Differentiation from other pelvic cystic masses may be achieved with imaging. We present typical imaging findings in a case of Zinner syndrome where the patient presented with painful ejaculation.
terminating in the pelvis (figure 1). Based on the morphology and location, this was considered to be a remnant ureter but it was not clear if it was communicating with the bladder. On contrast administration, the right kidney showed normal enhancement and excretion but the remnant ureter did not show change in attenuation value. The left seminal vesicle appeared to be enlarged, while the right seminal vesicle was normal. To further characterise the remnant ureter and seminal vesicle, ultrasonography was performed. Transrectal ultrasonography showed normal right seminal vesicle with numerous cysts within the left seminal vesicle (figure 2). The remnant ureter was noted to communicate with one of the seminal vesicle cysts (figure 3). A midline cyst was seen within the prostate and was considered to be either a cyst of the prostatic urethra or cyst of the prostatic utricle (figure 4). To further characterise and study the relationship between the remnant ureter, seminal vesicle cysts and midline cyst within the prostate, MRI of the abdomen was performed. On MRI, the left kidney was not visualised (figure 5). Multiple cysts were seen in the left seminal vesicle, which followed signal intensity of simple fluid, being hypointense on T1-weighted sequences and hyperintense on T2-weighted sequences. The remnant ureter was noted to communicate with one of these cysts, close to the midline (figures 6 and 7). The midline prostatic cyst was hypointense on T1-weighted sequences and hyperintense on T2-weighted sequences, and did not extend above the level of the prostate, consistent with cyst of the prostatic utricle (figure 8). A retrograde urethrogram showed the entire urethra to be normal with no communication with the seminal vesicle or prostatic utricle cyst (figure 9).
CASE PRESENTATION A 25-year-old man presented with a 5-year history of urinary frequency, intermittent perineal pain and pain during ejaculation. He claimed not to be sexually active and was otherwise healthy. Routine laboratory studies of blood and urine were normal. Clinical examination was normal and he was referred for CT of the abdomen for suspected urinary calculus.
INVESTIGATIONS To cite: Sundar R, Sundar G. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014207618
CT of the abdomen revealed the right kidney to be normal with no calculus and a normal collecting system. The left kidney was not visualised in the abdomen. A fluid-attenuation tubular non-enhancing structure was noted in the region of the left ureter extending from the level of L2 vertebra and
Figure 1 Axial excretory phase CT showing normal right ureter excreting contrast (blue arrow head) and non-excreting remnant left ureter (yellow arrow head).
Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
1
Rare disease TREATMENT Laparoscopic surgical excision of the cyst of the left seminal vesicle was performed. For the procedure, the patient was placed in the Trendelenburg position and a transperitoneal approach with five ports was used. After insufflation of the peritoneum, the rectovesical peritoneum was transversely incised and the left seminal vesicle and the ampulla of the vas deferens were identified and carefully dissected sharply with minimal use of bipolar coagulation. The seminal vesicle cyst was incised, and its orifice over the ampulla was closed with absorbable sutures.
OUTCOME AND FOLLOW-UP Patient was asymptomatic at 1 year of clinical follow-up.
DISCUSSION
Figure 2 Parasagittal TRUS showing multiple cysts within the left seminal vesicle (red arrow) and normal right seminal vesicle (blue arrow). Based on the above findings of left renal agenesis, cyst of the prostatic utricle and remnant ureter draining into ipsilateral seminal vesicle cysts, a diagnosis of Zinner syndrome was made.
DIFFERENTIAL DIAGNOSIS The differential diagnosis of cystic pelvic masses in the male includes cystic dilation of the prostatic utricle, cyst of the prostatic utricle ( previously known as Müllerian duct cysts) and ejaculatory duct cysts, all of which are midline in location (figure 10). Other possibilities include prostatic cysts, diverticulosis of the ampulla of the vas deferens, ectopic ureterocele and abscess.
Figure 3 Parasagittal TRUS showing the remnant left ureter (green arrow) to communicate with the seminal vesicle cyst. 2
Zinner syndrome refers to the triad of ipsilateral renal agenesis, seminal vesicle cysts and ejaculatory duct obstruction.1 The common embryological origin of the mesonephric duct and the ureteral bud leads to associated anomalous development of the genital and urinary tracts, including obstruction of the ejaculatory duct, seminal vesicle cysts and ipsilateral renal agenesis. The ureteral bud arises from the dorsal aspect of the distal mesonephric duct and extends in a cranial direction to meet the metanephric blastema, which will form the adult kidney. Normal development of the kidney requires the ureteral bud to meet the metanephric blastema and induce its differentiation. Abnormal development can result from faults at various stages of embryogenesis. An insult during the first trimester adversely affects the embryogenesis of kidney, ureter, seminal vesicle and vas deferens.2 Maldevelopment of the distal part of mesonephric duct leads to atresia of the ejaculatory duct (leading to the obstruction and cystic dilation of seminal vesicle) and abnormal ureteral budding (leading to renal agenesis or dysplasia). The obstruction at the level of ejaculatory duct can also be due to cysts in the prostatic utricle, as in our case, and can lead to gradual accumulation of secretions in the seminal vesicle with consequent cyst formation. Patients with Zinner syndrome can present with a variety of symptoms, many of which are non-specific, and the diagnosis is often made only on imaging studies. Small cysts in the seminal vesicle, less than 5 cm in size, are usually asymptomatic and are often detected incidentally. Larger cysts, including giant cysts measuring more than 12 cm, may present with symptoms related to bladder compression and obstruction.3 4 Commonly reported symptoms include abdominal, perineal and pelvic
Figure 4 Axial TRUS showing a midline cystic lesion within the prostate (green arrow). The remnant ureter is noted to communicate with the seminal vesicle cyst (orange star). Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
Rare disease
Figure 7 Parasagittal T2-weighted SE MRI showing the tubular remnant ureter (red arrow) communicating with one of the seminal vesicle cysts near the midline (blue arrow).
Figure 5 Coronal T1-weighted balanced steady-state free precession MRI showing normal right kidney and agenesis of left kidney.
pain; ejaculatory pain; dysuria; urinary frequency; haematuria; urinary tract infections; and symptoms of epididymitis and prostatitis.3–9 Our patient presented with urinary frequency, intermittent perineal pain and painful ejaculation, possibly due to
Figure 6 Consecutive axial T2-weighted SE MRI showing hyperintense left remnant ureter (green arrow head), which is draining into an ipsilateral seminal vesicle cyst. Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
the compressive effect of the seminal vesicle cyst and cyst of the prostatic utricle on the bladder base, urethra and ejaculatory duct. In the evaluation of cystic lesions in the male pelvis, there is a complementary role of several imaging modalities. Ultrasonography can confirm the cystic nature of the pelvic mass, and evaluate the number, size, location and relationship to the prostate and seminal vesicles.10–12 The cysts may have clear anechoic contents or echogenic fluid-debris levels, indicating haemorrhage or infection.10 12 The wall may be thin, thick or show calcification. Communication between seminal vesicle cysts and urinary tract may also be seen, as was demonstrated in our case.13 Retrograde urethrogram can be used to confirm or refute communication of cysts within the prostate with the urethra. CT can better demonstrate the associated renal anomalies, confirm agenesis or dysplasia of the kidney and define the anatomy of pelvic structures. However, accurate depiction of the relationship of the ureteral remnant with the seminal vesicle, prostate and bladder can be difficult. MR is the modality of choice in imaging masses in the pelvis due to its multiplanar capability to define anatomy, and its ability to characterise cystic and solid lesions in the pelvis. It has proven to be accurate in differentiating seminal vesicle cysts from other pelvic cystic malformations.14 Seminal vesicle cysts are usually similar to cystic lesions elsewhere in the body, having a high signal intensity on T2-weighted sequence and low signal intensity on T1-weighted sequence. Occasionally, they may have high signal intensity on T1-weighted and T2-weighted
Figure 8 Axial T2-weighted SE MRI showing cyst of the prostatic utricle as a hyperintense midline limited to the prostate cyst ( pink arrow head). 3
Rare disease Learning points
Figure 9 Oblique radiograph from retrograde urethrogram showing the entire urethra to be normal, with no communication with the seminal vesicle cyst. sequences due to the presence of haemorrhage or proteinaceous content.13 In the differentiation of cystic lesions in the prostate, it is important to differentiate cysts of the prostatic utricle from cysts of the prostatic urethra, as both appear as midline cystic lesions within the prostate.15 While both are midline in location, cysts of the prostatic urethra can grow to large sizes, even above the level of the prostate, and show haemorrhage (high signal intensity on T1-weighted sequence); cysts of the prostatic utricle are confined to the prostate and have signal intensity similar to water.16 Most cases of Zinner syndrome are managed conservatively without need for surgery. Surgical excision of a seminal vesicle cyst depends on the size and location of the cyst, and presence of clinical symptoms. Nowadays, laparoscopic and robotic techniques have replaced the open surgical approach.17 18 Removal of the seminal vesicle cysts can resolve symptoms while preserving fertility and erectile function.19 As the condition is benign, periodic clinical follow-up is sufficient and imaging is reserved for those patients who become symptomatic. Symptomatic patients may be imaged optimally with MRI to assess for any recurrence of seminal vesicle cysts or surgery-related complications.
▸ Zinner syndrome refers to a triad of ipsilateral renal agenesis, seminal vesicle cysts and ejaculatory duct obstruction. ▸ Most patients are asymptomatic and the diagnosis is made incidentally, while some symptomatic patients may present with urinary symptoms and ejaculatory difficulty or pain. ▸ The complementary role of various imaging modalities is useful to confirm the diagnosis. ▸ The treatment depends on patient symptoms. While asymptomatic patients may be followed up with conservative management, surgical excision of seminal vesicle cysts may be needed for large cysts causing obstructive symptoms.
Contributors GS helped with the images and radiological description of case. RS wrote up the case. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2
3 4 5 6 7 8
9
10
11 12 13 14
15 16 17
18
Figure 10 Diagrammatic representation of various cystic lesions in the male pelvis. 4
19
Zinner A. Ein fall von intravesikaler Samenblasenzyste. Wien Med Wochenschr 1914;64:605. McCallum T, Milunsky J, Munarriz R, et al. Unilateral renal agenesis associated with congenital bilateral absence of the vas deferens: phenotypic findings and genetic considerations. Hum Reprod 2001;16:282–8. Heaney JA, Pfister RC, Meares EM Jr. Giant cyst of the seminal vesicle with renal agenesis. AJR Am J Roentgenol 1987;149:139–40. Kenney PJ, Leeson MD. Congenital anomalies of the seminal vesicles: spectrum of computed tomographic findings. Radiology 1983;149:247–51. Rappe BJ, Meuleman EJ, Debruyne FM. Seminal vesicle cyst with ipsilateral renal agenesis. Urol Int 1993;50:54–6. Beeby DI. Seminal vesicle cyst associated with ipsilateral renal agenesis: case report and review of literature. J Urol 1974;112:120–2. Levisay GL, Holder J, Weigel JW. Ureteral ectopia associated with seminal vesicle cyst and ipsilateral renal agenesis. Radiology 1975;114:575–6. Okada Y, Tanaka H, Takeuchi H, et al. Papillary adenocarcinoma in a seminal vesicle cyst associated with ipsilateral renal agenesis: a case report. J Urol 1992;148:1543–5. Kaneti J, Lissmer L, Smailowitz Z, et al. Agenesis of kidney associated with malformation of the seminal vesicle: various clinical presentations. Int Urol Nephrol 1988;20:29–33. Trigaux JP, Van Beers B, Delchambre F. Male genital tract malformations associated with ipsilateral renal agenesis: sonographic findings. J Clin Ultrasound 1991;19:3–10. Walls WJ, Lin F. Ultrasonic diagnosis of seminal vesicle cysts. Radiology 1975;114:693–4. King BF, Hattery RR, Lieber MM, et al. Congenital cystic disease of the seminal vesicle. Radiology 1991;178:207–11. Narlawar RS, Hanchate V, Raut A, et al. Renal agenesis and seminal vesicle cyst. J Ultrasound Med 2003;22:225–8. Murphy JO, Power RE, Akhtar M, et al. Magnetic resonance imaging in the diagnosis of seminal vesicle cysts and associated anomalies. J Urol 2003;170:2386. Kuligowska E, Fenlon HM. Transrectal US in male infertility: spectrum of findings and role in patient care. Radiology 1998;207:173–81. Ammar T, Sidhu PS, Wilkins CJ. Male infertility: the role of imaging in diagnosis and management. Br J Radiol 2012;85:S59–68. Altobelli E, Bove AM, Falavolti C, et al. Robotic-assisted laparoscopic approach in the treatment for Zinner’s syndrome associated with ipsilateral megaureter and incomplete double-crossed ectopic ureter. Int Urol Nephrol 2013;45:635–8. Jarzemski P, Lisopadzki S, Kowalski M. Laparoscopic removal of a congenital seminal vesicle cyst in Zinner’s syndrome. JSLS 2014;18:367–71. Benyó M, Berczi C, Jozsa T, et al. Fertility preservation in cases of laparoscopic treatment of seminal vesicle cysts. Cent European J Urol 2012;65:144–5.
Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
Rare disease Copyright 2015 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
5
CASE REPORT
Zinner syndrome: an uncommon cause of painful ejaculation Raghav Sundar,1 Gaurav Sundar2 1
National University Health System, Singapore, Singapore 2 Department of Radiology, Division of Vascular and Interventional Radiology, Christian Medical College, Vellore, India Correspondence to Dr Raghav Sundar, [email protected] Accepted 13 February 2015
SUMMARY Zinner syndrome refers to the triad of ipsilateral renal agenesis, seminal vesicle cysts and ejaculatory duct obstruction. Ipsilateral renal agenesis may be associated with seminal vesicle cysts in 70% of cases, but a remnant ureteral bud has been shown to coexist in only 27% of these cases. While some patients may remain asymptomatic and are discovered incidentally, others present with symptoms related to seminal vesicle cysts or ejaculatory duct obstruction: voiding or ejaculatory difficulty or pain. The diagnosis is made with imaging findings, and differentiation from other pelvic cysts requires a multimodality approach. In this report, we present typical imaging findings of a patient who presented with painful ejaculation where there was a congenital seminal vesicle cyst with ipsilateral renal agenesis associated with a remnant ureteral bud draining into the seminal vesicle cyst and also associated with a cyst of the prostatic utricle. We discuss the relevant embryological basis for this unusual combination of findings. BACKGROUND Zinner syndrome refers to the triad of ipsilateral renal agenesis, seminal vesicle cysts and ejaculatory duct obstruction. The common embryological origin of the mesonephric duct and the ureteral bud lead to associated anomalous development of the genital and urinary tracts. While some patients may remain asymptomatic and are discovered incidentally, others may present with symptoms related to seminal vesicle cysts, voiding or ejaculatory difficulty or pain or prostatitis. Differentiation from other pelvic cystic masses may be achieved with imaging. We present typical imaging findings in a case of Zinner syndrome where the patient presented with painful ejaculation.
terminating in the pelvis (figure 1). Based on the morphology and location, this was considered to be a remnant ureter but it was not clear if it was communicating with the bladder. On contrast administration, the right kidney showed normal enhancement and excretion but the remnant ureter did not show change in attenuation value. The left seminal vesicle appeared to be enlarged, while the right seminal vesicle was normal. To further characterise the remnant ureter and seminal vesicle, ultrasonography was performed. Transrectal ultrasonography showed normal right seminal vesicle with numerous cysts within the left seminal vesicle (figure 2). The remnant ureter was noted to communicate with one of the seminal vesicle cysts (figure 3). A midline cyst was seen within the prostate and was considered to be either a cyst of the prostatic urethra or cyst of the prostatic utricle (figure 4). To further characterise and study the relationship between the remnant ureter, seminal vesicle cysts and midline cyst within the prostate, MRI of the abdomen was performed. On MRI, the left kidney was not visualised (figure 5). Multiple cysts were seen in the left seminal vesicle, which followed signal intensity of simple fluid, being hypointense on T1-weighted sequences and hyperintense on T2-weighted sequences. The remnant ureter was noted to communicate with one of these cysts, close to the midline (figures 6 and 7). The midline prostatic cyst was hypointense on T1-weighted sequences and hyperintense on T2-weighted sequences, and did not extend above the level of the prostate, consistent with cyst of the prostatic utricle (figure 8). A retrograde urethrogram showed the entire urethra to be normal with no communication with the seminal vesicle or prostatic utricle cyst (figure 9).
CASE PRESENTATION A 25-year-old man presented with a 5-year history of urinary frequency, intermittent perineal pain and pain during ejaculation. He claimed not to be sexually active and was otherwise healthy. Routine laboratory studies of blood and urine were normal. Clinical examination was normal and he was referred for CT of the abdomen for suspected urinary calculus.
INVESTIGATIONS To cite: Sundar R, Sundar G. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014207618
CT of the abdomen revealed the right kidney to be normal with no calculus and a normal collecting system. The left kidney was not visualised in the abdomen. A fluid-attenuation tubular non-enhancing structure was noted in the region of the left ureter extending from the level of L2 vertebra and
Figure 1 Axial excretory phase CT showing normal right ureter excreting contrast (blue arrow head) and non-excreting remnant left ureter (yellow arrow head).
Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
1
Rare disease TREATMENT Laparoscopic surgical excision of the cyst of the left seminal vesicle was performed. For the procedure, the patient was placed in the Trendelenburg position and a transperitoneal approach with five ports was used. After insufflation of the peritoneum, the rectovesical peritoneum was transversely incised and the left seminal vesicle and the ampulla of the vas deferens were identified and carefully dissected sharply with minimal use of bipolar coagulation. The seminal vesicle cyst was incised, and its orifice over the ampulla was closed with absorbable sutures.
OUTCOME AND FOLLOW-UP Patient was asymptomatic at 1 year of clinical follow-up.
DISCUSSION
Figure 2 Parasagittal TRUS showing multiple cysts within the left seminal vesicle (red arrow) and normal right seminal vesicle (blue arrow). Based on the above findings of left renal agenesis, cyst of the prostatic utricle and remnant ureter draining into ipsilateral seminal vesicle cysts, a diagnosis of Zinner syndrome was made.
DIFFERENTIAL DIAGNOSIS The differential diagnosis of cystic pelvic masses in the male includes cystic dilation of the prostatic utricle, cyst of the prostatic utricle ( previously known as Müllerian duct cysts) and ejaculatory duct cysts, all of which are midline in location (figure 10). Other possibilities include prostatic cysts, diverticulosis of the ampulla of the vas deferens, ectopic ureterocele and abscess.
Figure 3 Parasagittal TRUS showing the remnant left ureter (green arrow) to communicate with the seminal vesicle cyst. 2
Zinner syndrome refers to the triad of ipsilateral renal agenesis, seminal vesicle cysts and ejaculatory duct obstruction.1 The common embryological origin of the mesonephric duct and the ureteral bud leads to associated anomalous development of the genital and urinary tracts, including obstruction of the ejaculatory duct, seminal vesicle cysts and ipsilateral renal agenesis. The ureteral bud arises from the dorsal aspect of the distal mesonephric duct and extends in a cranial direction to meet the metanephric blastema, which will form the adult kidney. Normal development of the kidney requires the ureteral bud to meet the metanephric blastema and induce its differentiation. Abnormal development can result from faults at various stages of embryogenesis. An insult during the first trimester adversely affects the embryogenesis of kidney, ureter, seminal vesicle and vas deferens.2 Maldevelopment of the distal part of mesonephric duct leads to atresia of the ejaculatory duct (leading to the obstruction and cystic dilation of seminal vesicle) and abnormal ureteral budding (leading to renal agenesis or dysplasia). The obstruction at the level of ejaculatory duct can also be due to cysts in the prostatic utricle, as in our case, and can lead to gradual accumulation of secretions in the seminal vesicle with consequent cyst formation. Patients with Zinner syndrome can present with a variety of symptoms, many of which are non-specific, and the diagnosis is often made only on imaging studies. Small cysts in the seminal vesicle, less than 5 cm in size, are usually asymptomatic and are often detected incidentally. Larger cysts, including giant cysts measuring more than 12 cm, may present with symptoms related to bladder compression and obstruction.3 4 Commonly reported symptoms include abdominal, perineal and pelvic
Figure 4 Axial TRUS showing a midline cystic lesion within the prostate (green arrow). The remnant ureter is noted to communicate with the seminal vesicle cyst (orange star). Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
Rare disease
Figure 7 Parasagittal T2-weighted SE MRI showing the tubular remnant ureter (red arrow) communicating with one of the seminal vesicle cysts near the midline (blue arrow).
Figure 5 Coronal T1-weighted balanced steady-state free precession MRI showing normal right kidney and agenesis of left kidney.
pain; ejaculatory pain; dysuria; urinary frequency; haematuria; urinary tract infections; and symptoms of epididymitis and prostatitis.3–9 Our patient presented with urinary frequency, intermittent perineal pain and painful ejaculation, possibly due to
Figure 6 Consecutive axial T2-weighted SE MRI showing hyperintense left remnant ureter (green arrow head), which is draining into an ipsilateral seminal vesicle cyst. Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
the compressive effect of the seminal vesicle cyst and cyst of the prostatic utricle on the bladder base, urethra and ejaculatory duct. In the evaluation of cystic lesions in the male pelvis, there is a complementary role of several imaging modalities. Ultrasonography can confirm the cystic nature of the pelvic mass, and evaluate the number, size, location and relationship to the prostate and seminal vesicles.10–12 The cysts may have clear anechoic contents or echogenic fluid-debris levels, indicating haemorrhage or infection.10 12 The wall may be thin, thick or show calcification. Communication between seminal vesicle cysts and urinary tract may also be seen, as was demonstrated in our case.13 Retrograde urethrogram can be used to confirm or refute communication of cysts within the prostate with the urethra. CT can better demonstrate the associated renal anomalies, confirm agenesis or dysplasia of the kidney and define the anatomy of pelvic structures. However, accurate depiction of the relationship of the ureteral remnant with the seminal vesicle, prostate and bladder can be difficult. MR is the modality of choice in imaging masses in the pelvis due to its multiplanar capability to define anatomy, and its ability to characterise cystic and solid lesions in the pelvis. It has proven to be accurate in differentiating seminal vesicle cysts from other pelvic cystic malformations.14 Seminal vesicle cysts are usually similar to cystic lesions elsewhere in the body, having a high signal intensity on T2-weighted sequence and low signal intensity on T1-weighted sequence. Occasionally, they may have high signal intensity on T1-weighted and T2-weighted
Figure 8 Axial T2-weighted SE MRI showing cyst of the prostatic utricle as a hyperintense midline limited to the prostate cyst ( pink arrow head). 3
Rare disease Learning points
Figure 9 Oblique radiograph from retrograde urethrogram showing the entire urethra to be normal, with no communication with the seminal vesicle cyst. sequences due to the presence of haemorrhage or proteinaceous content.13 In the differentiation of cystic lesions in the prostate, it is important to differentiate cysts of the prostatic utricle from cysts of the prostatic urethra, as both appear as midline cystic lesions within the prostate.15 While both are midline in location, cysts of the prostatic urethra can grow to large sizes, even above the level of the prostate, and show haemorrhage (high signal intensity on T1-weighted sequence); cysts of the prostatic utricle are confined to the prostate and have signal intensity similar to water.16 Most cases of Zinner syndrome are managed conservatively without need for surgery. Surgical excision of a seminal vesicle cyst depends on the size and location of the cyst, and presence of clinical symptoms. Nowadays, laparoscopic and robotic techniques have replaced the open surgical approach.17 18 Removal of the seminal vesicle cysts can resolve symptoms while preserving fertility and erectile function.19 As the condition is benign, periodic clinical follow-up is sufficient and imaging is reserved for those patients who become symptomatic. Symptomatic patients may be imaged optimally with MRI to assess for any recurrence of seminal vesicle cysts or surgery-related complications.
▸ Zinner syndrome refers to a triad of ipsilateral renal agenesis, seminal vesicle cysts and ejaculatory duct obstruction. ▸ Most patients are asymptomatic and the diagnosis is made incidentally, while some symptomatic patients may present with urinary symptoms and ejaculatory difficulty or pain. ▸ The complementary role of various imaging modalities is useful to confirm the diagnosis. ▸ The treatment depends on patient symptoms. While asymptomatic patients may be followed up with conservative management, surgical excision of seminal vesicle cysts may be needed for large cysts causing obstructive symptoms.
Contributors GS helped with the images and radiological description of case. RS wrote up the case. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2
3 4 5 6 7 8
9
10
11 12 13 14
15 16 17
18
Figure 10 Diagrammatic representation of various cystic lesions in the male pelvis. 4
19
Zinner A. Ein fall von intravesikaler Samenblasenzyste. Wien Med Wochenschr 1914;64:605. McCallum T, Milunsky J, Munarriz R, et al. Unilateral renal agenesis associated with congenital bilateral absence of the vas deferens: phenotypic findings and genetic considerations. Hum Reprod 2001;16:282–8. Heaney JA, Pfister RC, Meares EM Jr. Giant cyst of the seminal vesicle with renal agenesis. AJR Am J Roentgenol 1987;149:139–40. Kenney PJ, Leeson MD. Congenital anomalies of the seminal vesicles: spectrum of computed tomographic findings. Radiology 1983;149:247–51. Rappe BJ, Meuleman EJ, Debruyne FM. Seminal vesicle cyst with ipsilateral renal agenesis. Urol Int 1993;50:54–6. Beeby DI. Seminal vesicle cyst associated with ipsilateral renal agenesis: case report and review of literature. J Urol 1974;112:120–2. Levisay GL, Holder J, Weigel JW. Ureteral ectopia associated with seminal vesicle cyst and ipsilateral renal agenesis. Radiology 1975;114:575–6. Okada Y, Tanaka H, Takeuchi H, et al. Papillary adenocarcinoma in a seminal vesicle cyst associated with ipsilateral renal agenesis: a case report. J Urol 1992;148:1543–5. Kaneti J, Lissmer L, Smailowitz Z, et al. Agenesis of kidney associated with malformation of the seminal vesicle: various clinical presentations. Int Urol Nephrol 1988;20:29–33. Trigaux JP, Van Beers B, Delchambre F. Male genital tract malformations associated with ipsilateral renal agenesis: sonographic findings. J Clin Ultrasound 1991;19:3–10. Walls WJ, Lin F. Ultrasonic diagnosis of seminal vesicle cysts. Radiology 1975;114:693–4. King BF, Hattery RR, Lieber MM, et al. Congenital cystic disease of the seminal vesicle. Radiology 1991;178:207–11. Narlawar RS, Hanchate V, Raut A, et al. Renal agenesis and seminal vesicle cyst. J Ultrasound Med 2003;22:225–8. Murphy JO, Power RE, Akhtar M, et al. Magnetic resonance imaging in the diagnosis of seminal vesicle cysts and associated anomalies. J Urol 2003;170:2386. Kuligowska E, Fenlon HM. Transrectal US in male infertility: spectrum of findings and role in patient care. Radiology 1998;207:173–81. Ammar T, Sidhu PS, Wilkins CJ. Male infertility: the role of imaging in diagnosis and management. Br J Radiol 2012;85:S59–68. Altobelli E, Bove AM, Falavolti C, et al. Robotic-assisted laparoscopic approach in the treatment for Zinner’s syndrome associated with ipsilateral megaureter and incomplete double-crossed ectopic ureter. Int Urol Nephrol 2013;45:635–8. Jarzemski P, Lisopadzki S, Kowalski M. Laparoscopic removal of a congenital seminal vesicle cyst in Zinner’s syndrome. JSLS 2014;18:367–71. Benyó M, Berczi C, Jozsa T, et al. Fertility preservation in cases of laparoscopic treatment of seminal vesicle cysts. Cent European J Urol 2012;65:144–5.
Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
Rare disease Copyright 2015 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Sundar R, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-207618
5
