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Zinner syndrome in children: clinical presentation, imaging findings, diagnosis, and outcome

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Zinner syndrome (ZS), the association of congenital seminal vesicle cyst (SVC) and ipsilateral kidney anomalies, is rarely diagnosed in childhood. This study aimed to assess presentation, imaging findings, management, and outcome of pediatric ZS.


Sixteen children with ZS were diagnosed and managed at our hospital from 2003 to 2021. We reviewed the medical records to collect data on initial symptoms, results of imaging studies, complications, operation, and follow-up.


Ultrasound was used in all 16 cases as initial diagnostic tool. Fourteen patients were asymptomatic at diagnosis: these were transferred from obstetricians or pediatricians for evaluation of the prenatally or postnatally detected ultrasonic kidney anomalies. SVCs were incidentally noted on ultrasonography. The other two cases initially presented with urinary tract infection (UTI). Kidney anomalies included multicystic dysplastic kidney in 3 and kidney agenesis in 13 patients. Eleven (68.7%) patients had ipsilateral ectopic ureters entering SVC. Four (36.4%) patients had a reflux from urethra into SVC (urethro-cystic reflux) on voiding cystourethrography. Ten (62.5%) patients remained asymptomatic over a mean of 58 months (range, 7–216 months), two patients developed lower urinary tract dysfunction, and five patients had UTIs. Two boys needed SVC removal, and SVC had disappeared in two patients after 2.5–4 years of follow-up.


Unilateral kidney hypodysplasia with ectopic ureter inserting into the ipsilateral SVC is a characteristic sign for diagnosis of ZS. In our case series, ZS was mainly asymptomatic. Urethro-cystic reflux was associated with UTIs in young infants. SVC removal was rarely required.

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  1. Zinner A (1914) Ein fall von intravesikaler samen-blasencyste. Wien Med Wochenschr 64:605–609

    Google Scholar 

  2. Sheih CP, Hung CS, Wei CF, Lin CY (1990) Cystic dilatations within the pelvis in patients with ipsilateral renal agenesis or dysplasia. J Urol 144:324–327

    Article  CAS  Google Scholar 

  3. Schukfeh N, Kuebler JF, Schirg E, Petersen C, Ure BM, Glüer S (2009) Dysplastic kidney and not renal agenesis is the commonly associated anomaly in infants with seminal vesicle cyst. BJU Int 103:816–819

    Article  Google Scholar 

  4. Haddock P, Wagner JR (2015) Seminal vesicle cyst with ipsilateral renal agenesis and ectopic ureter (Zinner Syndrome). Urology 85:e41-42

    Article  Google Scholar 

  5. Kanavaki A, Vidal I, Merlini L, Hanquinet S (2015) Congenital seminal vesicle cyst and ipsilateral renal agenesis (Zinner Syndrome): a rare association and its evolution from early childhood to adolescence. Eur J Pediatr Surg Reports 3:98–102

    Article  Google Scholar 

  6. Livingston L, Larsen CR (2000) Seminal vesicle cyst with ipsilateral renal agenesis. AJR Am J Roentgenol 175:177–180

    Article  CAS  Google Scholar 

  7. Ahallal Y, Tazi MF, Khallouk A, Elammari J, Elfassi MJ, Farih MH (2011) Conservative management of a congenital seminal vesicle cyst associated with ipsilateral renal agenesis revealed by cystitis: one case report. Case Rep Urol 2011:125753

  8. Steffens J, Oberschulte-Beckmann D, Siller V, Röttger P, Polsky MS (2000) Ectopic refluxing ureter entering a seminal vesicle cyst associated with ipsilateral renal dysplasia. World J Urol 18:232–234

    Article  CAS  Google Scholar 

  9. Takemura K, Sato A, Morizawa Y, Kufukihara R, Iwasa S, Satoh H (2021) Seminal vesicle cysts with upper urinary tract abnormalities: a single-center case series of pediatric Zinner syndrome. Urology 149:e44–e47

    Article  Google Scholar 

  10. Jiang XS, Wang HJ, Lin JH, Guo Y, Sun CH, Lin L, Guan J (2018) Zinner’s syndrome: clinical features and imaging diagnosis. Asian J Androl 20:316–317

    Article  Google Scholar 

  11. Cascini V, Renzo DD, Guerriero V, Lauriti G, Chiesa PL (2019) Zinner syndrome in pediatric age: issues in the diagnosis and treatment of a rare malformation complex. Front Pediatr 7:129

    Article  Google Scholar 

  12. Hong YK, Onal B, Diamond DA, Retik AB, Cendron M, Nguyen HT (2011) Robot—assisted laparoscopic excision of symptomatic retrovesical cysts in boys and young adults. J Urol 186:2372–2378

    Article  Google Scholar 

  13. Chen HW, Huang SC, Li YW, Chen SJ, Sheih CP (2006) Magnetic resonance imaging of seminal vesicle cyst associated with ipsilateral urinary anomalies. J Formos Med Assoc 105:125–131

    Article  Google Scholar 

  14. Sheih CP, Liao YJ, Li YW, Yang LY (1993) Seminal vesicle cyst associated with ipsilateral renal malformation and hemivertebra: report of 2 cases. J Urol 150:1214–1215

    Article  CAS  Google Scholar 

  15. Valla JS, Carfagna L, Tursini S, Mohaidi MAL, Bosson N, Steyaert H (2003) Congenital seminal vesicle cyst: prenatal diagnosis and postnatal laparoscopic excision with an attempt to preserve fertility. BJU Int 91:891–892

    Article  CAS  Google Scholar 

  16. King BF, Hattery RR, Lieber MM, Berquist TH, Williamson B, Hartman GW (1991) Congenital cystic disease of the seminal vesicle. Radiology 178:207–211

    Article  CAS  Google Scholar 

  17. Moore CD, Erhard MJ, Dahm P (2007) Robot-assisted excision of seminal vesicle cyst associated with ipsilateral renal agenesis. J Endourol 21:776–779

    Article  Google Scholar 

  18. Sheih CP, Li YW, Liao YJ, Hung CS (1998) Bilateral congenital cysts of the seminal vesicle with bilateral duplex kidney. J Urol 160:184–185

    Article  CAS  Google Scholar 

  19. Basillote JB, Shanberg AM, Woo D, Perer E, Rajpoot D, Clayman RV (2004) Laparoscopic excision f a seminal vesicle cyst in a child. J Urol 171:369–371

    Article  Google Scholar 

  20. Murphy JO, Power RE, Akhtar M, Torreggiani WC, McDermott TED, Thornhill JA (2003) Magnetic resonance imaging in the diagnosis of seminal vesicle cysts and associated anomalies. J Urol 170:2386

    Article  CAS  Google Scholar 

  21. Jung YS, Wee JH, Choi JB, Choi MS, Kim S, Sohn JH, Lee JH, Kang SH, Lee YS, Han CH (2011) Laparoscopic excision of a congenital seminal vesicle cyst and coexisting ipsilateral renal agenesis. Korean J Androl 29:251–253

    Article  Google Scholar 

  22. Humke U, Uder M, Siemer S, Ziegler M, Kramann B (1997) Congenital cysts of the seminal vesicles with ipsilateral kidney agenesis. Clinical aspects of 7 cases. Ann Urol (Paris) 31:184–190

    CAS  Google Scholar 

  23. Fujinaga S, Hirano D, Hara S, Uchida H, Kitano Y, Kobayashi K, Tada M, Someya T, Ohtomo Y, Shimizu T (2008) Seminal vesicle abscesses associated with ipsilateral multicystic dysplastic kidney in an infant. Pediatr Nephrol 23:1551–1554

    Article  Google Scholar 

  24. Terai A, Tsuji Y, Terachi T, Yoshida O (1995) Ectopic ureter opening into the seminal vesicle in an infant: a case report and review of the Japanese literature. Int J Urol 2:128–131

    Article  CAS  Google Scholar 

  25. Moore KL (1997) The developing human, 2nd edn. Saunders, Philadelphia, pp 220–246

    Google Scholar 

  26. Williams RD, Sandlow JI (1998) Surgery of the seminal vesicles. In: Walsh PC, Retik AB, Vaughan ED, Wein AJ (eds) Campbell’s urology, 7th edn. Saunders, Philadelphia, pp 3299–3307

    Google Scholar 

  27. Shebel HM, Farg HM, Kolokythas O, El-Diasty T (2013) Cysts of the lower male genitourinary tract: embryologic and anatomic considerations and differential diagnosis. Radiographics 36:1373–1389

    Google Scholar 

  28. Denes FT, Montanellato NI, Lopes RN, Barbosa Filho CM, Cabral AD (1986) Seminal vesicle cyst and ipsilateral renal agenesis. Urology 28:313e5

    Article  Google Scholar 

  29. Paliwalla M, Park K (2014) A practical guide to urinary tract ultrasound in a child: pearls and pitfalls. Ultrasound 22:213–222

    Article  CAS  Google Scholar 

  30. Berrocal T, López-Pereira P, Arjonilla A, Gutiérrez J (2002) Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features. Radiographics 22:1139–1164

    Article  Google Scholar 

  31. Hains DS, Bates CM, Ingraham S, Schwaderer AL (2009) Management and etiology of the unilateral multicystic dysplastic kidney: a review. Pediatr Nephrol 24:233–241

    Article  Google Scholar 

  32. van den Ouden D, Blom JH, Bangma C, de Spiegeleer AH (1998) Diagnosis and management of seminal vesicle cysts associated with ipsilateral renal agenesis: a pooled analysis of 52 cases. Eur Urol 33:433–440

    Article  Google Scholar 

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Authors and Affiliations



Study conception and design: Chun-Chen Lin and Jeng-Daw Tsai. Material preparation, data collection and analysis: Pei-Shan Tsai, Ming-Dar Lee and Tsu-Hua Lin. Writing—original draft preparation: Chun-Chen Lin. Writing—review and editing: Jeng-Daw Tsai. Supervision: Jin-Cherng Sheu.

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Correspondence to Jeng-Daw Tsai.

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Lin, CC., Sheu, JC., Tsai, PS. et al. Zinner syndrome in children: clinical presentation, imaging findings, diagnosis, and outcome. Pediatr Nephrol 37, 3075–3084 (2022).

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