Zinner Syndrome Versus Herlyn-Werner-Wunderlich syndrome; Rare Congenital Genitourinary Malformations and Literature Review

Year 2024, Volume: 19 Issue: 2, 95 - 102, 29.06.2024

Saim Türkoğlu , Cemil Göya , Murat Demir

https://doi.org/10.33719/nju1421307

Abstract

Zinner syndrome(ZS) and Herlyn-Werner-Wunderlich syndrome (HWWS) are congenital genitourinary anomalies accompanied by ipsilateral renal agenesis. These syndromes, which can cause symptoms such as infertility, pelvic pain, and bladder irritation, can be diagnosed by digital rectal examination, transrectal and abdominal ultrasonography(US), Computer Tomography(CT) and more ideally Magnetic resonance imaging (MRI). Similar physiopathological processes are observed in both syndromes and urogenital malformations accompany renal agenesis. We aimed to discuss the three cases of ZS in men and two HWWS cases in women, which are rarely observed in the literature.

Keywords

Zinner syndrome, Herlyn-Werner-Wunderlich syndrome, Seminal vesicle cysts, renal agenesis, mullerian duct anomaly, blind hemivagina, uterus didelfis

References

• Slaoui A, Regragui S, Lasri A, Karmouni T, El Khader K, Koutani A, Ibn Attya A. Zinner’s syndrome: report of two cases and review of the literature. Basic Clin Androl. 2016 Sep 20;26:10. https://doi.org/10.1186/s12610-016-0037-4.
• Zhang H, Zheng Y, Ning G, Fu C, Bao L. Preoperative MRI presentations of Herlyn-Werner-Wunderlich syndrome. Congenit Anom (Kyoto). 2022 Nov;62(6):228-235. https://doi.org/10.1111/cga.12489.
• Gutiérrez-Montufar OO, Zambrano-Moncayo CP, Otálora-Gallego MC, Meneses-Parra AL, Díaz-Yamal I. Herlyn-Werner-Wunderlich syndromne: case review and report of the literature. Rev Colomb Obstet Ginecol. 2021 Dec 30;72(4):407-422. English, Spanish. https://doi.org/10.18597/rcog.3699.
• George Wilson J. A case of double uterus and vagina with unilateral hematocolpos and hematometra. J Obstet Gynaecol Br Emp. 1925;32: 127-8.
• Nicolaou N, Renkema KY, Bongers EM, Giles RH, Knoers NV. Genetic, environmental, and epigenetic factors involved in CAKUT. Nat Rev Nephrol. 2015 Dec;11(12):720-31. https://doi.org/10.1038/nrneph.2015.140.
• Heidet L, Morinière V, Henry C, De Tomasi L, Reilly ML, Humbert C, Alibeu O, Fourrage C, Bole-Feysot C, Nitschké P, Tores F, Bras M, Jeanpierre M, Pietrement C, Gaillard D, Gonzales M, Novo R, Schaefer E, Roume J, Martinovic J, Malan V, Salomon R, Saunier S, Antignac C, Jeanpierre C. Targeted Exome Sequencing Identifies PBX1 as Involved in Monogenic Congenital Anomalies of the Kidney and Urinary Tract. J Am Soc Nephrol. 2017 Oct;28(10):2901-2914. https://doi.org/10.1681/ASN.2017010043.
• Georgas KM, Armstrong J, Keast JR, Larkins CE, McHugh KM, Southard-Smith EM, Cohn MJ, Batourina E, Dan H, Schneider K, Buehler DP, Wiese CB, Brennan J, Davies JA, Harding SD, Baldock RA, Little MH, Vezina CM, Mendelsohn C. An illustrated anatomical ontology of the developing mouse lower urogenital tract. Development. 2015 May 15;142(10):1893-908. https://doi.org/10.1242/dev.117903.
• Hannema SE, Hughes IA. Regulation of Wolffian duct development. Horm Res. 2007;67(3):142-51. https://doi.org/10.1159/000096644.
• Kao CC, Wu CJ, Sun GH, Yu DS, Chen HI, Chang SY, Ma CP, Cha TL. Congenital seminal vesicle cyst associated with ipsilateral renal agenesis mimicking bladder outlet obstruction: a case report and review of the literature. Kaohsiung J Med Sci. 2010 Jan;26(1):30-4. https://doi.org/10.1016/S1607-551X(10)70005-X.
• Shah S, Patel R, Sinha R, Harris M. Zinner syndrome: an unusual cause of bladder outflow obstruction. BJR Case Rep. 2017 Feb 15;3(2):20160094. https://doi.org/10.1259/bjrcr.20160094. Protogerou, V., Chorti, M. & Kostakopoulos, A. Congenital dilation of seminal vesicle with agenesis of ipsilateral kidney: Symptoms and treatment. Int Urol Nephrol 35, 69–71 (2003). https://doi.org/10.1023/A:1025902304134
• Girardi Fachin C, Aleixes Sampaio Rocha JL, Atuati Maltoni A, das Chagas Lima RL, Arias Zendim V, Agulham MA, Tsouristakis A, Dos Santos Dias AIB. Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature. Int J Surg Case Rep. 2019;63:129-134. https://doi.org/10.1016/j.ijscr.2019.08.035.
• Behr SC, Courtier JL, Qayyum A. Imaging of müllerian duct anomalies. Radiographics. 2012 Oct;32(6):E233-50. https://doi.org/10.1148/rg.326125515.
• Sadler TW. Langman’s medical embryology. 12th ed. Philadelphia: Lippincott Williams & Wilkins; 2012.
• Acién P, Acién MI. The history of female genital tract malformation classifications and proposal of an updated system. Hum Reprod Update. 2011 Sep-Oct;17(5):693-705. https://doi.org/10.1093/humupd/dmr021.
• Tan Z, Li B, Zhang L, Han P, Huang H, Taylor A, Li X. Classifying seminal vesicle cysts in the diagnosis and treatment of Zinner syndrome: A report of six cases and review of available literature. Andrologia. 2020 Feb;52(1):e13397. https://doi.org/10.1111/and.13397.
• Rappe BJ, Meuleman EJ, Debruyne FM. Seminal vesicle cyst with ipsilateral renal agenesis. Urol Int. 1993;50(1):54-6. https://doi.org/10.1159/000282451.
• Kenney PJ, Leeson MD. Congenital anomalies of the seminal vesicles: spectrum of computed tomographic findings. Radiology. 1983 Oct;149(1):247-51. https://doi.org/10.1148/radiology.149.1.6611933.