Herlyn — Werner — Wunderlich syndrome: the importance of timely diagnosis (presentation of the clinical case and literature review)
Abstract
Herlin — Werner — Wunderlich syndrome (HWWS) is a very rare defect of the genitourinary system, which occurs due to abnormal development of the Mullerian ducts. The defect is characterized by a variety of forms and combinations. Most often, uterine duplication occurs in combination with OHVIRA syndrome (Obstructed HemiVagina and Ipsilateral Renal Anomaly). Hence the difficulties in diagnosing and treating this pathology.
The article presents a description of a clinical case of HWWS in a 14-year-old patient and a brief review of the literature. Also described: clinical picture, diagnostic methods, observation and surgical treatment. In this patient, HWWS was complicated by hematopiocolpos, hematometra and hematosalpinx on the left. A description of a clinical case of this syndrome in the Republic of Belarus is provided for the first time.
About the Authors
A. D. Zhukovets
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
O. O. Pavlyuchenya
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
D. A. Ovseichik
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
P. K. Marakhouskaya
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
I. A. Seukouski
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
A. A. Svirsky
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
T. M. Lechtchinskaya
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
L. N. Nestsiaruk
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
K. V. Nikalayeva
Республиканский научно-практический центр детской хирургии
Belarus
Belarus
References
1. Алгоритм диагностики и лечения девочек с врожденными пороками развития органов репродуктивной системы / В. А. Крутова [и др.] // Репродуктив. здоровье детей и подростков. — 2015. — № 1. — С. 30—36.
2. Clinical implications of uterine malformations and hysteroscopic treatment result / G. F. Grimbizis [et al.] // Human Reprod. Update. — 2001. — Vol. 7, № 2. — P. 161—174.
3. Saravelos, S. H. Prevalence and diagnosis of congenital uterine anomalies in women with reproductive failure: a critical appraisal / S. H. Saravelos, K. A. Cocksedge, T.-C. Li // Human Reprod. Update. — 2008. — Vol. 14, № 5. — P. 415—429.
4. Panaitescu, A. M. Herlyn — Werner — Wunderlich syndrome: case report and review of the literature / A. M. Panaitescu, G. Peltecu, N. Gica // Diagnostics. — 2022. — Vol. 12, № 10. — P. 2466.
5. Синдром Херлина — Вернера — Вундерлиха: клиническое наблюдение пациентки 17 лет / С. С. Никитин [и др.] // Педиатрия. Журнал им. Г. Н. Сперанского. — 2021. — Т. 100, № 4. — С. 185—189.
6. The Herlyn — Werner — Wunderlich triad (OHVIRA syndrome) with good pregnancy outcome: two cases and literature review / D. M. Albulescu [et al.] // Romanian J. of Morphol. and Embryol. — 2018. — Vol. 59, № 4. — P. 1253—1262.
7. Clinical and radiological findings for early diagnosis of Herlyn — Werner — Wunderlich syndrome in pediatric age: experience of a single center / C. Noviello [et al.] // Gynecological Endocrinology. — 2018. — Vol. 34, № 1. — P. 56—58.
8. Purslow, C. E. A case of unilateral haematocolpos, haematometra and haematosalpinx / C. E. Purslow // The J. of Obstetrics and Gynaecol. of the Brit. Empire. — 1922. — Vol. 29, № 4. — P. 643.
9. Herlyn, U. Das gemeinsame Vorkommen von effener Gartner-Gang-Zyste, gleichseitiger Nierenaplasie und Uterusdoppelmissbildung als typisches Missbildungssyndrom / U. Herlyn, H. Werner // Geburtshilfe und Frauenheilkunde. — 1971. — Vol. 31, № 4. — P. 340—347.
10. Wunderlich, M. SelteneVariante einer Genitalmissbildung mit Aplasie der rechten Niere / M. Wunderlich // Zentr.-Bl. für Gynakologie. — 1976. — Vol. 98, № 9. — P. 559—562.
11. Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis inteenagers: report of three cases / G. Tridenti [et al.] // Amer. J. of Obstetrics and Gynecol. — 1988. — Vol. 159, № 4. — P. 882—883.
12. Yi, S. Clinical characteristics and management of patients with complete septate uretus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis / S. Yi, J. Jiang // The J. of Obstetrics and Gynaecol. Res. — 2021. — Vol. 47, № 4. — P. 1497—1501.
13. Herlyn — Werner — Wunderlich syndromne: case review and report of the literature / O. O. Gutierrez-Montufar [et al.] // Rev. Colombiana de Obstetricia y Ginecologia. — 2021. — Vol. 72, № 4. — P. 407—422.
14. An unusual case of Herlyn — Werner — Wunderlich syndrome with a complete septate uterus and complicated urinary tract with rare ectopic ureteral opening / A. Zhang [et al.] // Intern. J. of Gynecol. and Obstetrics. — 2018. — Vol. 143, № 2. — P. 248—250.
15. Roch, J. A. The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis / J. A. Roch, H. W. Jones // Amer. J. of Obstetrics and Gynecol. — 1980. — Vol. 138, № 3. — P. 339—342.
16. Vaginoscopic incision of oblique vaginal septum in adolescents with OHVIRA syndrome / C. Cheng [et al.] // Sci. Rep. — 2019. — Vol. 9, № 1. — P. 20042.
17. A variant of Herlyn — Werner — Wunderlich syndrome presenting with acute abdomen: a case report and review of literature / N. Gupta [et al.] // Global Pediatric Health. — 2018. — Vol. 5. — P. 1—5.
18. Smith, N. A. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: managament and follow-up / N. A. Smith, M. R. Laufer // Fertility a. Sterility. — 2007. — Vol. 87, № 4. — P. 918—922.
19. Proposal of the 3O (obstruction, ureteric orifice, and outcome) subclassification system associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) / J. Zhang [et al.] // J. of Pediatric and Adolescent Gynecol. — 2020. — Vol. 33, № 3. — P. 307—313.
20. Урологические аномалии при синдроме OHVIRA (Herlyn — Werner — Wunderlich синдром) / К. Лодейкина [и др.] // Вестник урологии. — 2022. — Т. 10, № 2. — С. 109—123.
21. Paccini, P. S. Herlyn — Werner — Wunderlich syndrome: a case report / P. S. Paccini, J. Doski // Rev. Brasil. de Ginecologia e Obstetricia. — 2015. — Vol. 37, № 4. — P. 192—196.
22. Afrashtehfar, C. D. Malformaciones mullerianas. Sindrome de hemivagina obstruida y anomalia renal ipsilateral (OHVIRA) / C. D. Afrashtehfar, A. Pina-Garcia, K. I. Afrashtehfar // Cirugia y Cirujanos. — 2014. — Vol. 82, № 4. — P. 460—471.
23. Burgis, J. Obstructive Müllerian anomalies: case report, diagnosis, and management / J. Burgis // Amer. J. of Obstetrics and Gynecol. — 2001. — Vol. 185, № 2. — P. 338—344.
24. Didelphys uterus: a case report and review of literature / S. Rezai [et al.] // Case Rep. in Obstetrics and Gynecol. — 2015. — Vol. 2015. — P. 865821.
25. Complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) in a young woman — a rare variant of Herlyn — Werner — Wunderlich syndrome / M. Surya [et al.] // BJR Case Rep. — 2016. — Vol. 2, № 2. — P. 20150241.
26. Herlyn — Werner — Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis / R. Del Vescovo [et al.] // BMC Med. Imaging. — 2012. — Vol. 12, № 1. — P. 4.
27. Santos, X. M. Obstructed hemivagina with ipsilateral renal anomaly / X. M. Santos, J. E. Dietrich // J. of Pediatric and Adolescent Gynecol. — 2016. — Vol. 29, № 1. — P. 7—10.
28. Shah, D. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus / D. Shah, D. Laufer // Fertility and Sterility. — 2011. — Vol. 96, № 1. — P. e39—e41.
29. Clinical characteristics and treatment of Herlyn — Werner — Wunderlich syndrome / J. Wang [et al.] // Arch. of Gynecol. and Obstetrics. — 2014. — Vol. 290, № 5. — P. 947—950.
30. A rare cause of acute abdominal pain: Herlyn — Werner — Wunderlich syndrome / R. Aydin [et al.] // Pediatric Emergency Care. — 2014. — Vol. 30, № 1. — P. 40—42.
31. Misina, A. Diagnosticul si tratamentul sindromului Herlyn — Werner — Wunderlich / A. Misina // Arch. of the Balkan Med. Union. — 2015. — Vol. 50, № 2 (suppl. 1). — P. 93—99.
32. Sindrome de hemivagina obstruida, con agenesia renal ipsilateral: causa inusual de piocolpos. Reporte de caso y revision bibliografica / D. K. Cortes-Contreras [et al.] // Ginecologia y Obstetricia de Mexico. — 2014. — Vol. 82, № 10. — P. 711—715.
33. Enfermedad inflamatoria pelvica causada por el síndrome de Herlyn — Werner — Wunderlich / M. T. Alumbreros-Andujar [et al.] // Cirugia y Cirujanos. — 2014. — Vol. 82, № 4. — P. 448—452.
34. Herlyn — Werner — Wunderlich syndrome : report of a prenatally recognised case and review of the literature / T. Tuna [et al.] // Urology. — 2019. — Vol. 125. — P. 205— 209.
35. Pitfalls in the diagnosis and management of obstructive uterovaginal duplication : a series of 32 cases / C. Capito [et al.] // Pediatrics. — 2008. — Vol. 122, № 4. — P. e891—e897.
36. Пренатальная диагностика синдрома обструкции одного из удвоенных влагалищ в сочетании с ипсилатеральной аномалией почки / К. В. Костюков [и др.] // Ультразвуковая и функциональная диагностика. — 2011. — № 3. — С. 78—83.
37. Youssef, M. A. F. M. Obstructed hemivagina and ipsilateral renal anomaly sindrom with uterus didelphys (OHVIRA) / M. A. F. M. Youssef // Middle East. Fertility Soc. J. — 2013. — Vol. 18, № 1. — P. 58—61.
38. In-office hysteroscopic treatment of Herlyn — Werner — Wunderlich syndrome: a case series / F. D. Fascilla [et al.] // J. of Minimally Invasive Gynecology. — 2020. — Vol. 27, № 7. — P. 1640—1645.
Review
For citations:
Zhukovets A.D., Pavlyuchenya O.O., Ovseichik D.A., Marakhouskaya P.K., Seukouski I.A., Svirsky A.A., Lechtchinskaya T.M., Nestsiaruk L.N., Nikalayeva K.V. Herlyn — Werner — Wunderlich syndrome: the importance of timely diagnosis (presentation of the clinical case and literature review). Healthcare. 2024;1(11):65-73. (In Russ.)
Views:
13
Email this article 