Андрология и генитальная хирургия. 2020; 21: 60-67
Синдром Herlyn–Werner–Wunderlich в препубертатном периоде (обзор литературы и клинические наблюдения)
https://doi.org/10.17650/2070-9781-2020-21-4-60-67Аннотация
Синдром Herlyn–Werner–Wunderlich (синдром OHVIRA) – сочетанный порок развития мочеполовой системы, характеризу ющийся различными комбинациями удвоения матки и влагалища с формированием замкнутой вагины с одной стороны и дисгенезией ипсилатеральной почки и мочеточника. Причинами ошибок диагностики и лечения являются относительная редкость порока, недостаточная информированность практических врачей о синдроме, отсутствие мультидисциплинарного подхода. Несвоевременная и тактически хаотичная диагностика синдрома Herlyn–Werner–Wunderlich приводит к неверному представлению о клинической ситуации, ошибочному выбору объема лечения и, как следствие, к таким осложнениям, как стриктуры, распространенный спаечный и воспалительный процесс, а также к необратимым изменениям топографии органов малого таза с последующим ухудшением репродуктивного потенциала пациентки. В данной статье представлен обзор литературы по проблеме, рассмотрены клинические случаи диагностики данного порока у пациенток в препубертатном периоде.
Список литературы
1. Piccinini P.S., Doski J. Herlyn–Werner–Wunderlich syndrome: a case report. Rev Bras Ginecol Obstet 2015;37(4):192–6. DOI: 10.1590/SO100-720320150005077.
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24. Tan Y.G., Laksmi N.K., Yap T.L. et al. Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): early diagnosis and management of asymptomatic Herlyn–Werner–Wunderlich syndrome. J Pediatr Surg 2020;55(7):1377–80. DOI: 10.1016/j.jpedsurg.2019.06.006.
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27. Divina F.F., Olivieri C., Cannone R. et al. In-office hysteroscopic treatment of Herlyn-Werner-Wunderlich syndrome: a case series. J Minim Invasive Gynecol 2020;27(7):1640–5. DOI: 10.1016/j.jmig.2020.04.013.
28. Oelschlager A.-M.A., Symons J., Shnorhavorian M. et al. Prepubertal vaginal septum resection for obstructed hemivagina ipsilateral renal anomaly. J Pediatr Adolesc Gynecol 2017;30(2):310–1. DOI: 10.1016/j.jpag.2017.03.091.
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30. Ameh E.A., Mshelbwala P.M., Ameh N. Congenital vaginal obstruction in neonates and infants: recognition and management. J Pediatr Adolesc Gynecol 2011;24(2):74–8. DOI: 10.1016/j.jpag.2010.08.016.
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Andrology and Genital Surgery. 2020; 21: 60-67
Herlyn–Werner–Wunderlich syndrome in the prepubescent period (literature review and clinical observations)
https://doi.org/10.17650/2070-9781-2020-21-4-60-67Abstract
Herlyn–Werner–Wunderlich syndrome (OHVIRA syndrome) is a combined malformation of the genitourinary systeme, characterized by various combinations of uterus dydelphys with unilateral obstructed (or blind) hemivagina and ipsilateral renal agenesis. The causes of mistakes in diagnosis and treatment are common because of relative rarity of anomaly, insufficient awareness of practitioners about the syndrome and the lack of multidisciplinary approach. Untimely and tactically chaotic diagnosis of Herlyn–Werner–Wunderlich syndrome leads to a misconception about the clinical situation, wrong choice in treatment, and, as a consequence, to complications such as strictures, widespread adhesions and inflammation, as well as irreversible changes in the topography of organs of the small pelvis with a subsequent deterioration in reproductive status of patient. This article provides a review of the literature on the problem, considers the clinical cases of diagnosing this defect in prepubertal patients.
References
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15. Gazárek F., Kudela M., Zenisek L., Nevrla F. [Herlyn–Werner and Wunderlich syndromes (In German)]. Zentralbl Gynakol 1979;101(21):1411–5.
16. Zhu L., Chen N., Tong J.-L. et al. New classification of Herlyn–Werner–Wunderlich syndrome. Chin Med J (Engl) 2015;128(2):222–5. DOI: 10.4103/0366-6999.149208.
17. Zhang J., Zhang M., Zhang Y. et al. Proposal of the 3O (obstruction, ureteric orifice, and outcome) subclassification system associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). J Pediatr Adolesc Gynecol 2020;33(3):307–13. DOI: 10.1016/j.jpag.2020.01.001.
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23. Wang S., Lang J.H., Zhu L., Zhou H.M. Duplicated uterus and hemivaginal or hemicervical atresia with ipsilateral renal agenesis: an institutional clinical series of 52 cases. Eur J Obstet Gynecol Reprod Biol 2013;170(2):507–11. DOI: 10.1016/j.ejogrb.2013.07.015.
24. Tan Y.G., Laksmi N.K., Yap T.L. et al. Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): early diagnosis and management of asymptomatic Herlyn–Werner–Wunderlich syndrome. J Pediatr Surg 2020;55(7):1377–80. DOI: 10.1016/j.jpedsurg.2019.06.006.
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28. Oelschlager A.-M.A., Symons J., Shnorhavorian M. et al. Prepubertal vaginal septum resection for obstructed hemivagina ipsilateral renal anomaly. J Pediatr Adolesc Gynecol 2017;30(2):310–1. DOI: 10.1016/j.jpag.2017.03.091.
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31. Skinner B., Quint E.H. Obstructive reproductive tract anomalies: a review of surgical management. J Minim Invasive Gynecol 2017;24(6):901–8. DOI: 10.1016/j.jmig.2017.04.020.
