DIAGNOSTICS AND TREATMENT OF HERLYN-WERNER-WUNDERLICH SYNDROME IN A NEWBORN GIRL
Igor N. Khvorostov,
Aleksey G. Sinitsyn,
Anastasiya V. Berdnikova,
Mariya V. Klimova,
Mariya A. Yakhontova https://doi.org/10.46563/1560-9561-2021-24-1-56-60
Abstract
Herlin-Werner-Vanderlich syndrome refers to the Müllerian duct anomalies, including uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. At the first stage of treatment, a reasonable intervention is opening the hemivaginal septum by extra-vaginal access under visual control or using vaginoscopy. We identified a rare variant of the defect identified, since the longitudinal septum of the hemivagina did not prolapse through the introinus, which required a complex diagnosis and surgical aid in the newborn. A significant volume of hydrometrocolpos was associated with the accumulation of urine from the right hypoplasmic kidney with an abnormal flow of the ectopic ureter into the cavity of the atresized vagina. It determined the need for surgical treatment for urgent indications. In this regard, a biochemical analysis of the fluid obtained after drainage of the hydrometrocolpos with the detection of the concentration of urea, creatinine, total protein, and cellular composition is mandatory.
About the Authors
Igor N. Khvorostov
Volgograd State Medical University
Russian Federation
Russian Federation
Aleksey G. Sinitsyn
Volgograd State Medical University
Russian Federation
Russian Federation
Anastasiya V. Berdnikova
Volgograd State Medical University
Russian Federation
Russian Federation
Mariya V. Klimova
Volgograd State Medical University
Russian Federation
Russian Federation
Mariya A. Yakhontova
Volgograd State Medical University
Russian Federation
Russian Federation
References
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19. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions. Fertil. Steril. 1988; 49(6): 944-55. https://doi.org/10.1016/s0015-0282(16)59942-7
20. Wunderlich M. Unusual form of genital malformation with aplasia of the right kidney. Zentralbl. Gynakol. 1976; 98(9): 559-62. (in German)
21. Smith N.A., Laufer M.R. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil. Steril. 2007; 87(4): 918-22. https://doi.org/10.1016/j.fertnstert.2006.11.015
22. Herlyn U., Werner H. Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Geburtshilfe Frauenheilkd. 1971; 31(4): 340-7. (in German)
23. Vercellini P., Daguati R., Somigliana E., Viganò P., Lanzani A., Fedele L. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review. Fertil. Steril. 2007; 87(4): 719-24. https://doi.org/10.1016/j.fertnstert.2007.01.173
24. Orazi C., Lucchetti M.C., Schingo P.M., Marchetti P., Ferro F. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr. Radiol. 2007; 37(7): 657-65. https://doi.org/10.1007/s00247-007-0497-y
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26. Шидаков И. Х., Калниязов Б. М. Гидрометрокольпос у новорождённого. Российский вестник детской хирургии, анестезиологии и реаниматологии. 2019; 9(4): 88-93. https://doi.org/10.30946/2219-4061-2019-9-4-88-9311
27. Epelman M., Dinan D., Gee M.S., Servaes S., Lee E.Y., Darge K. Müllerian duct and related anomalies in children and adolescents. Magn. Reson. Imaging Clin. N. Am. 2013; 21(4): 773-89. https://doi.org/10.1016/j.mric.2013.04.011
28. Acién P., Acién M., Sánchez-Ferrer M. Complex malformations of the female genital tract. New types and revision of classification. Hum. Reprod. 2004; 19(10): 2377-84. https://doi.org/10.1093/humrep/deh423
29. Костюков К.В., Подуровская Ю.Л., Кучеров Ю.И., Гус А.И. Пренатальная диагностика синдрома обструкции одного из удвоенных влагалищ в сочетании с ипсилатеральной аномалией почки. Ультразвуковая и функциональная диагностика. 2011; (3): 78-83
30. Wu T.H., Wu T.T., Ng Y.Y., Ng S.C., Su P.H., Chen J.Y., et al. Herlyn-Werner-Wunderlich syndrome consisting of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis in a newborn. Pediatr. Neonatol. 2012; 53(1): 68-71. https://doi.org/10.1016/j.pedneo.2011.11.014
31. Garge S., Bagga D., Acharya S.K., Yadav D.K., Khan T.R., Kumar R., et al. Herlyn-Weber-Wunderlich syndrome with ectopic ureter in prepubertal female. J. Indian Assoc. Pediatr. Surg. 2014; 19(2): 103-5. https://doi.org/10.4103/0971-9261.129607
32. Wang Z.J., Daldrup-Link H., Coakley F.V., Yeh B.M. Ectopic ureter associated with uterine didelphys and obstructed hemivagina: preoperative diagnosis by MRI. Pediatr. Radiol. 2010; 40(3): 358-60. https://doi.org/10.1007/s00247-009-1454-8
33. Gholoum S., Puligandla P.S., Hui T., Su W., Quiros E., Laberge J.M. Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). J. Pediatr. Surg. 2006; 41(5): 987-92. https://doi.org/10.1016/j.jpedsurg.2006.01.021
34. Wang J., Zhu L., Lang J., Liu Z., Sun D., Leng J., et al. Clinical characteristics and treatment of Herlyn-Werner-Wunderlich syndrome. Arch. Gynecol. Obstet. 2014; 290(5): 947-50. https://doi.org/10.1007/s00404-014-3286-5
35. Capito C., Echaieb A., Lortat-Jacob S. Pitfalls in the diagnosis and management of obstructive uterovaginal duplication: A series of 32 cases. Pediatrics. 2008; 122(4): 891-7. https://doi.org/10.1542/peds.2008-0219
36. Smith N.A., Laufer M.R. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil. Steril. 2007; 87(4): 918-22. https://doi.org/10.1016/j.fertnstert.2006.11.015
Review
For citations:
Khvorostov I.N., Sinitsyn A.G., Berdnikova A.V., Klimova M.V., Yakhontova M.A. DIAGNOSTICS AND TREATMENT OF HERLYN-WERNER-WUNDERLICH SYNDROME IN A NEWBORN GIRL. Russian Pediatric Journal. 2021;24(1):56-60. (In Russ.) https://doi.org/10.46563/1560-9561-2021-24-1-56-60
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