Uterus didelphys with obstructed hemivagina and contralateral multicystic dysplastic kidney.

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This case report describes uterus didelphys with an obstructed hemivagina and a contralateral multicystic dysplastic kidney, an unusual variation of the Herlyn-Werner-Wunderlich syndrome.

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This paper reports an unusual case of uterus didelphys with an obstructed hemivagina and a contralateral multicystic dysplastic kidney, part of the broader spectrum of Müllerian anomalies that are often associated with urinary tract anomalies (classically Herlyn–Werner–Wunderlich or OHVIRA). The authors describe the diagnostic context and emphasize that early diagnosis is important to prevent downstream complications such as adhesions and subsequent infertility, based on clinical reasoning and prior literature they cite, while also arguing that careful evaluation of the female reproductive tract may be needed in children with certain lateral renal anomalies. As a single case report, it is limited in generalizability and does not provide comparative outcomes. The paper does not explicitly discuss endometriosis or adenomyosis; it was included in the corpus via a keyword match in the upstream search index.

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Abstract

Müllerian anomalies are often associated with urinary tract anomalies. Herlyn–Werner–Wunderlich syndrome or obstructed hemivagina and ipsilateral renal anomaly are rare Müllerian anomaly comprising uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. We report an unusual case of uterus didelphys with obstructed hemivagina and contralateral (not ipsilateral) multicystic dysplastic kidney. An early diagnosis is critical to prevent complications such as adhesions with subsequent infertility. A careful evaluation of the female reproductive tract may be necessary in children with not only renal aplasia but also lateral renal anomalies such as multicystic dysplastic kidney.

References

Lin PC, Bhatnagar KP, Nettleton S, et al. Female genital anomalies affecting reproduction. Fertil Steril. 2002;78:899–915. Rackow BW, Arici A. Reproductive performance of women with Müllerian anomalies. Curr Opin Obstet Gynecol. 2007;19:229–37. Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and followup. Fertil Steril. 2007;87:918–22. Park NH, Park HJ, Park CS, et al. Herlyn-Werner-Wunderlich syndrome with unilateral hemivaginal obstruction, ipsilateral renal agenesis, and contralateral renal thin GBM disease: a case report with radiological follow up. J Korean Soc Radiol. 2010;62:383–8. Adair L II, Georgiades M, Osborne R, et al. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: common presentation of an unusual variation. J Radiol Case Rep. 2011;5:1–8. Orazi C, Lucchetti MC, Schingo PMS, et al. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol. 2007;37:657–65. Arias MP, Vellibre RM, Sanchez MM, et al. Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney. Eur J Pediatr Surg. 2005;15:441–5. Han B, Herndon C, Rosen M, et al. Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Radiol Case Rep 2010;5:327. Mesrobian HG, Rushton HG, Bulas D. Unilateral renal agenesis may result from in utero regression of multicystic renal dysplasia. J Urol. 1993;150:793–4. Hiraoka M, Tsukahara H, Ohshima Y, et al. Renal aplasia is the predominant cause of congenital solitary kidneys. Kidney Int. 2002;61:1840–4. Conflict of interest The authors report no conflicts of interest. The authors also declare that they have no financial associations to disclose in the drafting of this manuscript. Author information Authors and Affiliations Corresponding author About this article Cite this article Shimizu, M., Sakai, S., Ohta, K. et al. Uterus didelphys with obstructed hemivagina and contralateral multicystic dysplastic kidney. CEN Case Rep 4, 61–64 (2015). https://doi.org/10.1007/s13730-014-0139-9 Received: Accepted: Published: Issue date: DOI: https://doi.org/10.1007/s13730-014-0139-9

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