Images in Gynecologic Surgery

Uterine Didelphys, Obstructed Hemivagina, and Ipsilateral Renal Agenesis: Presentation and Management Oluwamuyiwa Bolonduro, MD, MPH*, Rubin Raju, MD, Omar Abuzeid, BA, Mohammed Ashraf, MD, and Mostafa I. Abuzeid, MD From the Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, Hurley Medical Center, Flint, Michigan (Drs. Bolonduro, Raju, Ashraf, and Abuzeid), and IVF Michigan, Rochester Hills, Michigan (Drs. Ashraf and Abuzeid and Mr. Omar Abuzeid).

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A 14-year-old nulligravida with congenital absence of the left kidney came to our unit because of severe dysmenorrhea. Physical examination revealed a left-sided paravaginal bulge. Magnetic resonance imaging revealed uterine didelphys, left hematocolpos, and hematometra, which were Fig. 1 Large left-sided hematocolpos.

confirmed at laparoscopy (Fig. 1). The left paravaginal bulge was incised and drained of old menstrual effluent. The septum was excised, and the edges of the vaginal mucosa were approximated using 2-0 polyglactin 910 (Vicryl) sutures in an interrupted manner. Reevaluation with a laparoscope revealed that the hematocolpos had resolved (Fig. 2). The postoperative course was uneventful. The incidence of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is 1 in 20 000 [1]. It is thought to result from the abnormal development of the m€ullerian and wolffian ducts [2]. The condition usually is detected after puberty when menstrual blood accumulates in the obstructed side, leading to hematocolpos and pelvic pain [3]. Its rarity and variable clinical features contribute to a diagnostic delay for years after menarche [4]. Fig. 2 Resolved hematocolpos after vaginal septum excision.

Disclosures: None declared. Corresponding author: Oluwamuyiwa Bolonduro, MD, MPH, Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, Hurley Medical Center, One Hurley Plaza, Flint, MI 48503. E-mail: [email protected] Submitted June 25, 2014. Accepted for publication June 27, 2014. Available at www.sciencedirect.com and www.jmig.org 1553-4650/$ - see front matter Ó 2015 AAGL. All rights reserved. http://dx.doi.org/10.1016/j.jmig.2014.06.011

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Uterus didelphys with obstructed hemivaginas should be considered in the differential diagnosis in young female patients with renal anomalies, pelvic mass, severe dysmenorrhea, abdominal pain, and acute urinary retention [2,5]. In patients in whom the condition is diagnosed early, resection of the vaginal septum is the surgical method of choice [2,6,7]. References 1. Kimble RM, Khoo SK, Baartz D, Kimble RM. The obstructed hemivagina, ipsilateral renal anomaly, uterus didelphys triad. Aust N Z J Obstet Gynaecol. 2009;49:554–557. 2. Mandava A, Prabhakar RR, Smitha S. OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol. 2012;25:e23–e25.

Journal of Minimally Invasive Gynecology, Vol 22, No 3, March/April 2015 3. Tanaka YO, Kurosaki Y, Kobayashi T, et al. Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases. Abdom Imaging. 1998;23:437–441. 4. Varras M, Akrivis CH, Karadaglis S, Tsoukalos G, Plis Ch, Ladopoulos I. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis complicated by pyocolpos and presenting as acute abdomen 11 years after menarche: presentation of a rare case with review of literature. Clin Exp Obstet Gynecol. 2008;35:156–160. 5. Ozturk H, Dagistan E, Ozlu T. Role of OHVIRA syndrome in renal agenesis: a case report. Pediatr Urol Case Rep. 2014;1:5–11. 6. Adair L 2nd, Georgiades M, Osbourne R, Ng T. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: common presentation and an unusual variation. J Radiol Case Rep. 2011;5:1–8. 7. Mane SB, Shastri P, Dhende NP, et al. Our 10 year experience of variable M€ullerian anomalies and its management. Pediatr Surg Int. 2010;26: 795–800.

Uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis: presentation and management.

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