Rare Case of OHVIRA with Ectopic Inguinal Hemiuterus and Ovary

Abstract

A 12-year-old girl presented with inguinal swelling and recurrent groin pain since menarche. Ultrasound showed an inguinally located ovary with normal perfusion. Herniorrhaphy revealed an ectopic inguinal left ovary with fallopian tube and atretic hemiuterus and a closed internal inguinal ring. Laparoscopy revealed a right-sided hemiuterus and vaginally palpable cervix, leading to the diagnosis of ectopic OHVIRA syndrome type 1.2. The left hemiuterus was resected and the left ovary was pulled through the inguinal canal into the abdomen. During 12 months of follow-up, the left ovary showed normal perfusion and sonomorphologic appearance, menstrual periods were uneventful.

Keywords

OHVIRA syndrome - hemiuterus - hemivagina - vaginal obstruction

• References

• 1 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 (04) 340-347 Das gemeinsame Vorkommen von effener Gartner-Gang-Zyste, gleichseitiger Nierenaplasie und Uterusdoppelmissbildung als typisches Missbildungssyndrom.
• 2 Orvis GD, Behringer RR. Cellular mechanisms of Müllerian duct formation in the mouse. Dev Biol 2007; 306 (02) 493-504
• 3 Zhu L, Chen N, Tong JL, Wang W, Zhang L, Lang JH. New classification of Herlyn-Werner-Wunderlich syndrome. Chin Med J (Engl) 2015; 128 (02) 222-225
• 4 Mooren ERM, Cleypool CGJ, de Kort LMO, Goverde AJ, Dik P. A retrospective analysis of female Müllerian duct anomalies in association with congenital renal abnormalities. J Pediatr Adolesc Gynecol 2021; 34 (05) 681-685
• 5 Demir MK, Savas Y, Furuncuoglu Y. et al. Imaging findings of the unusual presentations, associations and clinical mimics of acute appendicitis. Eurasian J Med 2017; 49 (03) 198-203
• 6 Saini R, Bains L, Kaur T. et al. Ovarian inguinal hernia - a possibility in MURCS syndrome. J Ovarian Res 2021; 14 (01) 114
• 7 Kudela G, Wiernik A, Drosdzol-Cop A. et al. Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome - one clinical center case series and the systematic review of 734 cases. J Pediatr Urol 2021; 17 (05) 653.e1-653.e9
• 8 Liu Y, Li Z, Dou Y, Wang J, Li Y. Anatomical variations, treatment and outcomes of Herlyn-Werner-Wunderlich syndrome: a literature review of 1673 cases. Arch Gynecol Obstet 2023; 308 (05) 1409-1417
• 9 Bazi T, Berjawi G, Seoud M. Inguinal ovaries associated with Müllerian agenesis: case report and review. Fertil Steril 2006; 85 (05) 1510.e5-1510.e8
• 10 Chaudhry SR, Chaudhry K. Anatomy, Abdomen and Pelvis: Uterus Round Ligament. StatPearls; 2023
• 11 Adham IM, Steding G, Thamm T. et al. The overexpression of the insl3 in female mice causes descent of the ovaries. Mol Endocrinol 2002; 16 (02) 244-252
• 12 Candiani M, Ruffolo AF, Fedele F, Salvatore S, Parazzini F. Conservative treatment of didelphyc uterus with obstructed hemivagina and ipsilateral renal agenesis. J Minim Invasive Gynecol 2022; 29 (08) 929
• 13 Erkkinen E, Farahzad MM, O'Brien KE, Rosen MW. Pregnancy outcomes in patients with obstructed hemivagina ipsilateral renal agenesis. J Pediatr Adolesc Gynecol 2023; 36 (06) 545-548