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. 2019 Aug;69(4):350-355.
doi: 10.1007/s13224-019-01240-4. Epub 2019 Jun 10.

Minimally Invasive Endoscopic Approach for Management of OHVIRA Syndrome

Alka Kriplani  1 , Venus Dalal  2 , Garima Kachhawa  3 , Reeta Mahey  3 , Vikas Yadav  3 , Isha Kriplani  3
Affiliations

Minimally Invasive Endoscopic Approach for Management of OHVIRA Syndrome

Alka Kriplani et al. J Obstet Gynaecol India. 2019 Aug.

Abstract

Background: Herlyn-Werner-Wunderlich syndrome is an uncommon entity characterized by uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis (also called OHVIRA syndrome). Due to rarity and varied presentations, often correct diagnosis is missed out during work up, leading to management problems. We describe our dependence on detailed preoperative work up and minimally invasive endoscopic approach in management of the eight patients of OHVIRA syndrome.

Methods: In this retrospective case series study, eight patients of OHVIRA syndrome were managed from January 2012 to March 2018 with the help of improved imaging facility and diagnostic work up. Precise diagnosis helped in adopting minimally invasive approach in management. Patients were reviewed, focusing on presentation, radiologic findings and surgical management.

Results: Median age at diagnosis was 19 years (range 13-41 years). Abdominal pain and dysmenorrhea were the main presenting complaint. All patients except one had associated ipsilateral renal agenesis. Surgical excision of the obstructed hemivaginal septum and hematometra drainage was the main treatment. In seven patients, vaginoscopic resection of vaginal septum was done with resectoscope except one 41-year-old patient, where resection of vaginal septum was performed laparoscopically along with hysterectomy.

Conclusion: Vaginoscopic resection of obstructed hemivaginal septum is an effective method. Management has shifted to minimally invasive approach due to improved imaging, precise preoperative diagnosis and proper understanding of the disease.

Keywords: Hematometra; Laparoscopy; Minimally invasive; OHVIRA syndrome; Vaginoscopy.

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Conflict of interest statement

Conflict of interestThe authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
a MRI showing left hematocolpos and hematometra and right uterine horn (Hm hematometra, Hc hematocolpos, U normal uterine horn, B bladder). b MRI showing left hematocolpos and hematometra (Hm hematometra, Hc hematocolpos, B bladder)
Fig. 2
Fig. 2
a Laparoscopic view showing left hematocolpos, left hematometra and normal right uterine horn (Lt. Ut. left uterine horn with hematometra, Lt. V left obstructed vagina with hematocolpos, Rt. Ut right normal uterine horn). b Laparoscopic view post drainage of left hematometra and hematocolpos after vaginoscopic septum resection (Lt. Ut. left uterine horn, Lt. V left vagina, Rt. Ut right normal uterine horn)
Fig. 3
Fig. 3
a Vaginoscopic view showing drainage of left hematocolpos following left hemivaginal septum resection (Rt Cx right cervix). b vaginoscopic view showing resected left hemivaginal septum both cervical opening (Rt Cx right cervix, Lt Cx left cervix, S resected left hemivaginal septum)
Fig. 4
Fig. 4
a Laparoscopic view of uterine didelphys with left obstructed hemivagina with left hematocolpos (Lt. V. Hc left hemivaginal hematocolpos, Lt. U left uterine horn, Rt. U right uterine horn). b laparoscopic view of left hemivaginal septum with patent right hemivagina after drainage of hematocolpos laparoscopically. (Lt. V Sp left hemivaginal septum)
See this image and copyright information in PMC

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