Acute urinary retention caused by OVHIRA syndrome

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OVHIRA) syndrome is a congenital urogenital malformation that associates a bifid uterus with a longitudinal vaginal septum, resulting in a blind hemivagina and an ipsilateral renal agenesis. The clinical presentation is highly variable, delaying diagnosis and leading to important complications. An 18-year-old woman was diagnosed with OVHIRA syndrome following acute urinary retention. An agenesis in the left-sided renal system and an enormous pelvic mass compressing the adjacent structures were revealed in the intravenous urography. Nuclear magnetic resonance was the imaging modality that provided most diagnostic information, defining the pelvic mass as a giant left hematometrocolpos contained with a longitudinal vaginal septum. Resection of the septum was performed draining all reduced hematic content and both hemiuteri communicated with a single vagina, resulting in an asymptomatic patient. OVHIRA syndrome is a little known entity that can occur atypically, which makes the diagnosis difficult and delays the treatment. It is important to maintain a high degree of clinical suspicion to avoid irreversible complications.

Wolffian Origin of Vagina Unfolds the Embryopathogenesis of OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) Syndrome and Places OHVIRA as a Female Counterpart of Zinner Syndrome in Males

“…The pain arising out of this cryptomenorrhea is cyclical to begin with but later becomes continuous. Persistence of blood products in the vaginal/uterine cavity/tubes provides a nidus for microbial growth [7,8]. Later, pyocolpos and/or tubo-ovarian mass may ensue [7,8].…”

“…Persistence of blood products in the vaginal/uterine cavity/tubes provides a nidus for microbial growth [7,8]. Later, pyocolpos and/or tubo-ovarian mass may ensue [7,8]. The patient may also present with foul-smelling mucopurulent discharge and dyspareunia.…”

Herlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal anomaly: A case report and a comprehensive review of literature

“…The original articles were selected based on features such as age, major symptom, obstructed side, ipsilateral/contralateral renal anomaly, Müllerian anomaly, types of diagnostic imaging, obstruction mechanism and treatment. Data extraction compiled 521 into a descriptive analysis [ [12] , [13] , [14] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] , [37] , [38] , [39] , [40] , [41] , [42] , [43] , [44] , [45] , [46] , [47] , [48] , [49] , [50] , [51] , [52] , [53] , [54] , [55] , [56] , [57] , [58] , [59] , [60] , [61] , [62] , [63] , [64] , [65] , [66] , [67] , [68] , [69] , [70] , [71] , [72] , [73] , [74] , [75] , [76] , [77] , [78] , [79] , …”

“…There are several reported surgical approaches, including, laparotomic, laparoscopic [125] and transvaginal approaches, which allow relief of the obstruction and the pain associated with the distension generated by retained menses. The transvaginal approach is the most commonly used and different techniques are reported, such as sharp dissection [22] , hysteroscopy with monopolar electrocautery [146] , hysteroscopy with bipolar electrocautery [40] , resectoscopy with bipolar electrocautery [46] , bipolar electrocautery [102] and CO 2 laser [136] . However, septum resection may not be possible when the vaginal septum is in a proximal position, and a hemihysterectomy may be necessary [139] .…”

Pyometra and Pregnancy with Herlyn-Werner-Wunderlich Syndrome

“…Herlyn-Werner-Wunderlich syndrome is extremely rare. To our knowledge, there are five cases of pregnancies associated to HWWS, [12][13][14][15] seven cases of HWWS with pyocolpos, [16][17][18][19][20][21][22] one case of pregnancy in HWWS with pyocolpos, 23 and two cases of pregnancy with pyocolpos in a uterus didelphys. 24,25 According to the European Society of Human Reproduction and Embryology (ESHRE) and the European Society for Gynecological Endoscopy (ESGE) 26 consensus on the Classification of Female Genital tract congenital anomalies, HWWS appears to be included in Class U3B uterine anomaly, class C2 cervix anomaly, and class V3 vaginal anomaly.…”