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

Abstract

SummaryBackgroundThe classical theory of Müllerian origin of upper vagina fails to explain complex urogenital malformations like OHVIRA syndrome; the Acien’s hypothesis, however, unravels the hidden embryopathogenesis. As per Acien, Wolffian (mesonephric) ducts instead of Müllerian ducts and sinovaginal bulbs, give rise to the vagina. The new hypothesis, however, retains the concept of origin of the ureters (with ureters inducing renal development) by the former and the uterus by Müllerian ducts. Thus, a failure of development of mesonephros/mesonephric duct gives rise to absent ureters and hence absent homolateral kidney; blind ending (obstructed) ipsilateral hemivagina and cessation of support to paramesonephric ducts which leads to unfused uterus (uterus didelphys). Hence, the new hypothesis explains all components of OHVIRA syndrome. On a parallel track, unilateral anomalous development of the mesonephros in males causes atresia of the homolateral ejaculatory duct that results in obstruction of the proximally placed seminal vesicle. Besides, there is absence of the ipsilateral kidney (Zinner syndrome).Case ReportIn this manuscript, we describe four cases of OHVIRA syndrome. Case 1 was a 34-year-old nulligravida, married since fourteen years, who presented with a 5-month history of pelvic inflammatory disease and dyspareunia. Regular menses in the patient and azoospermia in her husband delayed the diagnosis. Case 2 was a 14-year-old girl who presented with dysmenorrhea and lower abdominal pain since a few months. Case 3 was a 27-year-old female who presented with infertility and dysmenorrhea. Case 4 was a 15-year-old female who presented with a one-year history of dysmenorrhea and cyclic pelvic pain. In all cases, one of the uterine horns revealed collection due to a hemivaginal septum and an absent ipsilateral kidney; thus, establishing the diagnosis of OHVIRA syndrome. The case 4 additionally revealed homolateral vaginal agenesis.ConclusionsOn the basis of our 4 cases, we support the Acien’s hypothesis of Wolffian origin of vagina which explains the development of OHVIRA syndrome. Besides, we emphasize the need to suspect this syndrome in a female with a pelvic mass and absence of homolateral kidney. Finally, we believe that OHVIRA due to its Wolffian origin is a female equivalent of Zinner syndrome in males. Therefore, we propose OSVIRA (Obstructed Seminal Vesicle and Ipsilateral Renal Agenesis) as an acronym for Zinner syndrome analogous to OHVIRA.

Congenital vesicouterine fistulas—A PRISMA‐compliant systematic review

“…Combined, this data prompts one to consider that renal agenesis is not an essential condition for the development of a congenital VUF, but rather an event accompanying vaginal atresia in select cases. From the Aciéns' and other later works, 36,41,42 failed interaction of the Müllerian tubercle with the Wolffian ducts would be then responsible. In turn, a lesson for the clinician is that assessment for the presence of the kidneys is highly recommended in cases of vaginal atresia and VUF.…”

Novel approach to locating a hypoplastic kidney in a unique variant of uterine didelphys syndrome presenting with continuous incontinence

“…The incidence of Mullerian tract anomalies is approximately 2%–3% in the general populace,10 11 and of these an estimated 0.16%–10% represent OHVIRA syndrome 11. This syndrome is characterised by uterus didelphis and ipsilateral renal tract anomalies with an obstructed hemivagina.…”

Prenatal Evaluation and Postnatal Follow-Up of Ureteral Ectopic Insertion in Multicystic Dysplastic Kidneys

“…This is related to the very small size of the ureters and the poor contrast resolution of prenatal US. Therefore, high-frequency linear probes and a perineal approach should be used [6]. Fetal MRI has a better contrast resolution and depicted the ectopic ureteral insertion in 2 cases (Fig.…”

“…In girls, ectopic insertion into a blind hemivagina does not require surgery during infancy and childhood. Surgical excision of the hemivaginal septum is performed at adolescence after menarche [6,26], most patients remaining asymptomatic. Recurrent abdominal pain and pelvic mass require early septum resection.…”

“…The association between ureteral ectopic insertion and renal dysplasia is probably linked to a common developmental anomaly. Moreover, the association between ureteral ectopic insertion and genital anomalies is well known, either in the context of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome in girls [4,5] or Zinner syndrome in boys [6,7].…”