[Diagnosis of OHVIRA syndrome in prepuberty: Is it possible?]

Abstract

Obstructed hemivagina with uterus didelphys and ipsilateral renal anomaly (OHVIRA) syndrome, formerly known as Herlyn--Werner--Wunderlich syndrome, is a rare congenital malformation that involves abnormal development of the Müllerian and Wolffian ducts during the development of genitalia in female embryos.1,2 It amounts to 0.16%--10% of all cases of Müllerian malformations.3 The most frequent presentation is an abdominal mass secondary to hematocolpos during adolescence associated with pain and dysmenorrhoea, and diagnosis before puberty is rare. We present 2 cases of OHVIRA syndrome diagnosed prepubertally. Case 1: girl aged 1 year managed in the nephrology department due to left renal agenesis with a vulvar cyst that became progressively larger. The patient underwent surgical intervention, with incision and full drainage of the cyst, which released a yellowish fluid, and resection of a posterior wall that appeared to form a hemivagina. The patient remained asymptomatic after surgery and was referred at age 13 years due to urinary incontinence and detection by abdominal ultrasound of a bicornuate bicollis uterus with otherwise normal morphology and structure (Fig. 1). The external genitalia appeared normal in the physical examination. A magnetic resonance imaging (MRI) scan revealed compensatory renal enlargement in the right side and features suggestive of a bicornuate uterus (Fig. 1). These radiological findings led to performance of a diagnostic cystoscopy and vaginoscopy. The cystoscopy revealed a smooth-walled bladder with a right ureteral orifice. A left ureteral meatus was not visualised either in the bladder or ectopically in the urethra. The vaginoscopy revealed a normal vagina in the distal two thirds and a vaginal septum in the proximal third associated with a double cervix. Case 2: girl aged 1 year managed in the nephrology department due to left renal atrophy. A follow-up ultra-