Clinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group

Abstract

PurposeObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by Müllerian duct and renal anomalies. It is usually regarded as a disease of adolescence; however, due to a number of possible problems, the management of patients before puberty should not be overlooked. We assessed the clinical course of prepubertal patients to propose appropriate management.Materials and MethodsWe retrospectively assessed 43 prepubertal OHVIRA syndrome patients who were diagnosed and followed up at our institution from July 2004 to June 2015. We reviewed medical records, focusing on presentation, radiologic findings, surgical management, and the overall clinical course.ResultsMedian age at diagnosis was 1.3 months and median follow-up period was 25.5 months. The most common accompanying ipsilateral urologic anomalies were ectopic ureter and ureterocele, while the most common contralateral anomaly was vesicoureteral reflux. During the follow-up period, six patients (14.0%) required surgery at a median age of 31.2 months due to recurrent urinary tract infection, uncontrolled vaginal distention compressing adjacent organs, urinary incontinence, or intractable abdominal pain.ConclusionsWhile OHVIRA syndrome is known as a postpubertal disease, about 13% of prepubertal patients in our study required surgery. When ectopic ureter insertion into the vagina is present, further treatment may be needed to address the complications caused by continuous urine production. Patients should be monitored for complications arising from either obstructed hemivagina or renal anomalies with regular follow-up, especially before the age of five years.

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

“…The functional value of both kidneys needs careful assessment, as 15% of contralateral kidneys have urologic anomalies [26]. In boys, ectopic ureteral insertion into the seminal vesicles may lead to recurrent epididymitis or dysuria and possible subsequent oligozoospermia.…”

“…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.…”

Atypical presentation of obstructed hemivagina and ipsilateral renal anomaly

“… 1 Approximately 2% of female patients with unilateral renal agenesis or multicystic dysplastic kidney will have OHVIRA syndrome. 2 …”

Herlyn–Werner–Wunderlich syndrome in a multiparous female

“…BJR|case reports Case Report: HWW syndrome in a multiparous female: A case report DISCUSSION Herlyn-Werner-Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract that involves abnormal development of the Mullerian and mesonephric ducts in the female embryos. [2][3][4] It is also known as OHVIRA syndrome (Obstructed hemivagina and Ipsilateral Renal agenesis). Its estimated occurrence is 0.1-3.8% with only a few reported cases occurring during pregnancy.…”