Journal of the Japanese Society of Pediatric Surgeons
Online ISSN : 2187-4247
Print ISSN : 0288-609X
ISSN-L : 0288-609X
Originals
Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in a Single Institution
Yasuo NakaharaTakafumi GotoShuichi KatayamaYu UenoKosuke HitomiKoji Aoyama
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2017 Volume 53 Issue 4 Pages 899-904

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Abstract

Purpose: While the classical type of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome includes uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, a recent series reveals several new types. The purpose of this study was to clarify the clinical features of OHVIRA syndrome in our institution.

Methods: All patients with OHVIRA syndrome were identified from 2003 to 2015 except for patients with anorectal malformation. The clinical and radiological findings were retrospectively reviewed. We also identified all female patients with renal agenesis in the same periods to estimate the frequency of OHVIRA syndrome among them.

Results: Nine patients were identified. Five patients had right side obstruction and four had left side. Three patients presented with abdominal pain during puberty. Four patients were detected by prenatal ultrasonography. One two-year-old patient was accidentally found by an imaging study of another disease. One patient was found during follow up of single kidney with vesicoureteral reflux. Four patients underwent vaginoplasty at the ages of fourteen, twelve, eleven, and six. One patient had spontaneous rupture of the obstructed hemivagina immediately after birth. Four asymptomatic patients were managed conservatively. Seven patients had renal agenesis and two patients had hypo/dysplastic kidney with ectopic ureter. Among the fifty patients with unilateral renal agenesis identified by an imaging study in the same periods, eight patients (53.3%) were diagnosed as having OHVIRA syndrome.

Conclusions: It is important to be aware of OHVIRA syndrome not only in patients with renal agenesis but also hypo/dysplastic kidney or multicystic dysplastic kidney (MCDK).

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© 2017 The Japanese Society of Pediatric Surgeons
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