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

 Jang Hee Han  ;  Yong Seung Lee  ;  Young Jae Im  ;  Sang Woon Kim  ;  Mi-Jung Lee  ;  Sang Won Han 
 PLOS ONE, Vol.11(11) : e166776, 2016 
Journal Title
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PURPOSE: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by M?llerian 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 METHODS: We 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.

RESULTS: Median 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.

CONCLUSIONS: While 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.
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Urology (비뇨의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Sang Woon(김상운) ORCID logo https://orcid.org/0000-0002-5790-1948
Lee, Mi-Jung(이미정) ORCID logo https://orcid.org/0000-0003-3244-9171
Lee, Yong Seung(이용승) ORCID logo https://orcid.org/0000-0003-3778-9888
Im, Young Jae(임영재)
Han, Sang Won(한상원) ORCID logo https://orcid.org/0000-0003-0941-1300
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