title: Clinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group.
abstract: OBJECTIVE
  Obstructed 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.
  
  
  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.
authors.affiliation: Department of Urology and Urological Science Institute, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jang Hee
authors.initials: JH
authors.lastname: Han
authors.affiliation: Department of Urology and Urological Science Institute, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Yong Seung
authors.initials: YS
authors.lastname: Lee
authors.affiliation: Department of Urology, College of Medicine, Seoul National University, Seoul, Korea.
authors.forename: Young Jae
authors.initials: YJ
authors.lastname: Im
authors.affiliation: Department of Urology and Urological Science Institute, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Sang Woon
authors.initials: SW
authors.lastname: Kim
authors.affiliation: Department of Radiology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Mi-Jung
authors.initials: MJ
authors.lastname: Lee
authors.affiliation: Department of Urology and Urological Science Institute, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Sang Won
authors.initials: SW
authors.lastname: Han
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000601:surgery*; D000367:Age Factors; D002675:Child, Preschool; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities*; D010641:Phenotype; D012189:Retrospective Studies; D012741:Sexual Maturation; D013577:Syndrome; D016896:Treatment Outcome; D014463:Ultrasonography; D014621:Vagina / Q000002:abnormalities*