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

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Purpose: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by MuÈ 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.

Original languageEnglish
Article numbere0166776
JournalPloS one
Volume11
Issue number11
DOIs
Publication statusPublished - 2016 Nov

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Age Groups
kidneys
Kidney
Surgery
ureter
Ureter
disease course
Ducts
surgery
Ureterocele
urinary tract diseases
Intractable Pain
Vesico-Ureteral Reflux
vagina
Urinary Incontinence
Vagina
puberty
Puberty
Urinary Tract Infections
Abdominal Pain

All Science Journal Classification (ASJC) codes

  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

Cite this

@article{646a2352e54a4e678c151a7d0f74d93a,
title = "Clinical implications of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome in the prepubertal age group",
abstract = "Purpose: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by Mu{\`E} 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.",
author = "Han, {Jang Hee} and Lee, {Yong Seung} and Im, {Young Jae} and Kim, {Sang Woon} and Lee, {Mi Jung} and Han, {Sang Won}",
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Clinical implications of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome in the prepubertal age group. / Han, Jang Hee; Lee, Yong Seung; Im, Young Jae; Kim, Sang Woon; Lee, Mi Jung; Han, Sang Won.

In: PloS one, Vol. 11, No. 11, e0166776, 11.2016.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Clinical implications of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome in the prepubertal age group

AU - Han, Jang Hee

AU - Lee, Yong Seung

AU - Im, Young Jae

AU - Kim, Sang Woon

AU - Lee, Mi Jung

AU - Han, Sang Won

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N2 - Purpose: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by MuÈ 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.

AB - Purpose: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by MuÈ 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.

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