OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature

Uğurlucan, Funda Güngör; Baştu, Ercan; Gülşen, Gökçe; Eken, Meryem; Akhan, Süleyman Engin

doi:10.1016/j.clinimag.2013.12.011

Cited by 40 publications

(40 citation statements)

References 15 publications

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“…OHVIRA syndrome, formerly known as Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare entity characterized by the triad of uterus didelphys, obstructive hemivagina, and ipsilateral renal agenesis. [5][6][7]10,11,[22][23][24][25] There have been also few reports of varied uterine anatomy, such as a septate uteri, a unicornuate uterus with noncommunicating horn, or a unicornuate uterus without horn. [5][6][7] The standard surgery is defined as septectomy, or marsupialization of the blind hemivagina.…”

Section: Discussionmentioning

confidence: 99%

Approaches to Female Congenital Genital Tract Anomalies and Complications

İnce¹,

Oğuzkurt²,

Ezer³

et al. 2017

International Surgery

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Objective: Female congenital genital tract anomalies may appear with quite confusing and deceptive complications. This study aims to evaluate the difficulties in diagnosis and treatment of female congenital genital tract anomalies that frequently present with complications.Summary: During a 10-year period, we evaluated 20 female patients with congenital genital tract anomalies aged between 3 days and 16 years. All patients were retrospectively analyzed in terms of the results of diagnostic studies, surgical intervention, and treatment.Methods: Ultrasonography and magnetic resonance imaging revealed hydromucocolpos or hematocolpometra, imperforate hymen, distal vaginal atresia, didelphys uterus, an obstructed right hemivagina, uterovaginal atresia, a unicornuate uterus with a noncommunicating rudimentary horn, a vesicovaginal fistula, a utero-rectal fistula, intraabdominal collection, and a vaginal calculus.Results: Two patients had Mayer-Rokitansky-Küster-Hauser syndrome and 6 patients had obstructed hemivagina and ipsilateral renal anomaly syndrome. Definitive surgical interventions were hymenotomy, vaginal pull-through, vaginovaginostomy, and vesicovaginal fistula repair using a transvesical approach. In conclusion, female congenital genital tract anomalies may appear with a wide range of complications.Conclusions: There is a potential to do significant harm, if the patient's anatomic problems are not understood using detailed imaging. Revealing the anatomy completely and defining the complications that have already developed are critical to tailor the optimal treatment strategies and surgical approaches.

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Section: Discussionmentioning

confidence: 99%

Approaches to Female Congenital Genital Tract Anomalies and Complications

İnce¹,

Oğuzkurt²,

Ezer³

et al. 2017

International Surgery

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show abstract

“…Symptom relief of dysmenorrhoea with non‐steroidal anti‐inflammatory and oral contraceptive medication may also lead to a delay in diagnosis 9 . The clinical presentation may be variable and non‐specific features such as acute abdominal, 4 , 10 pelvic 9 , 11 or vaginal 12 pain, dysuria, 13 urinary retention, 7 vaginal discharge 14 , 15 and infertility 16 have been reported. The two cases presented were both known to have an absent kidney and this should prompt investigation for Mullerian duct anomalies and vice versa.…”

Section: Discussionmentioning

confidence: 99%

“…, 9 , 10 , 17 , 20 This may be performed as a single‐stage or two‐stage procedure. The single stage procedure involves excising the vaginal septum completely and marsupialising the vaginal cuff.…”

Section: Discussionmentioning

confidence: 99%

Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases

Mishra

2014

Australas J Ultrason Med

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Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare urogenital anomaly. Patients typically present at puberty, shortly after menarche with increasing pelvic pain, dysmenorrhea and pelvic mass. There may be a known history of unilateral renal agenesis. Diagnosis can usually be established by ultrasound or magnetic resonance imaging. Resection of the vaginal septum is the treatment of choice. Early diagnosis and treatment can relieve symptoms, prevent complications and preserve fertility. We present two cases of OHVIRA syndrome diagnosed by ultrasound to promote recognition of this rare but important condition.

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“…The Wolffian ducts induce the normal development of the Müllerian ducts in addition to giving origin to the kidneys. Abnormal development of the Wolffian ducts leads to the unilateral renal agenesis and imperforated hemivagina associated with OHVIRA syndrome [2]. …”

Section: Introductionmentioning

confidence: 99%

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

Han

Lee

et al. 2016

PLoS ONE

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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.

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OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature

Cited by 40 publications

References 15 publications

Approaches to Female Congenital Genital Tract Anomalies and Complications

Approaches to Female Congenital Genital Tract Anomalies and Complications

Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases

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

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