Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: A case report

El-Gohary, Mohamed Amin; Naik, Rushikesh; Elkafafi, Mohamed; Hamed, Hussein; Ali, Yousef

doi:10.1016/j.epsc.2023.102662

Cited by 3 publications

(1 citation statement)

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“…Se recomienda en el futuro asesoramiento preconcepcional y controles prenatales estrechos, siendo esenciales para la detección temprana y manejo oportuno de estas complicaciones obstétricas potenciales. (7) Según Candiani et al, (9) mayor del 85 % de las pacientes que buscaron embarazo tuvieron éxito, como es el caso de Reis et al (10) Casos clínicos similares fueron reportados en otros países es el caso de Kueppers et al, (6) se utilizaron la clasificación de VCUAM y la clasificación especifica elaborado por Zhu et al, siendo un caso "Clasificación 1" "Subclase 1.1", por presentación de clínica aguda, evitando las complicaciones que estas pueden evolucionar rápidamente; con "Clasificación 2" existe la presentación clínica postpubertad, presentándose años después de la menarquia. (6) Debemos considerar diagnósticos diferenciales en los neonatos, como la rabdomiosarca embrionaria (sarcoma botryoides), prolapsos ureterales quistes himenales o vaginales (Gartner), ureterocele ectópicos prolapsados, hydometrocolpos.…”

Section: Discussionunclassified

OHVIRA syndrome: report of a case in Bolivia

Rivero Castedo,

Auza-Santivañez,

Jimenez-Fernandez

et al. 2024

Interamerican Journal of Health Sciences

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Introduction. The OHVIRA Syndrome was described by Herlyn Werner Wunderlich and in 1976 Wunderlich described a grouping of right renal aplasia with bicornuate uterus and simple vagina in the presence of an isolated hematocervix, as a characteristic triad uterus didelphys, intercepted hemivagina and ipsilateral renal anomaly, it is generally performed The diagnosis occurs in puberty at the beginning of menarche, with manifest symptoms of progressive dysmenorrhea and non-specific abdominal pain in the hypochondrium; urinary retention, urinary infection or a pelvic mass usually appear.Clinical case. The case of a 13-year-old patient is presented, who comes to the clinic with abdominal pain and transvaginal bleeding. Imaging studies are performed and due to the characteristics of said studies, the presence of OHVIRA Syndrome is suspected.Discussion. Once the imaging studies were performed and the diagnosis confirmed, a surgical procedure was performed under general anesthesia. Through exploratory laparotomy, a bicornuate uterus was visualized, the right uterus larger than the left, and adherence to the abdominal wall, so an open intervention was decided.Conclusions. OHVIRA syndrome coexists with a rare malformation and is often misdiagnosed as other more common etiologies of dysmenorrhea in adolescents, as a consequence it delays a correct and early diagnosis, increasing the risk of kidney damage and its complications. In our clinical case, a timely diagnosis was made and surgical treatment was subsequently planned with a favorable outcome.

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Section: Discussionunclassified

OHVIRA syndrome: report of a case in Bolivia

Rivero Castedo,

Auza-Santivañez,

Jimenez-Fernandez

et al. 2024

Interamerican Journal of Health Sciences

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A rare case of obstructed hemivagina with uterus didelphys and ipsilateral renal anomaly syndrome

AlBaik,

Ibrahim,

AbuKaresh

et al. 2024

SAGE Open Medical Case Reports

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Obstructed hemivagina and ipsilateral renal anomaly syndrome, also known as Herlyn–Werner–Wunderlich syndrome, represents a rare congenital anomaly characterized by the presence of an obstructed hemivagina with uterus didelphys and concomitant ipsilateral renal abnormalities. Typically, the clinical presentation includes cyclical abdominal pain, vaginal discharge, and/or a vaginal mass occurring post-menarche. Accurate diagnosis requires a high index of suspicion among clinicians, coupled with a comprehensive understanding of the distinctive features associated with this anomaly. Herein, we present the case of a 13-year-old female patient who complained of persistent lower abdominal pain. Magnetic resonance imaging confirmed the diagnosis of obstructed hemivagina and ipsilateral renal anomaly syndrome, and the patient was successfully treated with a laparotomy involving blood aspiration and hysterectomy. This case report highlights the significance of clinical awareness, prompt diagnosis, and timely therapeutic interventions to mitigate the adverse effects and optimize outcomes in individuals affected by obstructed hemivagina and ipsilateral renal anomaly syndrome.

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Congenital Abnormalities Causing Hematocolpos: A Pictorial Essay

Dorthu,

Danthine

2024

Journal of the Belgian Society of Radiology

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Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: A case report

Cited by 3 publications

References 10 publications

OHVIRA syndrome: report of a case in Bolivia

OHVIRA syndrome: report of a case in Bolivia

A rare case of obstructed hemivagina with uterus didelphys and ipsilateral renal anomaly syndrome

Congenital Abnormalities Causing Hematocolpos: A Pictorial Essay

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