Diagnosis of OHVIRA syndrome in prepuberty: Is it possible?

Ruiz, Yurema González; Alvira, R. Delgado; Hinojosa, Alexander Siles; Hernández, Beatriz Izquierdo; Heras, M.A. Rihuete

doi:10.1016/j.anpede.2019.02.001

Cited by 3 publications

(2 citation statements)

References 4 publications

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“…OHVIRA syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare congenital anomaly of the female reproductive system characterised by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis or dysplasia 1–4. The presented case highlights the complexity and challenges in diagnosing this condition, especially when the patient has underlying neurodiversity that may mask the symptoms.…”

Section: Discussionmentioning

confidence: 95%

A Novel cause of abdominal pain presenting with anuria and renal failure

Corkill,

Rupret,

Scrivens

et al. 2024

BMJ Case Rep

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A girl in early adolescence with autism presented with 3 months of abdominal pain and 36 hours of anuria. She had recently received treatment for urinary tract infections, anxiety and menorrhagia (she had undergone menarche a few months earlier). Due to the pain, she had pulled out an incisor. Bladder scan showed 923 mL, creatinine was 829 mmol/L but urethral catheter insertion did not drain urine. An unenhanced CT scan revealed an absent left kidney, didelphys uterus and right-sided hydroureteronephrosis caused by haematocolpos in keeping with a diagnosis of OHVIRA syndrome and ureteric obstruction of a single kidney causing acute renal failure. She underwent vaginal septoplasty, drainage of the haematocolpos and right ureteric stent.

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Section: Discussionmentioning

confidence: 95%

A Novel cause of abdominal pain presenting with anuria and renal failure

Corkill,

Rupret,

Scrivens

et al. 2024

BMJ Case Rep

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“…Septectomy remains the treatment of choice and should be performed in all affected patients. Knowledge of this rare condition and maintaining high levels of suspicion is critical in these cases [5,6].…”

Section: Figure 1 Axial T2-weighted Mr Image Demonstrates Uterus Dide...mentioning

confidence: 99%

Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis Syndrome in Adulthood: A Diagnostic Challenge

Grammatis,

Ajibade,

Warakaulle

et al. 2023

Diagnostics

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A patient in her early 20s presented with constant and progressive lower abdominal and back pain, mainly on the right side of the abdomen, purulent vaginal discharge and pyrexia. A radiological assessment revealed a possible tubo-ovarian abscess and the incidental diagnosis of ipsilateral renal agenesis. The patient was treated for pelvic inflammatory disease (PID); however, after antibiotic administration and since the symptoms did not resolve, an abdominal MRI was requested, which revealed uterus didelphys with two cervices, an obstructed haemivagina and evidence of haematocolpos. The diagnosis of Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome was confirmed, and the patient underwent the excision of the vaginal septum, the drainage of the haematopyocolpos and the laparoscopic drainage of the tubo-ovarian abscess. She achieved a good recovery.

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OHVIRA Syndrome in Adulthood: A Diagnostic Challenge

Grammatis¹,

Ajibade²,

Warakaulle³

et al. 2023

Preprint

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A patient in her early 20s presented with constant and progressive lower abdominal and back pain, mainly on the right side of the abdomen, purulent vaginal discharge and pyrexia. Radio-logical assessment revealed a possible tuboovarian abscess and incidental diagnosis of ipsilateral renal agenesis. The patient was treated for pelvic inflammatory disease (PID), however after antibiotic administration and since the symptoms were not resolving, an abdominal MRI was requested which revealed a uterus didelphys with two cervices, an obstructed haemivagina and evidence of haematocolpos. The diagnosis of OHVIRA syndrome was confirmed and the patient underwent an excision of the vaginal septum, drainage of the haematopyocolpos and laparoscopic drainage of the tuboovarian abscess. She achieved a good recovery.

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Diagnosis of OHVIRA syndrome in prepuberty: Is it possible?

Cited by 3 publications

References 4 publications

A Novel cause of abdominal pain presenting with anuria and renal failure

A Novel cause of abdominal pain presenting with anuria and renal failure

Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis Syndrome in Adulthood: A Diagnostic Challenge

OHVIRA Syndrome in Adulthood: A Diagnostic Challenge

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