Three Cases of Didelphys Uterus, Including Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA Syndrome) AKA Herlyn-Werner-Wunderlich Syndrome), with a Systematic Review

Introduction: Uterus didelphys with obstructed hemivagina associated with ipsilateral renal agenesis (OHVIRA syndrome) is a rare female urogenital malformation and delay in its diagnosis could lead to several complications. Case presentation: A 21-year-old virgin woman was admitted to the emergency department (ED) with severe abdominal pain, without fever and vaginal discharge. She reported a history of cyclic abdominopelvic pain and dysmenorrhea for 5 years. The primary diagnosis (OHVIRA syndrome) was made using ultrasonography, spiral computed tomography (CT) and magnetic resonance imaging (MRI). In addition, laparoscopy was performed to confirm diagnosis and drain hematosalpinx. Then, hysteroscopy was carried out for septum resection and catheter insertion. At one-month follow-up the ultrasonography showed normal left hemicavity of uterus associated with significant decrease in dysmenorrhea. Conclusion: Being aware of OHVIRA syndrome and clinical suspicion of this rare anomaly are essential for making a timely diagnosis, preventing complications, relieving symptoms, and preserving future fertility.

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HERLYN–WERNER–WUNDERLICH SYNDROME: CLINICAL CASE OF A 17 YEAR OLD PATIENT

PEDIATRIA Journal named after G N SPERANSKY ◽

10.24110/0031-403x-2021-100-4-185-189 ◽

2021 ◽

Vol 100 (4) ◽

pp. 185-189

Author(s):

S.S. Nikitin ◽

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N.B. Guseva ◽

M.E. Ukvalberg ◽

N.A. Ivanova ◽

...

Keyword(s):

Clinical Practice ◽

Reproductive Health ◽

Early Diagnosis ◽

Clinical Case ◽

Renal Agenesis ◽

Uterus Didelphys ◽

Mullerian Ducts ◽

Level 3 ◽

Ohvira Syndrome ◽

Obstructed Hemivagina

Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare syndrome in clinical practice in which there is a combination of complete uterus didelphys and OHVIRA syndrome (obstructed hemivagina and ipsilateral renal agenesis). HWWS refers to associative defects of the reproductive and urinary systems and occurs due to anomalies in the development of the Müllerian ducts. The review part of the article presents questions of its epidemiology, and provides historical information. The clinical picture, methods of examination and treatment are considered. Particular attention is paid to the difficulty of diagnosing HWWS and the need for its early diagnosis in order to preserve the reproductive health of girls. A clinical case of a 17-year-old patient with HWWS diagnosed during urgent laparoscopy, the complication of which was hematopiocolpos, is presented. The patient was referred to a level 3 hospital for specialized medical care, where she was operated on.

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Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA Syndrome)

JBRA Assisted Reproduction ◽

10.5935/1518-0557.20150049 ◽

2015 ◽

Vol 19 (4) ◽

Cited By ~ 2

Author(s):

Mauri José Piazza ◽

Newton Sergio De Carvalho ◽

Ana Paula Lisboa Peixoto ◽

Almir Antonio Urbanetz

Keyword(s):

Renal Agenesis ◽

Uterus Didelphys ◽

Ohvira Syndrome ◽

Obstructed Hemivagina

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Management of Hematometra in Single Horn of Didelphic Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome: A Case Report with Brief Review of Literature

Journal of SAFOG with DVD ◽

10.5005/jp-journals-10006-1423 ◽

2016 ◽

Vol 8 (3) ◽

pp. 231-235

Author(s):

Ritu Agarwal

Keyword(s):

Case Report ◽

South Asian ◽

Renal Agenesis ◽

Rare Entity ◽

Review Of Literature ◽

Resonance Imaging ◽

Uterus Didelphys ◽

Future Fertility ◽

Ohvira Syndrome ◽

Obstructed Hemivagina

ABSTRACT The triad of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare entity. These cases are difficult to diagnose due to heterogeneous presentation and rarity of the condition. Ultrasonography and magnetic resonance imaging are important for initial diagnosis; however, laparoscopy is required for the confirmation of the diagnosis. Early and accurate diagnosis of this condition followed by prompt surgical therapy is essential to prevent complications and to preserve future fertility. Here we report a case of uterus didelphys with hemivagina and ipsilateral renal agenesis with hematometra and hematosalpinx in a young girl. How to cite this article Mishra VV, Agarwal R, Bandwal P, Choudhary S, Gandhi K. Management of Hematometra in Single Horn of Didelphic Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome: A Case Report with Brief Review of Literature. J South Asian Feder Obst Gynae 2016;8(3):231-235.

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