scholarly journals OHVIRA Syndrome: A Case Report

2020 ◽  
Vol 14 (1) ◽  
pp. 54-56
Author(s):  
Dilruba Zeba ◽  
Fahmida Zesmin ◽  
Rajib Roy
Keyword(s):  
Renal Agenesis ◽  
Lower Abdominal Pain ◽  
Vaginal Septum ◽  
Regular Cycle ◽  
Mullerian Ducts ◽  
Ohvira Syndrome ◽  
Obstructed Hemivagina ◽  
Lateral Fusion ◽  
Acute Retention

OHVIRA syndrome or Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of female urogenital tract which is represented by the triad of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. This rare variant of Müllerian duct anomalies represent failure of vertical and the lateral fusion of Müllerian ducts around 9 weeks of gestation. OHVIRA syndrome comprises about 2-3% of Müllerian abnormalities. A 13 years old girl got admission in FMCH on 15.3.2018 with lower abdominal pain and acute retention of urine. Her menarche was established 6 months back with regular cycle and associated with dysmenorrhoea. Imaging studies revealed uterine didelphys, highly dilated cervix with hematocolpos and absence of right kidney. The vaginal septum was excised. The patient recovered fully and was under follow up for 6 months. There was no evidence of further outflow tract obstruction. Faridpur Med. Coll. J. Jan 2019;14(1): 54-56

Abdominopelvic Pain in Patient with Uterus Didelphys and Unilateral Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA Syndrome)

2021 ◽  
Author(s):  
Zahra Tavoli ◽  
Ali Montazeri
Keyword(s):  
Renal Agenesis ◽  
Primary Diagnosis ◽  
Case Presentation ◽  
Uterus Didelphys ◽  
Future Fertility ◽  
History Of ◽  
Ohvira Syndrome ◽  
Obstructed Hemivagina ◽  
Magnetic Resonance Imaging Mri

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.

scholarly journals Vaginoscopic Incision of Oblique Vaginal Septum in Adolescents with OHVIRA Syndrome

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Chunxia Cheng ◽  
Jigyasa Subedi ◽  
Aiqian Zhang ◽  
Grace Johnson ◽  
Xingping Zhao ◽  
...  
Keyword(s):  
Postoperative Pain ◽  
Minimally Invasive ◽  
Renal Agenesis ◽  
Intraoperative Complications ◽  
Vaginal Wall ◽  
Efficacy And Safety ◽  
Vaginal Septum ◽  
Vaginal Stenosis ◽  
Ohvira Syndrome

AbstractThis study is to evaluate the efficacy and safety of vaginoscopic incision of oblique vaginal septum in adolescents with Obstructed hemi-vagina and ipsilateral renal agenesis (OHVIRA) syndrome. It is about Fourteen adolescents with OHVIRA syndrome managed by vaginoscopic incision of the oblique vaginal septum using a “No-touch” technique over an 8-year period. In all fourteen adolescents with OHVIRA the oblique vaginal septum was incised successfully without any intraoperative complications. Postoperative pain was unremarkable and each patient’s symptoms resolved postoperatively. The 3-month postoperative follow up office vaginoscopy revealed that the vaginal septum had not reformed nor was any vaginal stenosis noted. Vaginoscopic incision of the oblique vaginal septum using a “No-Touch” technique is a safe, minimally invasive, and effective approach for treating OHVIRA syndrome in adolescents with hematocolpos. This technique may be utilized to minimize disruption to the undeveloped vaginal wall and postoperative pain while providing excellent surgical visualization throughout the procedure.

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

2021 ◽  
Vol 100 (4) ◽  
pp. 185-189
Author(s):  
S.S. Nikitin ◽  
◽  
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.

Female counterpart of the Zinner syndrome in men: decoding the ‘OHVIRA’ syndrome

2021 ◽  
Vol 14 (2) ◽  
pp. e239625
Author(s):  
Vikas Kumar Panwar ◽  
Tushar Aditya Narain ◽  
Ankur Mittal ◽  
Harkirat Singh Talwar
Keyword(s):  
Pelvic Pain ◽  
Renal Agenesis ◽  
Surgical Intervention ◽  
Developmental Anomaly ◽  
Male Factor ◽  
Vaginal Septum ◽  
Uterine Anomalies ◽  
Ohvira Syndrome ◽  
Obstructed Hemivagina ◽  
Female Counterpart

The obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, a rare Mullerian duct anomaly, is a triad of uterine anomalies with ipsilateral renal agenesis and obstructed hemivagina. The aetiopathogenesis of this developmental anomaly is debatable, with several theories being postulated to explain its occurrence. We report two cases of this rare syndrome which were detected on imaging done for unrelated reasons. Case 1 is a 24-year-old woman who presented with primary infertility. After an incidental detection of the syndrome and ruling out a male factor, she has been planned for excision of the vaginal septum. Case 2 is a 47-year-old woman with OHVIRA detected during the evaluation of urolithiasis and is asymptomatic for the syndrome. The OHVIRA syndrome can be asymptomatic or may present as haematocolpos, pelvic pain and infertility which requires surgical intervention, after which successful pregnancies have been reported.

scholarly journals Herlyn–Werner–Wunderlich syndrome in a multiparous female

2020 ◽  
pp. 20200132
Author(s):  
HarSumeet Singh Sidhu ◽  
Prateek Kumar Madaan
Keyword(s):  
Renal Agenesis ◽  
Single Case ◽  
Correct Diagnosis ◽  
Imaging Features ◽  
Atypical Presentation ◽  
Single Case Report ◽  
Mullerian Ducts ◽  
Ohvira Syndrome ◽  
Obstructed Hemivagina ◽  
Multiparous Female

Herlyn–Werner–Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agenesis). It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Patients usually present after menarche with pelvic pain, dysmenorrhea, mass, and rarely with primary infertility in later years. We report a case of a multiparous female who presented to the hospital with pain in lower abdomen for the past 2 months and acute retention of urine for 1 day. This is an atypical presentation in a multiparous female that has been described in a single case report so far. 1 Intravenous pyelogram and MRI of the patient revealed uterine didelphys, obstructed right hemivagina causing hematohemicolpos and right renal agenesis. Thorough knowledge of imaging features can enable a radiologist to make a correct diagnosis even in an atypical presentation.

scholarly journals Herlyn Werner Wunderlich Syndrome with Vagino-umbillical Fistula: A Most Unique Presentation

2021 ◽  
pp. 1-8
Author(s):  
Pamela Grace Vidal Valera ◽  
Madonna Victoria Calderon Domingo
Keyword(s):  
Surgical Management ◽  
Renal Agenesis ◽  
Abdominal Mass ◽  
Three Dimensional ◽  
Transvaginal Ultrasonography ◽  
Unique Case ◽  
Vaginal Septum ◽  
Obstructed Hemivagina ◽  
Antibiotic Coverage

The Herlyn–Werner–Wunderlich (HWW) syndrome is an uncommon Mullerian duct anomaly composed of a triad of obstructed hemivagina, renal agenesis, uterine didelphys with a prevalence of [Formula: see text] females. Aside from its usual presentation of cyclic hypogastric pain and abdominal mass, there has been no other reported case in literature of the syndrome presenting with a vagino-umbilical fistula. Presented here is a unique case of a 35-year-old woman presenting with purulent, foul-smelling discharge extruding from a fistula on her umbilicus and her vagina associated with a tender hypogastric mass. Three-dimensional transvaginal ultrasonography aided the diagnosis of HWW syndrome with a concomitant vagino-umbilical fistula. Excision of the vaginal septum together with antibiotic coverage resolved her symptoms. The syndrome’s embryopathogenesis, classification, diagnostics, definitive surgical management and other issues during follow up are discussed.

scholarly journals Herlyn-Werner-Wunderlich Syndrome: A Case Report

2020 ◽  
Vol 18 (2) ◽  
pp. 70-73
Author(s):  
SM Saiful Islam
Keyword(s):  
Developmental Disorder ◽  
Renal Agenesis ◽  
Lower Abdominal Pain ◽  
Pediatric Emergency ◽  
Vaginal Opening ◽  
Vaginal Septum ◽  
Unilateral Renal Agenesis ◽  
Abdominal Mri ◽  
Obstructed Hemivagina ◽  
The Right

Herlyn-Werner-Wunderlich Syndrome (HWWS) is a rare developmental disorder of female urogenital tract consisting of paired uterus, obstructed hemivagina and unilateral renal agenesis. Here, authors described a case of a 10 years old girl with HWWS who presented to pediatric emergency with recurrent lower abdominal pain shortly following menstruation and dysmenorrhea. The diagnosis was confirmed by abdominal MRI. Examination under anesthesia revealed two vaginal opening, of which left is widely open and the right is completely closed with bulging hymen. A hymeneal incision was made with evacuation of the cavity. At present, the preferred surgical option is resection of as completely of the obstructing vaginal septum as possible and marsupialization for patients with completely obstructed hemivagina with blind hemivagina and incompletely obstructed hemivagina. The outcome of patients with HWWS is generally good with early detection of illness and prompt management. They can have normal sexual relations with preserved fertility. Chatt Maa Shi Hosp Med Coll J; Vol.18 (2); July 2019; Page 70-73

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