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 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.

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 Ultrasound Examination and Navigation for Repeat/Delayed Reconstruction of the Ankle Extensor Tendons

Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1408
Author(s):  
Kamal Mezian ◽  
Karolína Sobotová ◽  
David Zámečník ◽  
Levent Özçakar
Keyword(s):  
Surgical Management ◽  
Surgical Repair ◽  
Ultrasound Examination ◽  
Penetrating Injury ◽  
Unique Case ◽  
Extensor Tendons ◽  
The Third ◽  
One Year ◽  
Delayed Reconstruction

Herein, we describe a 46-year-old woman with persistent pain and weakness in her left ankle/foot one year after surgical repair of all three ankle extensor tendons following a penetrating injury. This report presents a unique case whereby US imaging played a paramount role in the diagnosis and surgical management of a previous nonanatomic repair of the ankle extensor tendons after a penetrating injury one year prior. The above-quoted findings were subsequently corrected with end-to-end sutures. On the third postoperative month follow-up, the patient was free of any complaints or complications.

scholarly journals Diagnosis and Treatment in a Tertiary Hospital of a Series of Complex Genital Malformations Corresponding to Double Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Júlia Kefalás Troncon ◽  
Júlio César Rosa-e-Silva ◽  
Rayssa Miranda ◽  
Francisco José Candido-dos-Reis ◽  
Omero Benedicto Poli-Neto ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Clinical Features ◽  
Renal Agenesis ◽  
Case Series ◽  
Tertiary Hospital ◽  
University Hospital ◽  
Pelvic Ultrasound ◽  
Vaginal Septum ◽  
First Choice ◽  
Obstructed Hemivagina

Aim. To evaluate the clinical features, diagnostic routine, treatment, and prognosis of patients with double uterus with obstructed hemivagina and ipsilateral renal agenesis at a University Hospital. Methods. A retrospective study analyzing the medical charts of outpatients with similar complex genital malformations seen at the University Hospital of the Ribeirão Preto Medical School from 1994 to 2015. Results. Fourteen patients were included in this retrospective study, all presenting with double uterus with obstructed hemivagina and ipsilateral renal agenesis. The main symptom was dysmenorrhea occurring shortly after menarche, and pelvic ultrasound was the examination of choice. The treatment consisted of resection of the vaginal septum, complemented by an abdominal approach in 5 cases. Complications of the syndrome observed in this case series included severe endometriosis, pelvic abscess, need for hysterectomy, and salpingectomy. Conclusions. Severe dysmenorrhea shortly after menarche is a typical symptom of this kind of malformation, even though the diagnosis of patients who present with fistulization of the vaginal septum can be delayed due to milder clinical features. Pelvic ultrasound can be considered the first-choice examination in diagnostic routine. Relief of pain and prevention of complications can be achieved successfully in most cases by resection of the vaginal septum.

Unilateral blindness: a unique complication of choanal atresia surgery

2014 ◽  
Vol 128 (1) ◽  
pp. 101-103 ◽  
Author(s):  
L D'Ascanio ◽  
E Rebuffini
Keyword(s):  
Choanal Atresia ◽  
Three Dimensional ◽  
Endoscopic Stenting ◽  
Unique Case ◽  
Superior Orbital Fissure ◽  
Published Report ◽  
Dimensional Reconstruction ◽  
The Right ◽  
Acute Headache

AbstractObjective:To report a unique case of unilateral blindness secondary to transnasal endoscopic surgery and stenting for right choanal atresia.Case report:A 24-year-old man was referred with right eye blindness and acute headache, occurring immediately after transnasal endoscopic surgical repair of unilateral right choanal atresia with placement of an endonasal stent. Maxillo-facial computed tomography with three-dimensional reconstruction showed the endonasal stent entering the right nostril, passing through the lamina papyracea into the orbit and running anterior to the optic foramen towards the superior orbital fissure. Despite stent removal and medical treatment (ceftriaxone and dexamethasone), permanent right eye blindness secondary to an irreversible lesion of the optic nerve was diagnosed. At three-month follow up, an uncommon, complete fibrous obliteration of the right nasal fossa was noticed.Conclusion:To the best of our knowledge, this is the only published report of unilateral blindness following transnasal endoscopic stenting for right choanal atresia. Causes of this complication, and ways of avoiding it, are discussed.

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