Obstructed Hemivagina Research Articles

Surgical management of 12-year-old with complete septate uterus with duplicated cervices and obstructed left hemivagina: A case report

Introduction: When a patient with an obstructed hemivagina presents in childhood, either hormonal suppression or a staged procedure is proposed for management of the obstruction. Case Report: This case report is a 12-year-old patient presenting for evaluation of hematocolpos with a complete septate uterus, duplicate cervices, bilateral kidneys, and obstructed left hemivagina who was surgically managed with a complete vaginal septum resection and hysteroscopic metroplasty in one procedure shortly after diagnosis. Conclusion: This anomaly is not only rare, and the first presented in the literature, but it also importantly demonstrates that if desired by the family after a consideration of benefits and risks, resection of both vaginal and uterine septa can be safely performed in a young post-menarchal patient without the need for a staged procedure later in life.

Uterus Didelphys with Hematocervix in the Unilateral Obstructed Hemivagina and Ipsilateral Renal Agenesis: A Case Report of Herlyn–Werner–Wunderlich Syndrome

Background: Mullerian duct anomalies include a broad spectrum of genital tract defects that arise from developmental abnormalities of the genital tract. Herlyn–Werner–Wunderlich syndrome (HWWS) refers to the combination of uterus didelphys, blind hemivagina and ipsilateral renal agenesis. In the literature, the syndrome often appears as a few sporadic case reports. Case: We report a case of symptomatic Herlyn–Werner–Wunderlich syndrome diagnosed by transavaginal scan and MRI and successfully treated with a laparoscopic-assisted hysteroscopic vaginal septum resection. The postoperative course was without complications, and clinical symptoms completely resolved the surgery. Summary and Conclusions: Combination of laparoscopy and hysteroscopy was found to be an easy, safe, effective and appropriate approach for patients with HWWS.

Blind ectopic hemato-megaureter: A rare association with the rare obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome

Blind ectopic hemato-megaureter: A rare association with the rare obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome

Vaginoscopic Resection of an Obstructed Right Hemivagina in Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome

Vaginoscopic Resection of an Obstructed Right Hemivagina in Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome

Ohvira syndrome with rare presentations – A case report

Herlyn Werner Wunderlich syndrome is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is synonymous with OHIVRA syndrome which is Obstructed Hemivagina with Ipsilateral Renal Agenesis syndrome. Very few case reports have been reported regarding the syndrome. Herein, we report a case of 20 year old unmarried female with unusual presentation of OHVIRA syndrome with complaints of spotting per vagina and with vaginal cyst on examination. Diagnosis was confirmed by MRI which showed features of OHVIRA with pyometra caused by the obstructed hemivagina. She was managed surgically by diagnostic laparoscopy and per vaginal resection of the vaginal septum and drainage of around 50cc of pyometra followed by visualisation of two separate cervical canals post procedure. As a rare entity, a high degree of clinical suspicion is necessary to diagnose and intervene at the right time to ensure optimum results and prevent complications and preserve fertility.

Addressing the delayed diagnosis of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome for improved women health in Africa: A case series

Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a rare urogenital anomaly, presents significant challenges to young girls and women in Africa. As we strive to break barriers and reposition Africa in the global health architecture, the timely recognition and management of OHVIRA becomes crucial. Usingt a retrospective case series approach, we examined OHVIRA cases managed by the Pediatric and Adolescent Gynecology Unit at a Tertiary Health facility in Malaysia, between June and November 2022. The study findings have relevance for Africa because the challenges faced by young girls and women in obtaining adequate healthcare are not confined by borders.
 Results: The age range of presentation, 11-25 years, mirrors the age group of concern for the African population. The time interval between symptom onset and diagnosis, spanning from 1 to 12 years, raises concerns about delayed identification. We observed that worsening dysmenorrhea, often unresponsive to analgesia, was the main presenting symptom, accompanied by complications, such as persistent vaginal discharge (12.5%), urinary retention (25%), constipation (12.5%), and pelvic masses (37.5%), which resonate with potential cases in Africa.
 Conclusion: The delayed diagnosis of OHVIRA syndrome emphasizes the pressing need for proactive healthcare interventions, particularly among young girls and women in Africa. By understanding the parallels between our study and the challenges faced by young women on the continent, we can identify opportunities to improve healthcare access and address the barriers that hinder timely diagnosis and management.

How to Diagnose and Manage Septated Obstructed Hemivagina and Ipsilateral Renal Agenesis: A Rare Case

How to Diagnose and Manage Septated Obstructed Hemivagina and Ipsilateral Renal Agenesis: A Rare Case

Obstructed Hemivagina and Renal Anomalies in Patients with and without Anorectal Malformations

Study ObjectiveTo compare the anatomic variation between patients with a diagnosis of obstructed hemivagina with an anorectal malformation (ARM) and those without ARM. MethodsThis was a retrospective chart review conducted at a single tertiary Children's Hospital. Patients with an obstructed hemivagina seen from 2004 to 2019 were included. ResultsWe identified a total of 9 patients diagnosed with obstructed hemivagina: 4 patients with history of ARM and 5 patients without ARM. Patients presented with obstructive symptoms between the ages of 11 and 20. Two-thirds of patients had a left sided obstruction. All patients without ARM had ipsilateral congenital anomalies of the kidney and urinary tract. Half the patients with a history of ARM had an ipsilateral renal anomaly and the other half had a contralateral renal anomaly. ConclusionsObstructed hemivagina occurs in patients with a history of anorectal malformation. However, unlike patients with isolated obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), patients with ARM and an obstructed hemivagina can present with associated renal anomalies on either the ipsilateral or contralateral side. In our small case series, patients with history of ARM had high septa and required more complex surgical management due to the inability to access the septum vaginally. Knowledge of renal anatomy and ureteral path is important since hysterectomy may be needed to relieve the obstruction in patients with ARM. A larger case series is needed to better characterize the spectrum of complex anomalies in patients with anorectal malformations.

Surgical Management of OHVIRA and Outcomes

Study ObjectiveObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) includes uterine didelphys, unilateral obstructed hemivagina, and ipsilateral renal anomaly. Surgical management of this condition relies on accurate diagnosis to excise the obstructed longitudinal vaginal septum (OLVS). Vital considerations involve identifying the side affected, ipsilateral renal anomaly (IRA), thickness of the septum (TS), septal axis (SA), and distance of the septum to perineum (DSP). The study aimed to evaluate the preoperative characteristics, imaging findings, and surgical outcomes of OHVIRA. MethodsInstitutional review board approval was obtained for this retrospective chart review. ICD-10 codes identified OHVIRA cases between 2012 and 2019 at a single children's hospital. Patient demographic characteristics, magnetic resonance imaging findings, surgical management, outcomes, and complications were reviewed. Descriptive statistics were utilized. ResultsTwenty-six patients met inclusion criteria. Most were diagnosed at puberty (92%). Abdominal pain (50%) was the most common presenting complaint. The mean age of diagnosis was 13.2 years overall and 11.2 years for those with regular cycles vs 13.4 years for those with irregular cycles. Preoperative imaging showed predominant right-sided OLVS (50%), IRA (77%), and oblique SA (65%). All patients underwent vaginoscopy, septum resection, and vaginoplasty, except 1 who was managed with an abdominal drain as a neonate. Four required postoperative vaginal stent or Foley, with DSP greater than 5 cm in all cases. One intraabdominal abscess complication occurred. No instances of hematocolpos re-accumulation or reoperation were observed during the 3-year follow-up period. ConclusionThis study demonstrates that detailed preoperative planning and a systematic surgical approach lead to favorable outcomes in OHVIRA irrespective of the OLVS laterality, TS, SA, or DSP.

#112 : Obstructed Hemivagina with Ipsilateral Renal Anomaly (OHVIRA) with Ureteric Remnant in an Adolescent Female

Background and Aims: Müllerian duct anomalies are a group of uncommon entities, which cause specific symptoms in adolescent females. OHVIRA is a very rare congenital anomaly of the urogenital tract characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. This paper describes a case of OHVIRA with ectopic ureteral insertion to the obstructed hemivagina and non-functional urinary bladder who was diagnosed and treated early. This study aimed to increase awareness regarding mullerian anomalies, discuss the ideal diagnostic tools and management of OHVIRA, and promote early diagnosis and treatment to avoid long-term complications. Method: A case of 12-year-old came for a consult with cyclic pelvic pain and palpable pelvic mass after her menarche. A series of diagnostic tests were done which were compatible with the diagnosis of OHVIRA with an associated finding of ectopic ureteral insertion into the obstructed hemivagina and non-functional urinary bladder. The patient underwent resection of the non-communicating longitudinal vaginal septum, drainage of hematocolpos, diagnostic hysteroscopy, and cystoscopy, which are the main treatment of patients with OHVIRA. Results: One week after discharge, the patient followed up with no hypogastric pain, dysuria, and no vaginal bleeding. One month after, she had a menstrual period soaking 2-3 pads per day with no associated hypogastric pain nor recurrence of a pelvic mass with on digital rectal examination. Her subsequent menses were unremarkable occurring every month. Conclusion: OHVIRA should be suspected in cases with cyclic pelvic pain in adolescents and also in neonatal cases with any renal malformations. The delay in its diagnosis and management is inevitable due to its rarity; however, the complications can be devastating. Overall, timely surgical removal of the obstructing vaginal septum can provide rapid relief of symptoms, prevention of complications, and preservation of fertility in the majority of cases.

#393 : Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) — A Series of 6 Cases at a Tertiary Referral Centre

Background and Aims: OHVIRA is a rare congenital urogenital anomaly. Diagnosis is often delayed because patients have normal onset of puberty and menstruation if there is partial obstruction with a communicating fenestration in the hemivagina. Symptoms can be non-specific such as abdominal pain and dysmenorrhoea, but urinary retention and a pelvic mass have been reported. Haematocolpos can cause anatomical distortion, making imaging difficult. Definitive management is by vaginoplasty but that can be complex, especially in an anatomically small child, or cases with chronic inflammation. Retrograde bleeding during obstruction is common, leading to endometriosis and ascending infection. This is associated with complex surgery, increased morbidity and subfertility, which is why early diagnosis and timely management are important. Method: Medical records were retrieved from 6 patients with OHVIRA treated between 1 April 2021 and 31 March 2023 at a tertiary referral centre. For each patient, age of diagnosis, referral source, symptoms, diagnostic investigations, diagnostic challenges, anatomical findings, surgical managements, complications and outcomes were reviewed. Results: Patient’s ages ranged from 10 to 33 years. Median age of diagnosis was 30. Cases were referred from paediatric surgery (n=1), gynaecology (n=2), general surgery (n=2) and urology (n=1). Symptoms included abdominal pain (n=4), pyrexia (n=2), intra-menstrual bleeding (n=2) and vaginal discharge (n=1). 3 patients had been misdiagnosed before referral. All had unilateral haematocolpos and underwent ultrasound (n=6) and either MRI (n=4) or CT (n=2), followed by vaginoplasty. 2 patients had emergency surgery for pelvic abscesses. There were no intra- or post-operative complications, or adverse outcomes. Conclusion: Median age at diagnosis was advanced, and symptoms and referral source were variable. Misdiagnosis was frequent. Surgical management was successful with a single stage vaginoplasty. Ascending infection can be a consequence of delayed diagnosis and management. It is important to evaluate any female with dysplastic or absent kidneys for uterovaginal abnormalities.

Delayed Presentation of Herlyn-Werner-Wunderlich Syndrome; A Rare Congenital Anomaly: A Case Report and literature Review

Herlyn-Werner-Wunderlich syndrome (HWWs) is a rare müllerian abnormality resulting in varying presentation, especially after puberty. A baseline pelvic ultrasound scan can help in diagnosis. The quality of life for these patients can be improved by timely and adequate surgical care., who otherwise may suffer from prolonged ongoing symptoms and complications in the future due to these anatomical abnormalities. Background: Herlyn-Werner-Wunderlich Syndrome (HWWs) is a rare congenital condition involving uterus didelphys, obstructed hemi-vagina, and ipsilateral renal agenesis due to abnormal development of ducts. Diagnosing Müllerian anomalies can be challenging due to varying symptoms. The case of delayed HWW s presentation in a patient is discussed, emphasizing the need for early referral to specialized care. Case: A 25-year-old woman with a history of recurrent UTIs as well as a single right kidney was diagnosed with Herlyn-Werner-Wunderlich Syndrome (HWWs), a rare condition involving obstructed hemi-vagina, uterus didelphys and ipsilateral renal agenesis. Surgical intervention successfully was a suitable treatment for this condition, and the patient was referred to a urologist for further urinary symptoms management. Conclusion: Herlyn-Werner-Wunderlich syndrome diagnosis is challenging, but early detection with pelvic ultrasound can aid in diagnosis; treatment involves resecting the hemivaginal septum.

The Need for Earlier Diagnosis of Obstructed Hemivagina and Ipsilateral Renal Agenesis/Anomaly (OHVIRA) Syndrome in Case of Renal Agenesis in Girls—Case Report and Review of the Literature

Obstructed hemivagina and ipsilateral renal agenesis/anomaly (OHVIRA) syndrome is a very rare condition affecting girls. The time of diagnosis varies, from cases of prenatal diagnosis up to adulthood, including pregnancy or delivery. Most commonly, it is recognised during puberty and usually as an acute condition. We present a case report of an adolescent girl who underwent the treatment because of acute abdominal pain. The case is interesting due to a previous diagnosis of one-side renal agenesis. It appears to be useful to perform a diagnostic pelvic imaging at the time of diagnosis of renal agenesis or to plan to perform it at the beginning of puberty, to prevent the need for urgent surgery. This management may allow the planning of proper follow-up, minimising the risk of possible complications.

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

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.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome–A “no-touch-approach”: A case report

<b>Introduction:</b> Herlyn-Werner-Wunderlich (HWW)/obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare Müllerian duct anomaly (MDA). Usually presenting after menarche classically with dysmenorrhea. Here we present a challenging case, where the diagnosis was missed initially.<br /> <b>Case presentation:</b> A 10-year-old girl presenting with dysmenorrhea and constipation, treated repeatedly with analgesics, laxatives and enema. A pelvic ultrasound and magnetic resonance imaging (MRI) confirmed the diagnosis of OHVIRA syndrome, which requires surgical intervention. We describe a “no -touch- technique” for resecting the septum. The patient had immediate relief of symptoms and a smooth post-operative recovery.<br /> <b>Conclusions:</b> Early diagnosis and surgical management are crucial not only to relieve symptoms but to prevent long term complications that may affect the patients’ reproductive potential. Despite pelvic ultrasound being an affordable and a useful diagnostic tool, the diagnosis can be missed by inexperienced operators. Therefore, MRI is recommended to confirm and classify MDAs. The hysteroscopic “no-touch-technique” to resect the septum is an effective, safe and a convenient approach to treat the condition while preserving hymen integrity.

Management of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome and diminished ovarian reserve a video article

Management of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome and diminished ovarian reserve a video article

Diagnostic challenge in a case of foul-smelling vaginal discharge in a newly attained menarche girl

Foul-smelling vaginal discharge in adolescents often stems from infectious origin, foreign body insertion, neoplasm, congenital anomaly, and sexual abuse. Diagnosing the causes of the discharge remains challenging, especially in cases where sexual and reproductive health awareness is relatively low in the population from marginalized ethnographies and socio-cultural backgrounds. Suspicion of sexual abuse must be explored carefully and sensitively. A history of sexual exposure might not be forthcoming in adolescents where premarital sex is frowned upon. The process of eliciting the causation may be a challenge to healthcare professionals, delaying the diagnosis and subsequent management. In rare circumstances, imaging studies may help confirm the origin of the infection, giving insight into the presentation’s aetiology. We describe a case of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome presented with foul-smelling vaginal discharge in a newly attained menarche girl in the North-East region of Peninsular Malaysia. We also discussed the theories and reasons behind the occurrence of such a presentation, along with the diagnostic dilemma in this current case.

FRI413 Hormonal Menstural Suppression For Menstrual Obstruction: A Retrospective Review Of Patients With Complex Obstructed Uterovaginal Anomalies Who Underwent Surgical Management In A Tertiary Referral Centre

Abstract Disclosure: H.I. Learner: None. S.M. Creighton: None. S.A. Clarke: None. Aims: To describe the use of hormonal menstrual suppression in patients undergoing surgery for complex utero-vaginal anomalies with menstrual obstruction. Background: Obstructed menstruation can present with painful primary amenorrhea or progressive dysmenorrhea due to unilateral obstructing duplication utero-vaginal anomalies. Menstrual suppression is essential to relieve pain during investigation and surgical planning. The off licence use of progestogens, hormonal contraception and GNRH analogues is common but there is little literature describing their best use in this context. Methods: This was a retrospective case series review of patients undergoing surgical management of complex menstrual obstruction in a tertiary referral centre between 2016-2022. Data collected on age, diagnosis, symptoms, duration of symptoms, surgery, the type of endometrial suppression used and associated side effects.Patients with partial obstruction were excluded. Results: Eighty-four patients were identified from the surgical database. The median age at time of surgery was 16 years (range 11-36). Unilateral obstruction was managed in 53/84 and complete obstruction in 31/84. Diagnoses included obstructed hemi-vagina (32/84), unicornuate uterus with functional uterine remnant (19/84), transverse vaginal septum (15/84), cervical/vaginal agenesis (9/84), and Mullerian agenesis with functional uterine remnant (5/84). Symptom duration information was available in 74/84 with a median of 10 months(0-252 months). Changes to endometrial suppression treatment were required in most patients, with 50/84 (40%) remaining on their original prescription. The average number of treatment regimes was 2 (0-7). Menstrual suppression at time of surgery was Norethisterone 35/84 (42%), Continuous combined oral contraceptive pill18/84 (21%), Medroxyprogesterone 11/84 (13%), GNRH 10/84 (12%), Desogestrel 6/84 (7%), and Depo-Provera 4/84 (5%). Tibolone was used as add-back in five patients on GNRHa for more than 6 months duration of use (4/5) or hypoestrogenic side-effects (1/5) . Conclusion Menstrual suppression for pain relief is an essential component in management for complex uterovaginal anomalies. Suppression should be started at initial presentation but there is limited guidance for clinicians as to what and how to prescribe. Our study has demonstrated that this can lead to chaotic prescribing, unnecessary pain and multiple treatment changes. Presentation: Friday, June 16, 2023

EP33.02: Use of transperineal ultrasound in a case of obstructive hemivagina ipsilateral renal agenesis

EP33.02: Use of transperineal ultrasound in a case of obstructive hemivagina ipsilateral renal agenesis

LigaSure for Management of Obstructed Hemivagina with Ipsilateral Renal Anomaly (OHVIRA) Syndrome

LigaSure for Management of Obstructed Hemivagina with Ipsilateral Renal Anomaly (OHVIRA) Syndrome