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Background: Although vaginal stenosis following pelvic or vaginal radiotherapy for cancer treatment is a common complication, postpartum vaginal stenosis is a very rare obstetric complication. We report two cases of postpartum vaginal stenosis., Case Presentation: The first was case was a 30-year-old para-I Ethiopian woman who presented with a history of foul-smelling vaginal discharge on her 11th postpartum day. Pelvic magnetic resonance imaging (MRI) demonstrated a 1.4 cm distal vaginal stenosis. The second case was a 39-years-old para-II Ethiopian woman who presented with history of amenorrhea and dysparunia of 20 months duration. Abdominopelvic ultrasound examination revealed hematometrocolpos secondary to postinflammatory vaginal stenosis, and 3300 ml of hematocolpometra was drained. In both cases, adhesion excision with successful vaginal reconstruction was performed., Conclusion: Postpartum vaginal stenosis is a rare obstetric complication and vaginal adhesions excision with regular vaginal dilation is the recommended management for it., Competing Interests: Declarations Ethics approval and consent to participate Not applicable. Consent for publication Written informed consent was obtained from both patients for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests The authors have no competing interest., (© 2024. The Author(s).)

We present the case of a 6-year-old girl who initially presented with acute pelvic pain, ultimately diagnosed with imperforate hymen leading to hematocolpos. Further investigation revealed additional clinical features including academic struggles, mood swings, and cutaneous findings, prompting consideration of a neurocutaneous syndrome. Magnetic Resonance Imaging (MRI) revealed features consistent with tuberous sclerosis complex (TSC), including radial migration lines in the subcortical white matter and an incidental arachnoid cyst. Notably, this case exhibited a unique presentation with absence of typical TSC findings such as subependymal nodules or cortical tubers. Additionally, precocious puberty, rarely associated with TSC, was observed, suggesting a potential link between hypothalamic lesions and hormonal imbalance. This case underscores the importance of comprehensive evaluation in pediatric patients presenting with seemingly unrelated symptoms, as it may unveil underlying conditions necessitating tailored management strategies., (© 2024. Fondazione Società Italiana di Neurologia.)

Transverse vaginal septum (TVS) is a rare obstructive genital tract anomaly. It is associated with primary amenorrhoea and typically presents with cyclical abdominal pain due to obstruction of the menstrual flow. Caudal regression syndrome (CRS) is also a rare congenital anomaly that is frequently associated with anomalies of the neurological, musculoskeletal, cardiac, genitourinary and gastrointestinal systems. Obstructive genital tract anomaly in CRS is exceptionally rare. This report describes the case of a girl in early adolescence with underlying CRS who presented with severe abdominal pain associated with primary amenorrhoea. Clinical and radiological assessment revealed 'haematocolpos' manifesting as a tender 20 weeks' size abdominal mass and an absent vaginal opening. TVS was identified during examination under anaesthesia. The patient subsequently underwent a successful vaginoplasty with no recurrence of symptoms after 2 years., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Genital tuberculosis, a form of extrapulmonary tuberculosis (EPTB), exhibits distinct presentations. In the outpatient department, an adolescent girl reported severe pain and a feeling of heaviness in her lower abdomen for the past 6-7 days. An enlarged pelvic mass, resembling a 20-week pregnancy, was observed, seemingly originating from the pelvis. During the local examination, a transverse septum was felt in the lower vagina, with a vaginal length of 2-3 cm. The ultrasound revealed distension of the vagina with fluid containing fine internal echoes, indicating haematocolpos. MRI showed the uterus pushed upward and located at the level of the umbilicus, suggesting hydrocolpos. Based on these findings, a provisional diagnosis of transvaginal septum with haematocolpos was made. Under anaesthesia, a cruciate incision was made over the vaginal septum, resulting in the drainage of 700-800 mL of pus. The drained fluid was sent for microscopic examination, gram staining, acid-fast bacilli smear culture, and Cartridge-based nucleic acid amplification test (CBNAAT). The CBNAAT test confirmed the presence of tubercle bacilli. Antitubercular therapy was initiated, and on completion of the treatment, the girl experienced the onset of menarche. This is a typical case with an unusual presentation of EPTB. What makes this case noteworthy is its initial manifestation as haematocolpos, a condition that shares a similar clinical presentation with Müllerian anomalies., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear., (Copyright © 2024 SEEN and SED. Published by Elsevier España, S.L.U. All rights reserved.)

Haematocolpos, caused by imperforate hymen, is a rare condition where menstrual blood accumulates in the vagina. Adolescent girls presenting with amenorrhoea, cyclical abdominal pain, and pelvis mass should be evaluated for this condition. Diagnosis requires a gynaecological examination. However, myths surrounding the hymen may impede proper medical care. Addressing these misconceptions is essential for promoting gynaecological care and ensuring timely evaluation and treatment. This case report emphasises the significance of effective communication in preventing misdiagnoses and care delays., (Published under Open Access CC-BY-NC-BD 4.0. https://creativecommons.org/licenses/by-nc-nd/4.0/.)

Study Objective: To describe cases of image-guided drainage of symptomatic hematometrocolpos from obstructive Müllerian anomalies as a temporizing measure to manage acute pain symptoms and delay definitive management of the obstructive Müllerian anomalies that require complex reconstruction METHODS: Institutional Review Board exemption from all included institutions was obtained. A retrospective case series from 3 academic children's hospitals of 8 females under the age of 21 with symptomatic hematometrocolpos due to obstructive Müllerian anomalies drained by image-guided percutaneous transabdominal vaginal or uterine drainage with interventional radiology was reviewed and described., Results: Eight pubertal patients with obstructive Müllerian anomalies (6 patients with distal vaginal agenesis, 1 patient with an obstructed uterine horn, and 1 patient with a high obstructed hemi-vagina) and symptomatic hematometrocolpos are reported. All patients with distal vaginal agenesis had greater than 3 cm lower vaginal agenesis, which would usually require complex vaginoplasty and use of postoperative stents. Given their immaturity and inability to use stents or dilators postoperatively or medical complexity, they subsequently underwent ultrasound-guided drainage of hematometrocolpos with interventional radiology to relieve pain symptoms, followed by menstrual suppression. The patients with obstructed uterine horns had complex medical and surgical histories requiring perioperative planning; they also underwent ultrasound-guided drainage of hematometra as a temporizing measure to manage acute symptoms., Conclusion: Patients presenting with symptomatic hematometrocolpos due to obstructive Müllerian anomalies might not be psychologically mature enough to undergo definitive complex reconstruction, which requires vaginal stent or dilator use postoperatively to prevent stenosis and other complications. Image-guided percutaneous drainage of symptomatic hematometrocolpos serves as a temporizing measure by offering pain relief until patients are ready to undergo surgical management and/or to allow time for complex surgical planning., (Copyright © 2023. Published by Elsevier Inc.)

Introduction: OHVIRA-syndrome (obstructed hemivagina, ipsilateral renal agenesis/anomaly) is a rare Mullerian duct anomaly that can lead to complications in pubescent children., Case Report: We report a case of a 13-year-old patient with acute right-sided lower quadrant abdominal pain who was referred for exclusion of appendicitis. As a result of the examination (transvaginal ultrasound scan and gynecological examination), a female genital tract anomaly was suspected in the form of obstructed hemivagina with hematocolpos and hematometra. The MRI scan showed hematocolpos and hematometra on the right side, uterus didelphys accompanied by right-sided renal agenesis, consistent with OHVIRA-syndrome. Excision of the vaginal septum was performed and the accumulated old menstrual blood, as represented by hematocolpos and hematometra, was evacuated. Postoperative recovery was uneventful., Conclusion: The early surgical management of this rare Mullerian duct anomaly is important in order to prevent longterm complications. This malformation should be considered in the differential diagnosis of acute lower abdominal pain in pubescent girls., Key Words: Abdominal Pain, Genital Anomaly, Obstructed Hemivagina, Renal Anomaly.

A young adolescent girl with trisomy 13 was admitted twice to the paediatric department: the first time because of haematocolpos due to uterus didelphys and unilateral transverse vaginal septum, and the second time because of heart failure due to ruptured sinus of Valsalva aneurysm. As a consequence of the historical early high mortality rate in trisomy 13, we are not aware of known complications in older patients. With better survival nowadays through childhood, we advise structural ultrasonographic cardiac and female genital screening in trisomy 13 patients reaching adolescent age., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Study Objective: To show a conservative surgical treatment for a female adolescent affected by Wunderlich syndrome with didelphys uterus and obstructed hemivagina., Design: Stepwise demonstration of the technique with narrated video footage., Setting: In the context of obstructive congenital müllerian anomalies, involving a stagnation of menstrual blood, the Wunderlich syndrome is the most common and constantly characterized by the duplicity of the uterine body, by the presence of an imperforate hemivagina, and by renal agenesis ipsilateral to the obstructed hemivagina. The imperforate hemivagina leads to dysmenorrhea and abdominal pain, owing to the hematocolpos and the hematometra, which arose immediately after the menarche. This is the case of a 14-year-old female adolescent affected by Wunderlich syndrome referred to the San Raffaele Hospital adolescent center (Milan, Italy) for dysmenorrhea and abdominal pain. At vaginal exploration, a right imperforated hemivagina and hematocolpos were highlighted. A diagnostic magnetic resonance imaging (MRI) found the duplicity of the uterine body, the hematometra, and the right renal agenesis., Interventions: A combination of explorative laparoscopy and surgical vaginal time leads to the excision of the vaginal septum that allows the drainage of the hematocolpos and of the hematometra. Three surgical steps are described: 1. First laparoscopic step: exploration of the abdominal cavity with the detection of a double uterine body, an enlarged hemiuterus, and an enlarged hemivagina caused by the hematometra and the hematocolpos. Careful evaluation of adnexa for eventual presence of hematosalpinx. 2. Vaginal step divided into the following: (A) puncture of the vaginal tumescence (corresponding to the imperforated hemivagina) with a 19-gauge needle mounted on a syringe. Aspiration results in thick creamy black material (old menstrual blood). (B) In correspondence with the needle puncture, a full-thickness incision of the vaginal wall widely opening the second uterine cervix and (C) stabilization of the opening by the marsupialization of the edge of the obstructed hemivagina were performed. 3. Second laparoscopic step: having emptied the hematocolpos of the left hemiuterus, the didelphys uterus and the disappearance of the hematocolpos can be clearly seen., Conclusion: Here, we demonstrate a conservative surgical approach for the treatment of Wunderlich syndrome. This rare malformation is characterized by an extreme variability of the anatomic presentation, and the precise identification of the variety together with the early diagnosis is of fundamental importance for the surgical correction., (Copyright © 2022 AAGL. Published by Elsevier Inc. All rights reserved.)

Obstructed hemivagina and ipsilateral renal anomaly syndrome also known as Herlyn-Werner-Wunderlich syndrome is a rare congenital urogenital anomaly characterised by Mullerian duct anomalies associated with mesonephric duct anomalies. A 10-year old female presented with acute lower abdominal pain, urinary retention and scanty menstrual flow during her first menstruation. Ultrasonography and contrast computed tomography showed uterine didelphys, hematocolpos, obstructed hemivagina and left renal agenesis. Hemivaginal septal resection and drainage of the hematocolpos were done and operative findings also confirmed the final diagnosis. She was discharged and followed up after 2 weeks and her symptoms had resolved completely. Being a rare entity many clinicians and radiologists are unaware of this disease so this may lead to misdiagnosis whenever these cases present. So strong suspicion and knowledge of this disease entity are essential for a precise diagnosis., Keywords: case reports; hematocolpos; mullerian ducts; unilateral renal agenesis.

Objective: To demonstrate the advantage of using aqueous vaginal contrast and scheduled hematocolpos with magnetic resonance imaging (MRI) to improve the delineation of gynecologic anatomy and to recommend that this modality be considered in patients with complex müllerian anomalies., Design: Video demonstration of MRI adjuncts to improve visualization of gynecologic anatomy., Setting: Academic Hospital., Patient(s): A patient with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) who presented for definitive surgical management., Intervention(s): OHVIRA is a unilateral obstructed müllerian anomaly that presents typically after menarche with progressively worsening dysmenorrhea caused by progressive distension of the obstructed hemivagina and uterine horn. The definitive treatment for this anomaly is resection of the unilateral obstruction. When the obstructed hemivagina is within close proximity to the patent hemivagina, vaginal septum resection should be performed to relieve the obstruction successfully. However, when the obstructed hemivagina and uterine horn are not adjacent to the patent hemivagina, a simple septum resection is not feasible and there is a high rate of restenosis if anastomosis is attempted. In this case, laparoscopic removal of the obstructed uterine horn, fallopian tube, cervix, and vagina should be considered as an alternative approach to resolving the obstruction. A surgical approach can be recommended only once the surgeon has a clear understanding of the patient's pelvic anatomy and the magnitude of the obstruction. In the presented case, a 17-year-old patient with OHVIRA presented for definitive surgical management. While on hormonal suppression, a pelvic MRI was performed that identified a uterus didelphys with a left hemiuterus and cervix communicating with a patent vagina. The right hemiuterus and cervix were measured 2.5 cm from the patent vagina. However, because of hormonal suppression, the vaginal cavity was decompressed, making it very difficult to discern the relationship between the two uteri and vaginas. To better determine whether vaginal septum resection to relieve the obstruction was feasible, norethindrone was discontinued to allow menstrual blood to fill the obstructed hemivagina followed by a subsequent pelvic MRI with aqueous vaginal contrast to fill the patent vagina with contrast gel to improve the visualization of the decompressed vaginal cavities., Main Outcome Measure(s): Advantage of aqueous vaginal contrast and scheduled hematocolpos with MRI to image pelvic anatomy in a patient with a complex müllerian anomaly to guide surgical decision-making., Result(s): The addition of vaginal aqueous contrast clearly delineated the course and caliber of the patent vagina and its relationship to the obstructed hemivagina, now filled with blood. The inferior margin was in closer proximity to the patent vagina, but with only a very narrow segment (<1 cm) adjacent to the patent vagina and the obstructed cervix was displaced superiorly, now measuring 3.5 cm above the patent vagina. Surgical management options were discussed with the patient, and given the superior location of the obstructed uterus and cervix with only a narrow border of the vagina in continuity with the patent vagina, the risk of postoperative stenosis after vaginal septum resection was determined to be too high. The decision was made to proceed with a laparoscopic resection of the obstructed right side, and the patient underwent laparoscopic resection of the right hemiuterus, fallopian tube, cervix, and vagina. Intraoperatively, a survey of the pelvis again confirmed that the two vaginas were too far to reconnect safely without a high risk of stenosis. The patient recovered without complications postoperatively and her menses resumed without any pain., Conclusion(s): We highlight the use of two techniques to optimize MRI imaging of pelvic anatomy in a patient with a complex müllerian anomaly. First, the use of aqueous vaginal contrast with MRI is advantageous to clearly delineate the course and caliber of the patent vagina in patients with complex gynecologic anatomy. Second, cessation of hormonal suppression to allow menstruation to cause hematocolpos helped delineate the relationship between the obstructed vagina and patent vagina. In the presented case, these MRI adjuncts provided necessary detail that could not be appreciated with standard MRI to confirm that vaginal septum resection to preserve the right uterus would be too high a risk for postoperative stenosis in this patient. Aqueous vaginal contrast and scheduled hematocolpos should be considered as adjuncts to MRI when standard imaging modalities are unable to clearly describe the relationship between pelvic structures in cases of complex müllerian anomalies to help guide treatment recommendations., (Copyright © 2021 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.)

Objective: Comprehensive analysis of causes, clinical signs, dia-gnostic process, differential dia-gnosis and therapy of hymenal atresia., Methods: Literature search using the Web of Science, Google Scholar and PubMed databases with keywords and analysis of articles published in high impact and reviewed journals., Results: Hymenal atresia is a congenital malformation of a womans genitals, which is manifested by complete obstruction of the vaginal introitus by a closed hymen. It should be dia-gnosed in the neonatal period, but clinically it usually manifests itself only during puberty as a result of menstrual blood retention (cryptomenorrhea) with the cyclic abdominal pain at monthly intervals. The therapy is based on optimally timed surgical creation of communication in the hymen (hymenotomy, hymenectomy) enabling free evacuation of menstrual contents. The aim of this simple treatment method is immediate subjective relief from pain and a permanent solution to this congenital anomaly., Conclusion: Knowledge of all types of congenital malformations of the female genitalia is a basic condition for an early and effective dia-gnostic process in adolescent girls with abdominal pain. The girl who has not yet menstruated and has cyclic lower abdominal pain and a tumor behind the pubic symphysis should be examined by a specialist in pediatric and adolescent gynecology who will confirm hymenal atresia according to a bluish and closed hymen, and suggest prompt and effective therapy.

Distal vaginal agenesis is an obstructive congenital malformation of the female reproductive tract. The distal part of the vagina is replaced by fibrous tissue and the outflow of the menstrual blood and secretion of the cervical glands is disabled. This congenital anomaly most often manifests during expected menarche by primary amenorrhea and cyclic lower abdominal pain caused by cryptomenorrhea with gradually increasing hematocolpos and hematometra. The dia-gnosis is based on gynecological examination and the suspected anomaly is confirmed by ultrasound examination and magnetic resonance imaging. Therapy of distal vaginal agenesis is exclusively surgical. A pull-through vaginoplasty is the method of choice for distal vaginal agenesis not exceeding 3cm. With a greater extent of agenesis and the risk of postoperative vaginal stenosis, replacement of the missing part of the vagina with other tissues or modified balloon vaginoplasty can be used. The aim of the treatment is to enable the evacuation of the menstrual blood, ensure quality sexual intercourse and the possibility of reproduction.

Objectives: Imperforate hymen (IH) is a common genital tract anomaly in women which usually presents after puberty. However, surgical treatment is often considered controversial in religious or conservative communities for sociocultural reasons. This study therefore aimed to assess the efficacy of a novel reconstructive technique involving the preservation of the annular hymen., Methods: This prospective interventional study was performed between July 2013 and October 2019 at the minimally invasive surgery unit of a tertiary university hospital in Egypt. A total of 36 women presenting with primary amenorrhoea and haematocolpus were diagnosed with postpubertal IH. A circular hymenotomy was performed on each patient using a 10 mm laparoscopy trocar tip and sleeve to form a new annular hymen under general anaesthesia while preserving the annular hymen. The primary outcome measure was the persistence of hymenal patency and integrity at follow-up. The secondary outcome measure included post-operative patient satisfaction and pain relief., Results: The reported technique was feasible in all cases without intraoperative complications. Patency of the reconstructed annular hymen was confirmed at follow-up in all cases; moreover, no intraoperative complications were reported. There was a significant post-operative improvement in pain scores ( P <0.001). Both the patients and their parents/guardians reported a high level of satisfaction with the technique., Conclusion: This novel technique for the correction of IH involving the reconstruction of an annular hymen was found to be a safe, minimally invasive and effective procedure. This technique should be considered a feasible alternative to a conventional hymenotomy as it allows for the resumption of normal hymenal anatomy without overtreatment., Competing Interests: CONFLICT OF INTEREST The author declares no conflicts of interest., (© Copyright 2021, Sultan Qaboos University Medical Journal, All Rights Reserved.)

Introduction: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-diagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients., Methods: We carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date., Results: There were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina., Conclusion: Our case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment., Study Design: Case series, level IV evidence., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Acute urinary retention is rare in the pediatric population and is typically caused by an obstructing entity. It may result from hematocolpos caused by imperforate hymen. Imperforate hymen is rare, with an associated incidence of 1 in 2000 people. Nonetheless, in a pediatric age female who presents with urinary retention and who has a history of primary amenorrhea or a history of cyclical pelvic pain an examination of the external genitalia should be performed to rule out this condition., Case Report: A 12-year-old girl presented to the emergency department with urinary retention and was discharged with a diagnosis of urinary tract infection. She returned the following day to the emergency department with worsening abdominal pain. A computed tomography scan revealed a fluid-filled vagina measuring 12.5 cm. Her bladder measured 15.4 cm. The patient was taken to the operating room for further evaluation by the consulting gynecologist. At the time of surgery, 1000 mL of urine were evacuated after catheterization. Three hundred milliliters of chocolate-colored fluid, consistent with menstrual blood, were evacuated at time of hymenotomy. Hymenotomy was performed with a cruciate incision. No complications were encountered. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: To date, there have been >40 reported cases of imperforate hymen causing hematocolpos and subsequent urinary retention. However, given the rarity of imperforate hymen, hematocolpos leading to acute urinary retention can still be missed. Untreated urinary retention can lead to a compromise in bladder function and subsequent kidney damage., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Bone marrow transplantation is a lifesaving procedure for a range of serious benign or malignant hematological conditions. A proportion of patients, however, will develop graft vs host disease (GVHD), acute or chronic, with serious long-term sequalae., Cases: We present 2 cases of hematocolpos that developed in adolescence because of vaginal synechiae due to GVHD. The condition was initially asymptomatic, resolved spontaneously at first and recurred. In 1 girl blunt lysis of the adhesions was performed with the patient under general anesthesia, followed thereafter by local hydrocortisone and estriol treatment., Summary and Conclusion: Genital symptoms might not be readily reported by adolescents after bone marrow transplantation. Physicians should be aware of possible late effects of GVHD on genitalia, inquire about symptoms, and be acquainted with addressing complications, such as vaginal obstruction., (Copyright © 2018 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Genital chronic graft-versus-host disease (GVHD) in women posthaematopoietic cell transplantation may cause vaginal mucosal sclerosis. Human papillomavirus (HPV) reactivation, also common post-transplantation, limits local immunosuppressive, but not oestrogen treatment. A 36-year-old nulliparous woman developed coincidental genital chronic GVHD and HPV 22 months after transplant for aplastic anaemia. Topical immunosuppression for GVHD led to an eruption of warts successfully treated with laser surgery and cone biopsy. She maintained normal ovarian function and used extended cycle combined hormonal contraception. A vaginal oestrogen ring used continuously limited most scarring for 8 years. Progressive apical vaginal scarring obstructed menstrual flow leading to haematocolpos and haematometra. Normal anatomy was restored with a cruciate incision in the cervicovaginal scar performed during menses. When HPV disease limits use of topical immunosuppression in women with vaginal GVHD, the local scar-reducing effect of a vaginal oestrogen ring is limited, and surgery may be needed and can be successful in treating haematocolpos.This study was registered in ClinicalTrials.gov with trial registration number of NCT00003838., Competing Interests: Competing interests: None declared., (I, Pamela Stratton, The Corresponding Author has the right to grant and do so grant on behalf of all Contributors a licence to all intellectual property rights for all content within the submitted case report or as later submitted (which includes without limitation any diagrams photographs, other illustrative material, video, film or any other material howsoever submitted by any of the Contributors at any time and related to this article) other than as agreed with the BMJ Publishing Group Ltd) (“BMJ”)) has the right to grant on behalf of all Contributors and does grant on behalf of all Contributors , a licence to BMJ in any media known now or created in the future, and permit this case report (if accepted) to be published on BMJ Case Reports website and /or within any other BMJ products in any media and to be fully exploited within the remit of the licence which has been read.)

The imperforation of the hymen is a relatively rare congenital malformation. It usually manifests itself in adolescence by a hematocolpos. Hematocolpos is the vaginal retention of menstrual blood at puberty. It results clinically in painful amenorrhea and more rarely in a pelvic mass syndrome. The diagnosis is easy, it is primarily clinical. Pelvic ultrasound and nuclear magnetic resonance are the complementary examinations to be performed as a preoperative assessment and to detect any associated genito-urinary malformations.

We present four cases of atresia hymenalis with resultant haematometrocolpos diagnosed in quick succession in the emergency department with a literature review.

Background: Isolated hematotrachelos is an ex- tremely rare condition. More commonly it is associated with hematometra and/or hematocolpos. It may devel- op secondary to congenital anomalies of the reproductive tract or may be an acquired condition following cervical surgery or manipulation. No case has been reported fol- lowing abdominal myomec- tomy., Case: A 40-year-old, nul- ligravid woman was diag- nosed with isolated hemato- trachelos when she presented with severe abdominal pain following ovulation induction with clomiphene citrate. After the diagnosis was obtained, she recounted 2 similar presentations occurring months earlier. Both episodes had been attributed to gastroenteritis, but each had re- solved with "explosive" onset of menses. All 3 episodes plus 1 later recurrence happened within a 36-month period following an abdominal myomectomy., Conclusion: Acquired hematotrachelos is a rare condition, and the diagnosis is often missed. To our knowledge, this is the first reported case of isolated, recurrent hematotrachelos following the use of a uterine manipulator cannula in association with an abdominal myomectomy.

Study Objective: To demonstrate the technique of laparoscopic vaginal reconstruction in a rare case of mid-vaginal septum with secondary infertility., Design: A step-by-step explanation of the technique using videos and pictures (Canadian Task Force classification IV)., Setting: Transverse vaginal septum is a rare condition, with an incidence of only 1 in 30,000 women. It is usually a congenital mullerian fusion defect; few cases of acquired septum have been reported. Roughly 40% of cases occur in the mid-vagina. Transverse vaginal septum typically presents with primary amenorrhea and hematocolpos. The goal of surgery is to create a patent vagina with restoration of fertility. The laparoscopic approach has proven superiority over the open technique., Interventions: Laproscopic vaginal reconstruction was performed in a 24 year female with transverse vaginal septum. Dissection was done laproscopically up to mid vagina. Incision was given on vagina excising the septum followed by reconstruction., Conclusion: The laparoscopic approach to vaginal reconstruction avoids the abdominal incision, with its associated pain and possible adhesion formation. It also provides a better view for dissection. In this patient, a patent vagina was created in a single operation, with no postoperative dyspareunia, and fertility was restored., (Copyright © 2016 AAGL. Published by Elsevier Inc. All rights reserved.)

Transverse vaginal septum is a residual vaginal plate composed of the Müllerian duct and urogenital sinus. Imperforate hymen results from failure of perforation of the membrane between the urogenital sinus and vaginal cavity. We report a rare case of concurrence of these two conditions. A 16-year-old girl had been treated with puncture several times for hematometra and hematocolpos from 13 years of age because of monthly occurrence of lower abdominal pain without menstrual bleeding and was referred to our hospital. Magnetic resonance imaging demonstrated hematometra, hematocolpos and expansion of the vaginal fornix. The imperforate hymen was incised and a slight adhesion at the lower vaginal cavity was detached. After that, a complete transverse vaginal septum, which was 5 mm thick, was identified. It was excised after ultrasonography-guided puncture. Although two cycles of menstrual bleeding took place, molimina recurred. Re-operation was performed 6 months after the first operation, and recurrence of adhesion in the lower vaginal cavity was identified. A silicon dilator was inserted, but she could not use it at home and instead used a tampon. Cyclic menstrual bleeding is observed 4 months after the second operation., (© 2015 The Authors. Journal of Obstetrics and Gynaecology Research © 2015 Japan Society of Obstetrics and Gynecology.)

A 1-year-old female Labrador retriever was referred with a few days history of haematic-like vulvar discharge. Physical examination, vaginal inspection and palpation did not reveal any remarkable finding. Transabdominal ultrasound showed echogenic fluid accumulation in the vagina suggesting haemato-pyocolpos. An exploratory laparotomy was performed: a well-delimited ectasic vagina was identified. Ovariohysterectomy and partial vaginectomy and vaginoplasty were performed to spay the bitch and to remove the ectasic vagina. Post-operative recovery and 12-month follow-up were uneventful. Clinical, morphological and histological findings were consistent with a congenital abnormality of the muscular layer of the vagina complicated by haemato-pyocolpos. The disorganization of the vaginal tunica muscularis may have acted as locus minoris resistentiae in the vaginal wall. The organ was dilated and atonic due to the gradual accumulation of physiological fluids complicated by an overgrowth of genital bacteria. This congenital disorder has to be taken into account as differential diagnosis of haemato-pyocolpos with vaginal discharge in young bitches., (© 2015 Blackwell Verlag GmbH.)

Mullerian anomalies are a relatively uncommon occurrence with implication for adolescents and adults as they may result in specific gynaecologic, fertility and obstetrical issues. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis is a rare congenital anomaly. Patient may be asymptomatic and unaware of having double uterus or may present with severe dysmenorrhoea or dyspareunia or a palpable mass due to unilateral haematocolpos. We report a case of 12 year old girl with this condition who was diagnosed as uterus didelphys with unilateral haematocolpos with ipsilateral renal agenesis on the basis of clinical association, physical examination and sonography and intravenous urogram.

Congenital abnormalities of the female urogenital tract are not uncommon, with an estimated incidence of 2-4% across the female population. Within this population, up to 40% will have associated renal tract abnormalities. A previously well 12-year-old girl presented to the emergency department with abdominal pain, vomiting and a palpable pelvic mass. Ultrasound and MR scans were performed. The imaging revealed a didelphys uterus, an obstructed hemivagina and ipsilateral renal agenesis, characteristic of Herlyn-Werner-Wunderlich syndrome. The patient was noted at birth to have a single umbilical artery, which is associated with an increased risk of congenital abnormalities and useful information for the early identification of abnormalities that have implications for renal function and future fertility., (2014 BMJ Publishing Group Ltd.)

LeFort colpocleisis is a simple and effective procedure for pelvic organ prolapse in women who no longer wish to preserve coital function. If the vaginal channels created during this procedure are not large enough, blood or pus may collect within the uterus or proximal vagina. Hematocolpos, accumulation of blood in apical vagina, may be difficult to manage especially when a patient is frail and has medical comorbidities. Here, we present a case of LeFort colpocleisis in which excessive anticoagulation led to an infected hematocolpos and persistent bleeding. This was successfully managed with a Bakri balloon via a vaginal channel without recurrence of her prolapse.

The transverse vaginal septum is one of the rarest anomalies of the reproductive tract classified depending on location. It is manifested by primary amenorrhea, cyclic pain and progressive mass growth at abdominopelvic level, ultrasound and magnetic resonance imaging provides the diagnosis, location and thickness of a transverse vaginal septum, treatment is surgical. We present a case of a teenage patient with primary amenorrhea due to lower transverse vaginal septum with surgical resection and satisfactory follow-up with successful vaginal patency.

Background: Herlyn-Werner-Wunderlich syndrome is a congenital urogenital malformation that is associated with a uterus didelphys and a longitudinal vaginal septum, resulting in a blind hemivagina and ipsilateral renal agenesis. Clinical presentation is highly variable, delaying diagnosis and leading to important complications., Clinical Case: We present the case of a 13-year-old female who was diagnosed with Herlyn-Werner-Wunderlich syndrome following an acute abdomen due to a right tubo-ovarian abscess. She had a vaginal septum giving rise to a right blind hemivagina. It was microperforated, causing intermittent genital bleeding. This hematocolpos was colonized by microorganisms that ascended to the pelvic cavity, causing right tuboovarian abscess. Nuclear magnetic resonance imaging provided theWernermost diagnostic information. We performed a vaginal septum resection, and both hemiuteros communicated with a single vagina, resulting in an asymptomatic patient., Conclusion: Herlyn-Werner-Wunderlich syndrome is a little known entity and can be presented atypically, resulting in diagnostic difficulty and treatment delay. It is important to be aware of this syndrome in order to avoid irreversible complications.

Müllerian duct anomalies are a group of uncommon and underdiagnosed entities, which cause specific symptoms in adolescent females and may be associated with infertility as well as adverse pregnancy outcomes. These malformations occur as a result of an arrest or abnormal development of the Müllerian ducts in different stages of the female reproductive tract during gestation. Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA), formerly known as the Herlyn-Werner-Wunderlich syndrome, is a rare entity characterized by the presence of a uterus didelphys with an obstructed hemivagina cause by a vaginal septum and the association of a renal anomaly (most commonly renal agenesis) ipsilateral to the obstruction. This syndrome may remain undiagnosed during childhood and usually becomes symptomatic after menarche, causing obstructive symptoms. Occasionally it may be identified after the evaluation of a patient with infertility or recurrent pregnancy loss. The clinical diagnosis is very challenging and requires imaging studies in which ultrasound and MRI play an essential role in the diagnosis, classification and treatment plan. Opportune diagnosis and treatment achieve complete improvement of symptoms, adequate reproductive prognosis and avoid major complications such as endometriosis, pelvic adhesions and infertility. The purpose of this review is to demonstrate the pathophysiology, clinical manifestations, diagnostic methods and treatment of the obstructed hemivagina and ipsilateral renal anomaly syndrome.

Background: Herlyn Werner Wunderlich Syndrome (HWWS) is a congenital abnormality of the Müllerian duct system resulting in uterovaginal duplication, obstructive hemivagina, and ipsilateral renal agenesis. It typically presents shortly after menarche with gradual onset of progressive pelvic pain., Case Report: We report a case of a 19-year-old female who presented to the Emergency Department with sudden onset of severe vaginal pain that was determined to be due to hematocolpos; imaging confirmed the diagnosis of HWWS., Conclusions: To the best of our knowledge abrupt onset of vaginal pain due to HWWS has not been reported previously. We present this case to increase awareness among emergency physicians of this rare and interesting entity., (Published by Elsevier Inc.)

Imperforate hymen is a rare condition that presents with amenorrhea, cyclical abdominal pains and urine retention among pubertal girls. A 14 year old girl with imperforate hymen underwent hymenotomy for hematocolpometra, having presented with abdominal pains and tenesmus.

Purpose: The aim of this study was to evaluate the outcome of pregnancies in women who had uterine malformation and surgically treated obstructed hemivagina with ipsilateral renal agenesis., Methods: The study group comprised 21 women with malformed uterus (12 didelphic, 6 septate and 3 bicornuate uterus). All of them had a history of surgical excision of the longitudinal vaginal septum caused obstructed hemivagina and ipsilateral renal agenesis. All pregnancies and possible surgical interventions were evaluated during the follow-up period (median 13.2 years)., Results: Thirteen out of 21 women attempting pregnancy conceived. They produced 22 pregnancies, 17 (77 %) were contralateral to the treated obstructed hemivagina and unilateral renal agenesis. The median interval between surgical treatment of obstructed hemivagina and the first pregnancy was 10.5 years. Twenty (91 %) pregnancies ended in delivery of a living infant. Preeclampsia (14 %), preterm delivery (36 %), high frequency (38 %) of fetal breech presentation and the cesarean section rate (67 %) were found., Conclusions: Accurate diagnosis and appropriate surgery to open an obstructed hemivagina in adolescence assure fertility. Preterm birth is associated with malformed uterus and unilateral renal agenesis may predispose to preeclampsia.

Background: Absence of the lower vagina occurs from abnormal development of the sinovaginal bulbs and vaginal plate., Case: We present a case of an adolescent girl, with a history of VACTERL and VATER status post flap vaginoplasty at 8 m of age and correction of vaginal-urethral fistula, with primary amenorrhea and cyclic pelvic pain. MRI showed a right hematocolpos. On exam the obstructed vagina was deviated to the right. Given prior urologic and rectal surgery, and laterality of unicornuate system, intra-operative ultrasonography (US) was performed. A needle was used to enter the inferior aspect of the obstructed vagina. A pull-through vaginoplasty was performed., Summary and Conclusion: Vaginal pull-through is the standard treatment for lower vaginal agenesis. In a unicornuate system, the use of intra-operative US is helpful to determine the appropriate angle of dissection., (Copyright © 2013 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

This case highlights the importance of careful evaluation of girls presenting with imperforate hymen as this is accompanied by other female reproductive tract anomalies. It is of utmost importance that a correct timely diagnosis is made so that the right treatment can be chosen with the perspective of future fertility. Cervical dysgenesis associated with vaginal septum and imperforate hymen has not been reported in literature so far. Present case highlights the simple mode of management with a successful outcome.

Objectives: To develop a clear diagnostic and therapeutic strategy for adolescents presenting with abdominal pain and vaginal tumor caused by congenital female genital anomalies, such as blind hemivagina and uterine anomalies, as the lack of the correct diagnosis of the underlying anatomical genitourinary malformation frequently leads to destructive surgical procedures., Methods: Retrospective study, study group: patients with double/bicornuate uterus, blind hemivagina and hematocolpos (n = 13), controls: patients with uterine malformation and complete vertical vaginal septum (n = 11), analysis for: menarche, age at onset of symptoms, type of malformation, symptoms leading to admission and diagnostic/surgical techniques applied., Results: Median age at diagnosis study group 19.85 (SD ± 6.23, range 13-23 years) versus controls 26.09 years (SD ± 7.44, 16-36 years); predominance of imperforated hemivagina: 69.2 % right-sided versus 30.8 % left-sided septum; renal agenesis ipsilateral to imperforate hemivagina 100 % study group versus 9.1 % controls; 84.6 % previous surgical interventions in the study group, such as partial removal of the septum and re-obliteration, unilateral salpingo-ovarectomy and vaginal drainage of pyometra. We used a single transvaginal surgical procedure, including removal of the obstructed vaginal septum and marsupialization of the blind hemivagina., Conclusions: A diagnostic and therapeutic algorithm for young women presenting with progressive dysmenorrhea and abdominal pain and/or vaginal tumor reduces destructive interventions.

The presence of both a uterovaginal septum and imperforate hymen is described in a young patient presenting with ongoing chronic pelvic pain and a double pyocolpos. Ultrasound and magnetic resonance imaging scans were performed. The patient underwent laparoscopic adesiolysis, hymenotomy with drainage of 200 mL of pus, and excision of a complete longitudinal vaginal septum. Over the past 5 years of regular follow-up examinations, the patient has always reported regular menstrual cycles and an absence of pelvic pain.

Aim: The aim of this study was to underline the significance of premenarcheal gynecological examination in patients with transverse vaginal septum that could possibly be complicated with endometriosis., Design: Retrospective study including the period between January 2008 and December 2010., Setting: Second Department of Obstetrics and Gynecology., Patients: We searched our databases regarding cases of hematocolpos caused by transverse vaginal septum., Main Outcome: Among the patients presented with hematocolpos we identified 4 cases caused by transverse vaginal septum., Results: We present the management of these cases regarding diagnosis, differential diagnosis, and treatment. The mean age of the patients was 13.1 years. All patients presented in our department with hypogastric abdominal pain and hematocolpos. No problems in adrenarche or thelarche were mentioned. The U/S and MRI revealed a normal cystic in the upper part of the vagina--hematocolpos varying from 42 × 26 × 30 to 73 × 55 × 32 mm. Three of the patients had an upper transverse vaginal septum while one had a middle transverse vaginal septum. Only one patient had a concomitant anomaly of the urinary system (ectopic kidney). In our patients, after laparoscopic examination 3 out of 4 patients had findings of endometriosis (2/3 with stage I-minimal endometriosis and 1/3 with stage II-mild endometriosis)., Conclusion: Physicians should be aware of transverse vaginal septum in the differential diagnosis of hematocolpos with abdominal pain and primary amenorrhea in the early adolescent years. Early diagnosis could be based on premenarcheal gynecological examination and could lead to correct management in order to avoid the complications of endometriosis (dysmenorrhea or infertility).