OHVIRA-syndrome (Obstructed hemivagina with ipsilateral renal anomaly) as differential diagnosis of acute lower abdominal pain.

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.

Distal vaginal agenesis and differential diagnosis of other causes of hematocolpos.

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.

Hymenal atresia - a rare congenital anomaly with the risk of late diagnosis.

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.

Aqueous vaginal contrast and scheduled hematocolpos with magnetic resonance imaging to delineate complex müllerian anomalies.

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.

Himen imperforado con hematocolpos. A propósito de un caso / Imperforate hymen with hematocolpos. Apropos of a case

RESUMEN Los defectos en la canalización de la membrana himeneal causan obstrucción del tracto genital femenino. Como consecuencia aparece hematocolpos, resultante de la acumulación y retención de secreciones cervicovaginales -sangre en útero y vagina-, ante la imposibilidad de su evacuación por la presencia de un himen imperforado. Clínicamente aparece masa abdominal, asociada con malformaciones vaginales congénitas. Se presentó el caso de una adolescente de 13 años que refirió retención urinaria, disuria, dolor y masa en hipogastrio, y ausencia de la menarquía a pesar de un desarrollo puberal en estadio Tanner III. Al realizar ultrasonido se constató sangre en el útero, debido a imperforación himeneal que se resolvió mediante himenotomía. La patología de himen imperforado con hematocolpos debe estar entre los posibles planteamientos sindrómicos de aquellas pacientes con cuadros similares; puede diagnosticarse en la Atención Primaria de Salud a partir de una detallada anamnesis y exploración física, aunque la comprobación requiere ecografía (AU).

ABSTRACT The defects in the channeling of the himeneal membrane cause obstruction in the female genital tract. As consequence hematocolpos appears, resulting from the accumulation and retention of cervicovaginal secretions -blood in the uterus and vagina-, due to the impossibility of its evacuation because of the presence of an imperforate hymen. Clinically, abdominal mass appears associated to congenital vaginal malformations. We presented the case of teenager aged 13 years that referred urine retention, dysuria, pain, had a mass in the hypogastrium, and absence of menarche in spite of a Tanner III pubertal development. An ultrasound showed blood in the uterus due to hymeneal imperforation solved through hymenotomy. The pathology of imperforate hymen with hematocolpos should be among the possible syndrome considerations in those patients with the same characteristics; it can be diagnosed in the primary health care from anamnesis and physical exploration, although the verification requires an ultrasound (AU).

Lumbalgia persistente como única manifestación clínica de hematocolpos / Persistent low back pain as the only clinical symptom of hematocolpos

Las lumbalgias son frecuentes en niños y adolescentes. La mayoría tienen un origen mecánico benigno y transitorio. Se presenta el caso de una paciente de 12 años con lumbalgia persistente como único síntoma de hematocolpos por himen imperforado. La himenectomía resolvió totalmente la lumbalgia. Por su rareza, los pediatras no solemos pensar en esta posibilidad por lo que proponemos que ante una adolescente con lumbalgia persistente y amenorrea primaria aparente es necesario valorar el diagnóstico de hematocolpos por himen imperforado

Low back pain is common in children and adolescents. Most of those cases have a benign, transitory and mechanical origin. We present the case of a 12-year-old patient with persistent low back pain as the only symptom of hematocolpos due to imperforate hymen. The hymenectomy completely resolved the low back pain. Due to its rarity, pediatricians do not usually think about this possibility, so we propose that in a case of an adolescent with an adolescent with persistent low back pain and apparent primary amenorrhea, it is necessary to think about the diagnosis of hematocolpos due to imperforate hymen

Herlyn-Werner-Wünderlich syndrome: An unusual case with presentation of menorrhagia.

OBJECTIVE: Herlyn-Werner-Wünderlich (HWW) syndrome is a rare condition in which patients present with a palpable pelvic mass and pain caused by an obstructed hemivagina. Here we present a case of HWW syndrome characterized by prolonged menstrual bleeding. CASE REPORT: A 19-year-old nonsexually active unmarried women experienced irregular menstrual cycles and menorrhagia. The duration of menstrual bleeding was 10-14 days. She also suffered from mild dysmenorrhea since menarche at the age of 13. Transabdominal sonography revealed a double uterus and a heterogeneous myoma-mimicking mass over the left cervical region. The left kidney was absent. Magnetic resonance imaging revealed a double uterus, a double vagina with an unperforated left hemivagina, and ipsilateral renal agenesis. The patient underwent cervicovaginal orifice reconstruction surgery. CONCLUSION: Left hematocolpos compression, a partially obstructed right vaginal channel, and an orifice with local venous drainage abnormalities resulted in prolonged menstrual bleeding. In HWW syndrome, the occurrence of a pelvic mass and pain is common; however, prolonged menstrual bleeding is rare.

Missed diagnosis of imperforate hymen later presenting with hypertension.

An adolescent girl presented with hypertension and was found to have haematocolpos and imperforate hymen. She had a background of chronic abdominal pain and had sought medical attention multiple times prior, with the diagnosis being missed as pubertal evaluation and perineal examination had been neglected during those visits. Hypertension resolved following hymenectomy and drainage of haematocolpos with no long-term sequelae.

Cyclical Abdominal Pain in an Adolescent Female: A Case Report of Agenesis of the Lower Vagina.

Congenital agenesis of the lower vagina is a rare disorder characterized by separation between the unaffected proximal vagina and the distal vagina by a band of fibrous tissue. The typical presentation is an early adolescent female with chronic, cyclic abdominal pain and primary amenorrhea. In this case report, we describe an adolescent who presented to the pediatric emergency department on 2 occasions with a chief complaint of lower abdominal pain.

[Hematocolpos : an unappreciated diagnosis of hymen imperforation]. / Le cas clinique du mois. Hématocolpos : un diagnostic méconnu d'imperforation hyménéale.

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.

L'imperforation de l'hymen est une malformation congénitale relativement rare. L'hématocolpos, qui désigne l'accumulation progressive de sang menstruel dans la cavité vaginale, en est la manifestation la plus fréquente à l'adolescence. Il se traduit cliniquement par une cryptoménorrhée douloureuse et, plus rarement, par un syndrome de masse pelvien. Le diagnostic est facile et, avant tout, clinique. L'échographie et la résonance magnétique nucléaire sont les examens complémentaires de choix pour déceler d'éventuelles malformations génito-urinaires associées. Le traitement consiste en une hyménéotomie.

Atresia Hymenalis with Haematometrocolpos: A Benefit of Teaching a Case Series and Review of the Literature.

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

Síndrome de Herlyn-Werner-Wunderlich. Síndrome OHVIRA. Reporte de Caso / Herlyn-Werner-Wunderlich Syndrome or OHVIRA Syndrome. Case Report

Paciente Femenina de 11 años con el síndrome de Herlyn-Werner-Wunderlich, manejado multdisciplinariamente y resuelto endoscópicamente con una septotomía y dilataciones vaginales.

This case is about a female patent, 11 years old, with Herlyn-Werner-Wunderlich Syndrome, who received multdisciplinary approach and was endoscopically resolved with septotomy and vaginal dilatatons.

[Magnetic resonance imaging as supplement to clinical examination by suspicion of haematocolpos]. / MR-skanning som supplement til klinisk undersøgelse ved mistanke om hæmatokolpos.

Haematocolpos is a rare condition in young women that could be caused by imperforated hymen, vaginal atresia and vaginal agenesis. Haematocolpos is usually diagnosed at early puberty with unspecified abdominal pain and amenorrhoea. Three cases are presented. In one case the radiological examination showed incidentally a urogenital congenital anomaly known as Herlyn-Werner-Wunderlich syndrome. A gynaecological examination should be performed to confirm the presence of imperforated hymen, but in cases in which other pathology is suspected ultrasound and MRI should also be performed.

Herlyn-Werner-Wunderlich syndrome: merits of sonographic and magnetic resonance imaging for accurate diagnosis and patient management in 13 cases.

STUDY OBJECTIVE: To review the main sonographic and magnetic resonance imaging manifestations of Herlyn-Werner-Wunderlich syndrome, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, and to discuss the value of techniques for its diagnosis and treatment. PARTICIPANTS: Thirteen patients with Herlyn-Werner-Wunderlich syndrome referred to our department with acute abdomen were identified for the period from 2009-2012. DESIGN: Retrospective chart review. SETTING: Two tertiary academic centers. INTERVENTIONS: None. MAIN OUTCOME MEASURES: The evaluation of the clinical features, ultrasonography and magnetic resonance imaging findings, and the treatment protocols. RESULTS: Ultrasonographic evaluation of didelphic or double uterus in 10 and bicornuate-bicollis uterus in 3 patients with hematocolpos or hematometrocolpos associated with agenesis of the ipsilateral kidney revealed the diagnosis. In 10 patients, magnetic resonance imaging provided extra information. In 1 patient, a 25-week pregnancy was additionally detected and follow-up was suggested. Seven patients underwent surgical excision of the septum and drainage of the obstructed vagina. Total hysterectomy and hemicolpectomy were performed in 1 patient with 2 children suffering from severe chronic pelvic pain due to pelvic inflammatory disease. CONCLUSIONS: Despite its rarity, accurate diagnosis and morphologic description of Herlyn-Werner-Wunderlich syndrome with radiologic modalities are of importance in relieving the clinical complaints by enabling the suitable surgical treatment to be identified.

Scoliosis in Herlyn-Werner-Wunderlich syndrome: a case report and literature review.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a congenital Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Little is reported about spinal deformity associated with this syndrome. This study presents a case of scoliosis occurring in the setting of HWWS and explores the possible association between the 2 diseases. A previously unreported scoliosis in HWWS is described. The patient is a 12-year-old Chinese female with scoliosis that underwent a posterior correction at thoracic 5-thoracic 12 (T5-T12) levels, using the Moss-SI (Johnson & Johnson, American) spinal system. At 24-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. Six months after scoliosis correction surgery, the patient went to our clinics for the treatment of HWWS. She was performed a vaginal septum resection and detected with pyocolpos. Her follow-up was symptom free at the fourth postoperative month. The prevalence of scoliosis among patients with HWWS was 8.57% that is much higher than the incidence of congential scoliosis among general population (1/1000). To the best of our knowledge, this is the first report of HWWS with thoracic scoliosis. During surgery, surgeons and anesthesiologists must pay particular attention to the Müllerian duct anomaly and renal agenesis associated with HWWS. There is a potential association between congenital scoliosis and HWWS.

Congenital abnormalities of the urogenital tract: the clue is in the cord?

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.