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.

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.

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.

[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.

Rare clinical presentation of genital tuberculosis in an adolescent girl.

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.

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.

A uterovaginal septum and imperforate hymen with a double pyocolpos.

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.

Urinary retention in an adolescent with a history of urethral transection.

BACKGROUND: Urinary retention is rare in adolescence. Here I describe such a case in a patient who had transected her urethra in a motor vehicle accident at 3 years of age. The presumptive diagnosis of urethral stricture was proven incorrect. OBJECTIVES: Both to present a complicated and unique case whose history pointed strongly to one diagnosis initially but turned out very differently than expected; and to give an example of the use of bedside ultrasonography in the Emergency Department as a diagnostic tool in an unusual circumstance. CASE REPORT: A 13-year-old girl was evaluated for 3 days of urinary retention. Her history of pelvic fracture and urethral transection in childhood suggested a urethral stricture. Her work-up revealed, however, a vaginal stricture and resultant hematocolpos compressing the urethra. Upon surgical excision, the stricture was confirmed to be scar tissue, rather than a coincidental congenital septum. CONCLUSION: Hematocolpos is an uncommon but well-described cause of urinary retention in premenarchal females. This is a difficult diagnosis in any circumstance, and this patient's complex history complicated the process. It was the bizarre bedside ultrasound that prompted further work-up than simple urethral catheterization and urologic follow-up, which her urologist had suggested over the phone. This patient's case is unique in the literature.

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.

A novel image-guided balloon vaginoplasty method to treat obstructive vaginal anomalies.

Obstructive anomalies of the vagina that impair uterovaginal outflow and lead to hematocolpos have conventionally been corrected with surgery, but emerging nonsurgical therapies may offer an attractive alternative. The present report describes a method of serial balloon dilation over a transvaginally inserted guide wire to create a durable outflow tract from the uterus to the lower vagina. This technique was successfully used to treat one case each of vaginal atresia and transverse vaginal septum without the immediate need for surgery.

Congenital imperforate hymen with bilateral hydronephrosis, polydactyly and laryngocele: A rare neonatal presentation.

Imperforate hymen is a rare congenital malformation to present with symptoms in the neonatal period. We present a case of a 5-day-old neonate with a marked interlabial swelling causing urinary retention. Imaging revealed hydrometrocolpos, hydrosalpinx, and bilateral hydronephrosis. Additionally, our patient had polydactyly of both feet and hands, and laryngocele that have not been previously described in literature. Hymenectomy is the mainstay of management to prevent the development of hematocolpos, pain, and possible retrograde menstruation.

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.

An unusual cause of lower abdominal pain in pubertal girls.

A rare cause of acute lower abdominal pain in pubertal girls is described. The diagnosis is often missed at initial presentation and this may result in multiple presentations to the emergency department or general practitioner. The clinical features, diagnosis, management and possible complications of this condition are discussed. The case illustrates the importance of keeping this diagnosis in mind when seeing teenage girls with lower abdominal pain.

[The role of MRI in congenital cystic lesions in the pelvis: a case of uterus didelphys with double vagina, hematocolpos, and ipsilateral renal agenesis.]. / Lesión quística congénita en la pelvis: un caso de útero didelfo con doble vagina, hematocolpos y agenesia renal ipsilateral: papel de la resonancia magnética.

We report a case of uterus didelphys with blind hemivagina and ipsilateral renal agenesis. This is an uncommon anomaly of the development of the Mullerian ducts in which a defect in one of the Wolffian ducts leads to failed induction in kidney formation and in the fusion of the Mullerian ducts. MRI plays a decisive role in the diagnosis and characterization of the malformation.

Vaginal masses: magnetic resonance imaging features with pathologic correlation.

The detection of vaginal lesions has increased with the expanding use of cross-sectional imaging. Magnetic resonance imaging (MRI) - with its high-contrast resolution and multiplanar capabilities - is often useful for characterizing vaginal masses. Vaginal masses can be classified as congenital, inflammatory, cystic (benign), and neoplastic (benign or malignant) in etiology. Recognition of the typical MR imaging features of such lesions is important because it often determines the treatment approach and may obviate surgery. Finally, vaginal MR imaging can be used to evaluate post-treatment changes related to previous surgery and radiation therapy. In this article, we will review pertinent vaginal anatomy, vaginal and pelvic MRI technique, and the MRI features of a variety of vaginal lesions with pathological correlation.