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.

Beyond hemostasis: the challenge of treating plasminogen deficiency. A report of three cases.

Congenital plasminogen deficiency is a rare autosomal recessive disorder, characterized by chronic mucosal membranous lesions. Although the most common clinical manifestation is eye involvement as ligneous conjunctivitis, extra-ocular lesions affecting other mucosal surfaces indicates a systemic disease. In this report we describe two cases with atypical extra-ocular involvement that includes pericarditis and recurrent hematocolpos, and one with paradoxical correlation between ocular lesions and plasminogen levels. In ligneous conjunctivitis, although different treatment strategies have been tried with mild success, the only effective therapy is topical or systemic plasminogen concentrates that are not commercially available. Unfortunately there is not either effective management for cases with multisystemic disease. Hence, treatment for plasminogen deficiency is still a challenge and the variability of the clinical spectrum in this pathology makes necessary a multidisciplinary approach.

Cervical dysgenesis with transverse vaginal septum with imperforate hymen in an 11 year old girl presenting with acute abdomen.

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.

Uterus didelphys with partial vaginal septum and distal vaginal agenesis: an unusual anomaly.

Complex malformations of female genital tract are not so common. Their correct identification is of paramount importance for appropriate management. A thorough knowledge of embryology, pre-operative imaging with MRI and examination under anaesthesia is essential to identify accurately the constellation of anomalies and to plan appropriate management. This case reports the coexistence of Müllerian abnormality with vaginal agenesis in an 18 years old girl which was managed by dissecting the lower half of vagina and pull-through vaginoplasty.

Himen imperforado / Imperforate hymen

El himen imperforado es una anomalía obstructiva del tracto genital inferior femenino que se da en menos del 0,1% de los recién nacidos. El hecho de no realizar una exploración ginecológica a adolescentes sintomáticas por pudor conlleva frecuentemente al retraso diagnóstico. Presentamos a una paciente adolescente sin menarquia remitida a urgencias para estudio de dolor abdominal cíclico asociado a una masa abdominal con retención de orina y estreñimiento. La simple inspección de los genitales externos nos permitió realizar el diagnóstico después de 2 años de sintomatología(AU)

Imperforate hymen is an obstructive anomaly of the female reproductive tract which occurs in less than 0.1% of newborns. Not performing an adequate gynecologic physical examination in symptomatic adolescent girls due to embarrassment can often delay its diagnosis. We present the case of an adolescent girl without menarche who was referred to our hospital for cyclic abdominal pain associated with an abdominal mass accompanied by urinary retention and constipation. Examination of her external genitalia allowed rapid diagnosis after a 2-year history of these symptoms(AU)

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.

Hematocolpos-hematometra por imperforación himeneal / Haematocolpos-haematometra for imperforated hymen

Se han revisado las circunstancias del descubrimiento de un hematocolpos, los aspectos clínicos y las exploraciones complementarias. Los hematocolpos por imperforación himeneal son tratados por simple incisión estrellada con respeto de los orificios de las glándulas de Bartholino y separación meatohimeneal. Los hematocolpos como consecuencia de una duplicación con una hemivagina ciega son de más difícil tratamiento. El cribado sistemático al nacimiento y un tratamiento poco después del nacimiento se han propuesto para prevenir esta afección de la pubertad (AU)

[Diagnostic image (15). Hematocolpus due to atresia of the hymen]. / Diagnose in beeld (15). Haematocolpos door hymenale atresie.

In a 14-year-old girl who suffered from anuria and abdominal pain, atresia of the hymen was diagnosed.

[Possibilities of echography in the diagnosis of didelphia of the uterus and double vagina with unilateral disorder of the outflow of menstrual blood]. / Vozmozhnosti ékhografii v diagnostike udvoeniia matki i vlagalishcha s odnostoronnim narusheniem ottoka menstrual'noi krovi.

A study in 30 patients with an obstructed rudimentary vagina has demonstrated that sonography offers an early diagnosis and adequate management of patients with unilateral menstrual flow obstruction. It prevents an infection of the internal genitalia and results in complete reproductive rehabilitation of the patients. Renal sonography indirectly confirms the diagnosis since an aplastic kidney is located on the side of the accessory obstructed vagina in 100% of these patients.

[Large colpohematometra with bilateral hematosalpinx resulting from progressive obstruction caused by incomplete vaginal septum]. / Voluminoso colpoematometra con ematosalpinge bilaterale da ostruzione progressiva di setto vaginale incompleto.

Complete or incomplete transverse vaginal septum is a rare malformation of the female genital tract. Usually the complete congenital type occurs at puberty because of the collection of menstrual blood above the septum with amenorrhea and cyclic lower abdominal pain as presenting symptoms. On the contrary, in the case shown by the authors, the subacute epilogue occurred in the perimenopausal phase: a very large colpohematometra is reported in a 49 years old woman, with an incomplete vaginal septum resulting in progressive obstruction. The association between this malformation and the presence of endometriotic localizations in the genital tract, as reported by other authors, is interesting. In this case, endometriosis can be secondary to the presence of the septum or could have determined the impairment of the obstruction in consequence of the associated status of chronic flogosis.

[A case of Mayer-Rokitansky syndrome].

The Mayer-Rokitansky syndrome has been generally defined as a congenital absence of the vagina in genotypic and phenotypic female subjects with normal endocrine status. Many authors have reported that urological anomalies associate commonly with this syndrome, but recently a new concept of this syndrome was proposed by Tarry and associates. They considered the embryogenesis of the Müllerian system and recognized a spectrum of Müllerian anomalies without total vaginal agenesis as this syndrome. They also proposed a new classification of this syndrome (Müllerian grade) considering grade of Müllerian anomaly. We experienced a 14-year-old girl who had uterus didelphys, left hematocolpos due to ipsilateral vaginal agenesis and agenesis of the left kidney, and herein report this case as Mayer-Rokitansky syndrome. In addition to Tarry's classification, we propose another embryologic classification for Müllerian anomalies considering the period of faulty differentiation in this syndrome: type A-faulty differentiation of the mesonephros (before week 4), type B-faulty differentiation of the mesonephric duct (week 4 to 5), type C- faulty differentiation of the paramesonephric duct only (after week 6).