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Vaginal Tract Anomalies
Published in S Paige Hertweck, Maggie L Dwiggins, Clinical Protocols in Pediatric and Adolescent Gynecology, 2022
Typically, obstructive vaginal anomalies are not surgical emergencies and the complexity of these conditions in combination with the risk of operative complications requires referral to a center with expertise in the management of these disorders.
Congenital anomalies of the reproductive tract
Published in Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo, Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
Maggie Dwiggins, Veronica Gomez-Lobo
Incomplete atresia of the Müllerian ducts may result in partial vaginal agenesis (Figure 6.4). As with other vaginal anomalies, the incidence is approximately 1:4000 female births.23 They often present with increasing cyclic pain and a hematocolpos without bulging at the introitus. Surgical repair can be accomplished by vaginal pull-through procedures. The introitus is dissected below the urethra, and the areolar space between the urethra and rectum is bluntly dissected until the bulging vagina is encountered. The vagina is then grasped, dissected from the surrounding tissues, and “pulled through” to the introitus.24,25 The bulge is then entered and sutured to the mucosa at the initial incision (Figure 6.5). It is important to ascertain the distance from the hematocolpos to the introitus, as a distance of greater than 3 cm is associated with greater stricture rate.15 When the distance from the introitus to the hematocolpos is significant (>4 cm), tissues such as skin, buccal mucosa, or bowel have been used to bridge this gap.22 Postoperative stricture is again common, and follow-up for pain and reobstruction should be performed regularly.24
Congenital uterine anomalies and recurrent implantation failure
Published in Efstratios M. Kolibianakis, Christos A. Venetis, Recurrent Implantation Failure, 2019
Grigoris F. Grimbizis, Sara Y. Brucker, Rudi Campo
Recently, the European Society for Human Reproduction and Embryology (ESHRE) and the European Society for Gynaecological Endoscopy (ESGE) published a new classification of female genital anomalies, with clear anatomical definitions of the categories, giving the opportunity to evaluate their clinical impact; uterine, cervical, and vaginal anomalies were classified in independent categories.5,6 Furthermore, the two societies published a consensus for their diagnosis as an additional tool to clinicians and researchers to avoid subjectivity in their management.7,8 Uterine anomalies were categorized into five classes: class I, dysmorphic uterus (including T-shaped and infantilis uteri); class II, septate uterus (absorption defects/arcuate deleted); class III, bicorporeal uterus (fusion defects/including former didelphys and bicornuate uteri); class IV, hemi of unicorporeal uterus (formation defect/former unicornuate uterus); and class V, aplastic uterus (formation defect/including only cases of uterine aplasia).5,6
Disorders or Differences of Sex Development? Views of Affected Individuals on DSD Terminology
Published in The Journal of Sex Research, 2021
Elena Bennecke, Birgit Köhler, Robert Röhle, Ute Thyen, Katharina Gehrmann, Peter Lee, Anna Nordenström, Peggy Cohen-Kettenis, Clair Bouvattier, Claudia Wiesemann
According to the Chicago consensus, conditions can be classified into three groups depending on karyotype and pathogenesis: sex chromosome DSD, XY DSD and XX DSD. Sex chromosome DSD includes mixed gonadal dysgenesis (46,XY/45,XO), 46,XY/46,XX conditions as well as Turner syndrome and Klinefelter syndrome. XY DSD incorporates XY gonadal dysgenesis, androgen insensitivity syndrome (AIS), disorders of androgen synthesis, disorders of AMH synthesis and action, and severe hypospadias. The XX DSD category includes congenital adrenal hyperplasia (CAH), XX gonadal dysgenesis, and uterine and vaginal anomalies. Thus, the umbrella term Disorders of Sex Development includes conditions with diverse genetic etiology, varying levels of prenatal androgen effects, and varying phenotypes of genitalia (Jürgensen et al., 2010). It is recognized that this is not an ideal classification as, for example, ovotesticular DSD karyotypes vary to a large degree and may be listed within any of the three categories.
The accuracy of three-dimensional ultrasonography in the diagnosis of Müllerian duct anomalies and its concordance with magnetic resonance imaging
Published in Journal of Obstetrics and Gynaecology, 2022
Yasin Ertug Cekdemir, Uygar Mutlu, Demet Acar, Canan Altay, Mustafa Secil, Omer Erbil Dogan
There is a need for consensus and standardisation of classification systems. The ESHRE/ESGE classification yielded effective objective parameters for categorisation of MDAs and allows a classification of MDAs based on the female genital tract anatomy (Kougioumtsidou et al. 2019). Moreover, the ESHRE/ESGE classification was supposed to cover the majority of the uterine, cervical and vaginal anomalies whereas the ASRM classification performed suboptimal in the description of cervical and vaginal anomalies (Heinonen 2016). Even though the arcuate uterus of ASRM may be sometimes considered as U0 with ESHRE/ESGE classification system as well, we imply that acceptance of arcuate uterus of ASRM as U2a with ESHRE/ESGE classification system is more suitable (Sadek et al. 2016).