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      Clinical features of congenital complete vaginal atresia combined with cervical aplasia: A retrospective study of 19 patients and literature review

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          Abstract

          Congenital vaginal atresia is a rare congenital reproductive tract abnormality. To assess the clinical manifestations and feasibility of preserving uterus for congenital complete vaginal atresia with cervical aplasia, nineteen cases who underwent surgical treatment in West China Second Hospital of Sichuan University were retrospectively studied. The cervical status, clinical manifestations, the rate of vaginal re‐stenosis and pelvic inflammation after surgery were assessed. Additional 101 similar cases searched through digital Pub Med were included to analyze the feasibility of preserving the uterus. Periodic abdominal pain, primary amenorrhea, and pelvic mass were the primary signs and symptoms. According to the magnetic resonance imaging (MRI), all the uterine cavities expanded, and the atresia sites were above the inner urethral orifice. Data of the cases preserving uteri from both our hospital and the literature showed the rate of re‐stenosis in patients with external cervical obstruction was 15.9% while it was 40% in the other types of cervical aplasia ( P = .026). The rate of recurrent pelvic inflammation and hysterectomy was 2.3% for cervical external os obstruction and 8% for the other cervical aplasia types( P = .296). In conclusion, vaginoplasty and cervicovaginal anastomosis could preserve the fertility for complete vaginal atresia with cervical external os obstruction.

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          The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies.

          What classification system is more suitable for the accurate, clear, simple and related to the clinical management categorization of female genital anomalies?
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            The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions.

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              The history of female genital tract malformation classifications and proposal of an updated system.

              A correct classification of malformations of the female genital tract is essential to prevent unnecessary and inadequate surgical operations and to compare reproductive results. An ideal classification system should be based on aetiopathogenesis and should suggest the appropriate therapeutic strategy. We conducted a systematic review of relevant articles found in PubMed, Scopus, Scirus and ISI webknowledge, and analysis of historical collections of 'female genital malformations' and 'classifications'. Of 124 full-text articles assessed for eligibility, 64 were included because they contained original general, partial or modified classifications. All the existing classifications were analysed and grouped. The unification of terms and concepts was also analysed. Traditionally, malformations of the female genital tract have been catalogued and classified as Müllerian malformations due to agenesis, lack of fusion, the absence of resorption and lack of posterior development of the Müllerian ducts. The American Fertility Society classification of the late 1980s included seven basic groups of malformations also considering the Müllerian development and the relationship of the malformations to fertility. Other classifications are based on different aspects: functional, defects in vertical fusion, embryological or anatomical (Vagina, Cervix, Uterus, Adnex and Associated Malformation: VCUAM classification). However, an embryological-clinical classification system seems to be the most appropriate. Accepting the need for a new classification system of genitourinary malformations that considers the experience gained from the application of the current classification systems, the aetiopathogenesis and that also suggests the appropriate treatment, we proposed an update of our embryological-clinical classification as a new system with six groups of female genitourinary anomalies.
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                Author and article information

                Contributors
                2729493428@qq.com
                Journal
                Congenit Anom (Kyoto)
                Congenit Anom (Kyoto)
                10.1111/(ISSN)1741-4520
                CGA
                Congenital Anomalies
                John Wiley & Sons Australia, Ltd (Kyoto, Japan )
                0914-3505
                1741-4520
                04 May 2021
                July 2021
                : 61
                : 4 ( doiID: 10.1111/cga.v61.4 )
                : 127-132
                Affiliations
                [ 1 ] Gynecologic Department West China Second Hospital of Sichuan University Chengdu Sichuan China
                [ 2 ] Center for Translational Medicine Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, West China Second Hospital of Sichuan University Chengdu Sichuan China
                [ 3 ] Department of Radiology West China Second Hospital of Sichuan University Chengdu Sichuan China
                Author notes
                [*] [* ] Correspondence

                Xiaoyu Niu MD, Gynecologic Department, West China Second Hospital of Sichuan University, Address: No. 20, Section 3, South Renmin Road, Chengdu, Sichuan Province, China.

                Email: 2729493428@ 123456qq.com

                Author information
                https://orcid.org/0000-0001-7061-6757
                Article
                CGA12417
                10.1111/cga.12417
                8360086
                33829535
                aa767c86-f756-4142-ac15-c8e0d1550540
                © 2021 The Authors. Congenital Anomalies published by John Wiley & Sons Australia, Ltd on behalf of Japanese Teratology Society

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                : 03 March 2021
                : 04 January 2021
                : 30 March 2021
                Page count
                Figures: 4, Tables: 2, Pages: 6, Words: 3927
                Categories
                Original Article
                Original Articles
                Custom metadata
                2.0
                July 2021
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.0.5 mode:remove_FC converted:12.08.2021

                cervical aplasia,cervicovaginal anastomosis,congenital complete vaginal atresia,tracheloplasty,vaginoplasty

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