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DOI: 10.1055/s-0031-1292705
Systematization of female genital anomalies
Background: The experience of clinico-morphological classification considers to optimize the appropriate surgical correction and treatment of female patient with various genital malformations.
Methods: We did a systematic review of 134 full-text articles assessed for female genital original classifications, that found in PubMed, Medline, literature revews.
We had analysis our experience since 2000 to 2011, about surgical correction of 452 patient with various female genital malformations: 345 patients with utero-vaginal anomalies (MRKH syndrome 23, uterus duplex 36, bicornuate 48, and unicornuate uterus 22, partial vaginal aplasia 39, cervico-vaginal atresia 9, septate uterus 126, arcuate uterus 42). And 107 patient with intersexual disorders: Terner syndrome (15), XY gonadal dysgenesis (12), ovotesticular DSD (8), androgen receptor defect (10), adrenogenital syndrome (62).
Results: All the existing classifications were analysed. The American Fertility Society classification of the late 1983s included seven basic groups of malformations also considering the anatomical defects: Vagina, Cervix, Uterus, Adnex and Associated Malformation.
European Consensus Statement (2006) outlining the recommendations for the management of disorders of sex development, and new nomenclature of DSD classification system. There is mullerian malformations without of clinico-morphological descriptions.
Clinical analysis after surgical treatment showed: in 21% cases gonadal disgenesis compared with chromosomal abnormalities; 8% patient with intersexual disorders had mullerian anomalies.
Optimal surgical treatment (adnexectomy, gonadal biopsy, metroplasty, feminisation plastic) depends on clinical data and exactly correct diagnosed anatomical defects, included: gonadal dysgenesis, virilisation degree of external genitalia.
Conclusions: We proposed an update clinico-morphological classification, allows for surgical correction of various female genital malformations, include disorders of sex development and uterovaginal anomalies.