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Hum. Reprod. Advance Access originally published online on August 27, 2004
Human Reproduction 2004 19(10):2377-2384; doi:10.1093/humrep/deh423
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Human Reproduction vol. 19 no. 10 © European Society of Human Reproduction and Embryology 2004; all rights reserved

Complex malformations of the female genital tract. New types and revision of classification

Pedro Acién1,2,*, Maribel Acién2 and Marisa Sánchez-Ferrer1

1 Service of Obstetrics and Gynecology, San Juan University Hospital and 2 Department/Division of Gynecology, School of Medicine, ‘Miguel Hernández’ University, Campus of San Juan, Alicante, Spain

* To whom correspondence should be addressed at: Departamento/División de Ginecología, Facultad de Medicina de la Universidad ‘Miguel Hernández’, Campus de San Juan, 03550 Alicante, Spain. Tel.: 965919524; Fax: 965919550; Email: acien{at}umh.es

BACKGROUND: Complex malformations of the female genital tract are often incorrectly identified, treated and reported, probably due to not considering the malformation as a cause of the clinical symptoms and neither the embryological origin of the different elements of the genitourinary tract. METHODS: Complex malformations are studied and classified, and new types are presented. The new types of complex malformations presented are: (i) Cases of unilateral vaginal or cervico-vaginal atresia with renal agenesis and uterine duplication, with or without communication between hemiuteri; (ii) the unilateral Rokitansky syndrome; and (iii) the combination in the same patient of unilateral Rokitansky syndrome (Müllerian defect) on one side and blind vagina and ipsilateral renal agenesis syndrome (Wolffian defect) on the other side. RESULTS: A revised version of the clinical and embryological classification of genital malformations is presented and an associated diagram points out the origin of these malformations. CONCLUSIONS: These genital malformative anomalies reaffirm our hypothesis about the embryology of the human vagina as deriving from the Wolffian ducts and the Müllerian tubercle; and they show that gynecologists should be aware of the related symptons and the embryology of the female genital tract in order to achieve a better comprehension of the malformations for their right correction or therapeutic approach.

Key words: blind vagina/cervico-vaginal atresia/genital malformations/renal agenesis/Rokitansky syndrome


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