Pedro Acién1, Maribel Acién, Marisa Sánchez-Ferrer. 1. Service of Obstetrics and Gynecology, San Juan University Hospital and Department/Division of Gynecology, School of Medicine, Miguel Hernández University, Campus of San Juan, Alicante, Spain. acien@umh.es
Abstract
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 symptoms 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.
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 symptoms 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.
Authors: Bettina Toth; Dunja Maria Baston-Büst; Hermann M Behre; Alexandra Bielfeld; Michael Bohlmann; Kai Bühling; Ralf Dittrich; Maren Goeckenjan; Katharina Hancke; Sabine Kliesch; Frank-Michael Köhn; Jan Krüssel; Ruben Kuon; Jana Liebenthron; Frank Nawroth; Verena Nordhoff; Germar-Michael Pinggera; Nina Rogenhofer; Sabine Rudnik-Schöneborn; Hans-Christian Schuppe; Andreas Schüring; Vanadin Seifert-Klauss; Thomas Strowitzki; Frank Tüttelmann; Kilian Vomstein; Ludwig Wildt; Tewes Wischmann; Dorothea Wunder; Johannes Zschocke Journal: Geburtshilfe Frauenheilkd Date: 2019-12-11 Impact factor: 2.915
Authors: Pedro Acién; Francisco Galán; Irene Manchón; Eva Ruiz; Maribel Acién; Luis A Alcaraz Journal: Orphanet J Rare Dis Date: 2010-04-14 Impact factor: 4.123