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Septate uterus with complete vaginal agenesis: an undescribed Müllerian malformation

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Marotta, Maria-Laura [UCL] Donnez, Jacques [UCL] Michaux, Nathalie [UCL] Dupont, Michaël [UCL] Donnez, Olivier [UCL]

Müllerian malformations are the consequence of failure in the fusion or resorption processes of the two Müllerian ducts at around 9 weeks of gestation. The mean prevalence in the general population is as high as 7 %. Vaginal agenesis is a rare female congenital tract anomaly. Diagnosis is often made in adolescence due to amenorrhea or cyclic abdominal pain, and its incidence is around 1 in 4,000 female births. Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is the most frequent manifestation, with a vaginal dimple of varying depth (2–7 cm) and sometimes a rudimentary uterus or horn. In this particular case, the goal of treatment (surgical or nonsurgical) is to allow normal sexual intercourse, but restoring fertility is not possible. Different approaches have been described, such as Ingram’s modification of the Frank technique, the McIndoe procedure, the Vecchietti method, and the Williams vaginoplasty with its Creatsas modification. Here, we report the first case of complete vaginal agenesis associated with a septate uterus to be described in the literature.

Document type Article de périodique (Journal article) – Article de recherche
Access type Accès restreint
Publication date 2013
Language Anglais
Journal information "Gynecological Surgery : endoscopy, imaging, and allied techniques" - Vol. 10, no. 1, p. 87-90 (2013)
Peer reviewed yes
Publisher Springer-Verlag ((Germany) Berlin)
issn 1613-2076
e-issn 1613-2084
Publication status Publié
Affiliations UCL - SSS/IREC/GYNE - Pôle de Gynécologie
UCL - (MGD) Service de gynécologie - fécondation in vitro
UCL - (MGD) Service de radiologie - résonance magnétique
UCL - (SLuc) Service de gynécologie et d'andrologie
Links
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Bibliographic reference Marotta, Maria-Laura ; Donnez, Jacques ; Michaux, Nathalie ; Dupont, Michaël ; Donnez, Olivier. Septate uterus with complete vaginal agenesis: an undescribed Müllerian malformation. In: Gynecological Surgery : endoscopy, imaging, and allied techniques, Vol. 10, no. 1, p. 87-90 (2013)
Permanent URL http://hdl.handle.net/2078.1/121400