The Mayer-Rokitansky syndrome. Report of 5 cases.
Five cases of uterus didelphys, unilateral imperforate vagina and ipsilateral renal agenesis are reported with a follow-up from 1 to 5 years. Diagnosis included typical revelation at puberty (2 cases) but also in the first months of life (2 cases). One case was only diagnosed at the age of 6 years, and the presence of an ectopic ureter was noted in the imperforate vagina. The authors point out the necessity of precise evaluation and careful management in order to maintain an intact reproductory tract. Simple excision of the septum is recommended which can most of the time be done by the vaginal approach.
Guys
J M
JM
Department of Pediatric Surgery, CHU Timone, Marseille, France.
Borella
F
F
Panuel
M
M
Coquet
M
M
Monfort
G
G
D000293:Adolescent; D002648:Child; D005260:Female; D006399:Hematocolpos / Q000209:etiology; D006409:Hematometra / Q000209:etiology; D006801:Humans; D007668:Kidney / Q000002:abnormalities*; D013577:Syndrome; D014599:Uterus / Q000002:abnormalities*; D014621:Vagina / Q000002:abnormalities*