Müllerian dysgenesis.
The multifaceted syndrome of müllerian dysgenesis appears to represent a defect in mesodermal organization and is often associated with renal and skeletal anomalies. This report discusses the embryology, multifactorial etiology, clinical presentation and management of the syndrome from a historical standpoint and details our experience with 35 affected patients. Both Frank dilatation and McIndoe vaginoplasty appear to be acceptable means of therapy. The therapeutic success depends to a large extent on the psychosocial adaptation of the patient.
D000015:Abnormalities, Multiple / Q000209:etiology / Q000523:psychology / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004106:Dilatation; D005260:Female; D006801:Humans; D009095:Mullerian Ducts; D012729:Sex Chromosome Aberrations / Q000209:etiology / Q000523:psychology / Q000601:surgery; D014599:Uterus / Q000002:abnormalities; D014621:Vagina / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery