Congenital developmental defects of derivates of müllerian ducts

Congenital developmental defects of Müllerian derivates, understandable with the knowledge of embryological development of Wolffian and Müllerian ducts, are defects of canalisation (= gynatresias), defects in fusing, combined defects and uterovaginal agenesis. Gynatresias should be suspected in the newborn, but distinguished in puberty, on the basis of menstrual blood retention, as hymeneal atresia (haematocolpos), aplasia partis distalis vaginae (haematocolpos partialis), transversal vagina septum and aplasia of vagina and uterine cervix (isolated haematometra). Particular operations are described. Defects in the fusing of Müllerian ducts from the point of view of surgery could be single-coated or double-coated. Incomplete reduplication with unilateral renal aplasia syndrome could present as hemihaematocolpos, hemihaematometra and haematometra in rudimental horn. For diagnosis of these disorders menstrual blood retention is necessary. Surgical treatment in the first two types includes resection of the common wall and haematometra in rudimental horn needs metroplasty or hemihysterectomy. Congenital absence of uterus and vagina (Rokitanski Küster) appears in genetically, endocrinologically and psychosexually normal females. Diagnosis is based on clinical examination and ultrasonography. Initial examination should be nonsurgical (dilatation method). All surgical corrections create a place for the future vagina and reach its epithelization. Different procedures are criticised. The author recommends and describes Vecchietti's laparoscopic surgery.