Duplication of the müllerian ducts and genitourinary malformations. Part II: analysis of malformations.

Abstract

Forty-seven females with duplication of the uterus and cervix were retrospectively studied to obtain a better understanding of the reasons for failure of fusion of the müllerian ducts by analyzing the associated genitourinary malformations. Thirty-one of the 47 patients had major genitourinary malformations that corresponded to three main complexes of anomalies. Cloacal anomalies were present in 16 patients, exstrophy in two, and combined cloacal and exstrophic deformities in another two. Renal malformations ipsilateral to a unilateral occluded müllerian duct were seen in 11 patients, and in four of these an ectopic ureter opened into the occluded duct. From the analysis of these anomalies it is proposed that duplication of the female genital system, when not an isolated event, may be secondary to interference of the normal process of müllerian fusion by a persistent cloacal duct, an abnormal pelvic girdle, or an ectopic ureter.

Uterus didelphys with an obstructed unilateral vagina by a transverse vaginal septum associated with ipsilateral renal agenesis, duplication of inferior vena cava, high-riding aortic bifurcation, and intestinal malrotation: a case report

“…Other accompanying renal anomalies such as renal dysplasia, double collecting system, and ectopic ureter are also possible, although only a limited number of such cases have been reported (8).…”

Surgical correction of rare Müllerian anomalies and spontaneous pregnancies: a case report

“…The congenital defects in the pelvis and the perineal area, which are derived from defective development of the cloacal membrane, the urorectal septum, the mesonephric and paramesonephric (M€ ullerian) ducts, and the urogenital sinus, have been described elsewhere (3,4). However, an extensive review of the literature shows that a reconstructive procedure followed by spontaneous pregnancy in such cases has not been reported.…”

Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases

“…If the Wolffian origin of sinovaginal bulbs were reliable, then the unperforated hemivagina could have been explained by Wolffian ducts maldevelopment [8,19,20]; however, it does not seem to be the only definite cause of this anomaly [17]. And other types of urinary tract anomaly such as ectopic opening of the ureter with renal hypoplasia had also accompanied MDAs [21,22].…”