[The association of a dysplastic kidney with a single vaginal ectopic ureter and a homolateral genital abnormality in a girl. Comments based on 3 case reports].
We recently treated one female infant and two young girls with coincidence of three anomalies: --hypoplastic ectopic kidney; --single vaginal ectopic ureter; --ipsilateral genital abnormality. Clinical presentation was lifelong wetting, "multicystic" kidney, urinary tract infection, or abdominal pain. The diagnostic studies include ultrasonography, excretory urography, cystography and vaginoscopic examination with retrograde catheterization, rarely radionuclide scanning. This study failed in two cases, with non visualization of the hypoplastic and dysplastic kidney, and diagnosis was done at laparotomy. Genital anomalies were unilateral hydrocolpos with uterus didelphys in one case, dilated or cystic Gärtner's duct in the other two. The anomaly may be caused by a maldevelopment of the wolffian and müllerian ducts in early fetal life, between 5 and 6 weeks of gestation. A review of the literature revealed only 6 similar instances in childhood. The combination of lifelong wetting, pelvic mass or pain in girl with "solitary" kidney on excretory urography and ultrasonography should alert to this syndrome. Meticulous search for the hypoplastic kidney, the genital abnormality and the ectopic ureteral orifice are keys to early accurate diagnosis and treatment.
Bondonny
J M
JM
Service de Chirurgie Pédiatrique, Hôpital des Enfants, Bordeaux.
Boissinot
F
F
Vergnes
P
P
Diard
F
F
Sandler
B
B
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007668:Kidney / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014513:Ureter / Q000002:abnormalities*; D014567:Urography; D014599:Uterus / Q000002:abnormalities*; D014621:Vagina / Q000002:abnormalities
Association, chez la fille, d'un rein dysplasique avec uretère simple à abouchement ectopique vaginal et d'une anomalie génitale homolatérale. Commentaires, à partir de trois observations.