Bondonny J M, Boissinot F, Vergnes P, Diard F, Sandler B
Service de Chirurgie Pédiatrique, Hôpital des Enfants, Bordeaux.
Chir Pediatr. 1988;29(5):273-80.
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.
我们最近治疗了一名女婴和两名年轻女孩，她们同时存在三种异常情况：——发育不全的异位肾；——单一的阴道异位输尿管；——同侧生殖器异常。临床表现为终生尿床、“多囊性”肾、尿路感染或腹痛。诊断性检查包括超声检查、排泄性尿路造影、膀胱造影以及经阴道镜检查逆行插管，很少进行放射性核素扫描。本研究中有两例失败，发育不全和发育异常的肾脏未显影，诊断通过剖腹手术完成。生殖器异常方面，一例为单侧阴道积水合并双子宫，另外两例为扩张或囊性的加特纳管。这种异常可能是由于妊娠5至6周胎儿早期生活中中肾管和副中肾管发育异常所致。文献回顾显示，儿童期仅有6例类似病例。对于排泄性尿路造影和超声检查显示有“孤立”肾的女孩，若出现终生尿床、盆腔肿块或疼痛，应警惕这种综合征。仔细寻找发育不全的肾脏、生殖器异常以及异位输尿管口是早期准确诊断和治疗的关键。
