Potter综合征一例报告及文献复习  

A case report and literature review about Potter′s syndrome.

作　　者：吴琦嫦 王宏 王淑真 吴慧南 曾炳勋 钟晓红 田瑞华 杨华 

机构地区：[1]福建省厦门市妇幼保健院妇产科,361000

出　　处：《中国优生与遗传杂志》2006年第5期100-101,共2页Chinese Journal of Birth Health & Heredity

摘　　要：目的探讨Potter综合征的诊断方法及病因。方法对一例孕21周发现羊水过少的孕妇采用羊膜腔灌注法补充羊水进行B超产前诊断,引产前抽脐血查胎儿染色体,引产后进行胎儿尸体病理检查。结果经三次羊膜腔灌注后,孕妇的羊水量达到正常,B超检查未发现胎儿的双肾及膀胱,孕妇于孕26周引产,引产前抽脐血查胎儿的染色体。结果是46,XX,引产后发现胎儿的外观具有特殊的Potter面容,病理检查发现胎儿的双肾、输尿管、膀胱及尿道均缺如,盆腔内无子宫及双附件。结论结合文献,Potter综合征在产前可用B超诊断,羊膜腔灌注法补充羊水有利于产前的B超诊断,Potter综合征的病因不明,进行胎儿染色体和尸体病理的检查有利于进一步查找病因。Objective： To explore the diagnostic method and etiology about Potter＇s syndrome. Methods： A pregnant woman with severe oligohydramnios was found at 21 weeks. Amnioinfusion was performed for ultrasound examination. Cordocentesis was performed before termination of pregnancy, and postmortem examination was done. Results ： The amniotic volume was normal after three times of amnioinfusion. Ultrasound examination showed absence of bilateral kidneys and bladder. The pregnancy was terminated at 26 weeks. Chromosomal analysis found the fetal karyotype is 46, XX. Postmortem examination showed a female fetus with features of Potter＇s syndrome, bilateral renal agenesis was confirmed. The fetal bladder and urethra and bilateral renicapsule and ureter were found absent also. At the same time, the genital anomalies were found, the fetal uterus and bilateral tubo - ovarian were absent. Conclusion ： Combine with literature, Potter＇s syndrome can be prenatal diagnosis by ultrasound, anmioinfusion is beneficial for prenatal ultrasound diagnosis. The etiology about Potter＇s syndrome is unknown, chromosomal analysis and postmortem examination are benefit to explore the etiology.

关 键 词：羊水过少 胎儿 双肾缺如 Potter’s综合征 

分 类 号：R714.5[医药卫生—妇产科学]