家族性先天性幽门闭锁是一种胎儿胃肠道罕见的畸形,通常在超声扫描下可见母亲羊水过多和胎儿胃扩张。现报告一例,患者29岁,孕3产2,妊娠第33周,常规超声扫描观察到羊水过多而进入高危妊娠病房。家庭为犹太一伊拉克血统,双亲为表兄妹关系。首次妊娠因早产而于第34周终止,男性新生儿,生后第一天被诊断为幽门闭锁,并开腹行胃十二指肠吻合术。第二次妊娠未发现异常,42周后自然分娩,娩出一健康女婴。患者又妊娠33周住院时检查发现宫高38cm,子宫很大、软,其余正常,血型为“A”Rh~+,口 Familial congenital pyloric atresia is a rare form of fetal gastrointestinal abnormalities, usually seen in the ultrasound scan of the mother’s polyhydramnios and fetal stomach expansion. Now report a case, the patient 29 years old, 3 pregnant 2, 33 weeks of pregnancy, conventional ultrasound scan observed excessive amniotic fluid into the high-risk pregnancy ward. The family is Jewish and Iraqi, with parents as cousins. The first pregnancy was terminated at week 34 due to preterm birth. Male neonates, who were diagnosed as pyloric atresia on the first day of life, underwent gastro-duodenal anastomosis. The second pregnancy was not abnormal, natural delivery after 42 weeks, delivered a healthy baby girl. Patients and 33 weeks of gestation examination found that when the hospital high 38cm, the uterus large, soft, the rest of the normal blood type “A” Rh ~ + mouth