Adzick N S, Harrison M R, Glick P L, Golbus M S, Anderson R L, Mahony B S, Callen P W, Hirsch J H, Luthy D A, Filly R A
J Pediatr Surg. 1985 Oct;20(5):483-8. doi: 10.1016/s0022-3468(85)80470-x.
We studied the natural history and pathophysiology of congenital cystic adenomatoid malformation (CCAM) detected prenatally by ultrasound in twelve fetuses. Two types of fetal CCAM can be distinguished by gross anatomy, ultrasound findings, and prognosis. Microcystic lesions are usually associated with fetal hydrops and have a poor prognosis (five cases with one survivor). Antenatal diagnosis, maternal transport, and immediate thoracotomy after birth allowed the first reported survival of a newborn with a large microcystic CCAM. Macrocystic lesions are not usually associated with hydrops and have a favorable prognosis (five of seven survived). We conclude that fetuses with hydrops are at high risk for fetal or neonatal demise without intervention. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.
我们研究了12例经超声产前诊断的先天性囊性腺瘤样畸形（CCAM）胎儿的自然病史和病理生理学。根据大体解剖、超声表现和预后可区分两种类型的胎儿CCAM。微囊性病变通常与胎儿水肿相关，预后较差（5例，1例存活）。产前诊断、母亲转运以及出生后立即开胸手术使得首例报道的患有大型微囊性CCAM的新生儿存活。大囊性病变通常与水肿无关，预后良好（7例中有5例存活）。我们得出结论，未经干预，患有水肿的胎儿发生胎儿或新生儿死亡的风险很高。患有CCAM但无水肿的胎儿，通过母亲转运、计划分娩以及立即进行新生儿复苏和手术，有良好的存活机会。
