Diamond Ivan R, Wales Paul W, Smith Saxon D, Fecteau Annie
Division of Pediatric General Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.
J Pediatr Surg. 2003 Sep;38(9):E1-3. doi: 10.1016/s0022-3468(03)00413-5.
A congenital cystic adenomatoid malformation (CCAM) is a congenital lung lesion that, when associated with hydrops, is thought to have a dismal prognosis without fetal intervention. The authors report a case of a fetus with a CCAM associated with ascites as the only manifestation of hydrops, which was diagnosed at 19 weeks gestation. The CCAM regressed without intervention between 23 and 29 weeks of gestation with resolution of the ascites. The baby was born asymptomatic at term, and the infant underwent elective resection of the CCAM at 1 year of age. A review of the literature from 1980 to 2000 yielded 9 other cases of spontaneous resolution of hydrops associated with CCAM, suggesting that although rare, this outcome is a possibility in fetuses diagnosed with the condition. Because most fetuses diagnosed with hydrops undergo termination of pregnancy, the true incidence of hydrops resolution is potentially higher than predicted. This has important implications for prenatal counseling particularly in centers in which fetal intervention is not available.
先天性囊性腺瘤样畸形（CCAM）是一种先天性肺部病变，当与胎儿水肿相关时，若不进行胎儿干预，预后通常较差。作者报告了一例胎儿，其CCAM与腹水相关，是胎儿水肿的唯一表现，于妊娠19周时确诊。CCAM在妊娠23至29周期间未经干预而消退，腹水也随之消失。婴儿足月出生时无症状，1岁时接受了CCAM的择期切除手术。回顾1980年至2000年的文献，发现还有9例与CCAM相关的胎儿水肿自发消退的病例，这表明尽管这种情况罕见，但对于诊断为此病的胎儿来说仍有可能出现这种结果。由于大多数被诊断为胎儿水肿的胎儿会终止妊娠，所以胎儿水肿消退的实际发生率可能高于预期。这对产前咨询具有重要意义，尤其是在无法进行胎儿干预的医疗中心。
