Ierullo A M, Ganapathy R, Crowley S, Craxford L, Bhide A, Thilaganathan B
Fetal Medicine Unit, Department of Obstetrics and Gynaecology, St George's Hospital, Blackshaw Road, London, UK.
Ultrasound Obstet Gynecol. 2005 Aug;26(2):150-3. doi: 10.1002/uog.1920.
To investigate the natural history and outcome of antenatally diagnosed congenital cystic adenomatoid malformation (CCAM) of the lung.
This was a retrospective study of all cases of fetal CCAM of the lung diagnosed antenatally. All cases were referred to a tertiary center for further management. A computer search identified all referred cases, and the records of these patients were examined to determine the pregnancy outcome.
In a 4-year period, 34 cases of fetal CCAM were referred for further management. At presentation, all the cases were noted to be unilateral CCAMs and the majority (79%) were microcystic in nature. The CCAMs were complicated by varying degrees of mediastinal shift (79%) and hydrops fetalis (18%). During the course of the pregnancy, the lung lesion was seen to reduce in size or resolve spontaneously in 76% of cases without any prenatal intervention (including resolution of hydrops in three cases). The overall survival rate into infancy was 88%. One pregnancy was terminated for persisting hydrops fetalis and another resulted in infant death from complications of neonatal cardiac surgery for an associated aortic coarctation.
The outcome of antenatally detected CCAM is much better than previously reported even when complicated by hydrops fetalis at presentation. The latter seems to be related to the high spontaneous regression rate of this tumor. Despite the antenatal resolution of CCAMs on ultrasound, postnatal follow-up is recommended in view of the long-term complications of this malformation.
探讨产前诊断的先天性肺囊性腺瘤样畸形（CCAM）的自然病史及结局。
这是一项对所有产前诊断的胎儿肺CCAM病例的回顾性研究。所有病例均被转诊至三级中心进行进一步治疗。通过计算机检索确定所有转诊病例，并检查这些患者的记录以确定妊娠结局。
在4年期间，34例胎儿肺CCAM被转诊进行进一步治疗。就诊时，所有病例均为单侧CCAM，且大多数（79%）为微囊型。CCAM伴有不同程度的纵隔移位（79%）和胎儿水肿（18%）。在妊娠过程中，76%的病例在未进行任何产前干预的情况下（包括3例水肿消退），肺部病变体积缩小或自行消退。婴儿期总体生存率为88%。1例因持续性胎儿水肿而终止妊娠，另1例因相关主动脉缩窄的新生儿心脏手术并发症导致婴儿死亡。
即使产前诊断的CCAM就诊时伴有胎儿水肿，其结局也比先前报道的要好得多。后者似乎与该肿瘤的高自发消退率有关。尽管超声检查显示CCAM在产前已消退，但鉴于该畸形的长期并发症，仍建议进行产后随访。
