Wilson R Douglas, Pawel Bruce, Bebbington Michael, Johnson Mark P, Lim Foong-Yen, Stamilio David, Silber Angela, Zakii Elaine, Flake Alan W
The Center for Fetal Diagnosis and Treatment, Department of Surgery at The Children's Hospital of Philadelphia/University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4399, USA.
Prenat Diagn. 2006 Nov;26(11):1058-61. doi: 10.1002/pd.1555.
Case report and literature review for congenital pulmonary lymphangiectasis (CPL) CASE REPORT: Male fetus with bilateral pleural effusion, thoracoamniotic shunt, preterm delivery, and prolonged neonatal course with neonatal death at 3 months. Autopsy-identified CPL.
Review of pathology, clinical course, and genetics of CPL.
This postnatal diagnosis of CPL/Hennekam syndrome must be considered with prenatal counseling regarding a fetus with bilateral pleural effusions. This pathological entity is autosomal recessive and has a significant risk of lethality.
先天性肺淋巴管扩张症（CPL）的病例报告及文献综述
男性胎儿，伴有双侧胸腔积液、胸羊膜分流术、早产，新生儿期病程延长，3个月时死亡。尸检确诊为CPL。
对CPL的病理学、临床病程及遗传学进行综述。
对于有双侧胸腔积液的胎儿进行产前咨询时，必须考虑CPL/亨内坎综合征的产后诊断。这种病理实体为常染色体隐性遗传，具有较高的致死风险。
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