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Title: Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion. Author: Wilson RD, Pawel B, Bebbington M, Johnson MP, Lim FY, Stamilio D, Silber A, Zakii E, Flake AW. Journal: Prenat Diagn; 2006 Nov; 26(11):1058-61. PubMed ID: 16941717. Abstract: OBJECTIVE: 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. DISCUSSION: Review of pathology, clinical course, and genetics of CPL. CONCLUSION: 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.[Abstract] [Full Text] [Related] [New Search]