title: Congenital generalized lymphangiectasia: a rare developmental disorder for non-immune fetal hydrops.
abstract: Firstly described by Rudolf Virchow in the 19th century, congenital generalized lymphangiectasia is a rare entity characterized by dilation of lymphatic vessels, and was recently classified in primary or secondary lymphangiectasia. Generalized forms may be diagnosed during pre-natal follow-up with ultrasound examination, and, depending on its severity, the newborn outcome is very poor. The authors report the case of a female newborn with a previous diagnosis of fetal hydropsy who was born after a full-term gestation with respiratory failure due to bilateral voluminous pleural effusion and ascites. Physical examination also disclosed syndromic facies. Despite all efforts of the intensive supportive care, the patient died after 24 days of life. The autopsy findings were consistent with the diagnosis of congenital pulmonary lymphangiectasia. The authors call attention to this rare diagnosis in patients with cavitary effusion and respiratory insufficiency at birth.
authors.affiliation: Anatomy Pathology Service - Hospital Universitário - University of São Paulo, São Paulo/SP - Brazil .
authors.forename: Cristiane Rúbia
authors.initials: CR
authors.lastname: Ferreira
authors.affiliation: Department of Pathology - Hospital das Clínicas - University of São Paulo, São Paulo/SP - Brazil .
authors.forename: Verônica
authors.initials: V
authors.lastname: Sibre
authors.affiliation: Department of Pathology - Faculty of Medicine - University of São Paulo, São Paulo/SP - Brazil.; Anatomy Pathology Service - Instituto do Cancer de São Paulo, São Paulo/SP - Brazil.
authors.forename: Regina
authors.initials: R
authors.lastname: Schultz
authors.affiliation: Pediatrics Division - Hospital Universitário - University of São Paulo, São Paulo/SP - Brazil .
authors.forename: Ana Maria Andrello Gonçalves Pereira
authors.initials: AM
authors.lastname: de Melo
authors.affiliation: Pediatrics Division - Hospital Universitário - University of São Paulo, São Paulo/SP - Brazil .
authors.forename: Silvia Maria
authors.initials: SM
authors.lastname: Ibidi
authors.affiliation: Pediatrics Department - Faculty of Medicine - University of São Paulo, São Paulo/SP - Brazil .
authors.forename: Jackeline Della
authors.initials: JD
authors.lastname: Torre
keywords: Autopsy; Lymphangiectasia, pulmonary, congenital; Respiratory Insufficiency