Congenital generalized lymphangiectasia: a rare developmental disorder for non-immune fetal hydrops

Authors

  • Cristiane Rúbia Ferreira University of São Paulo. Hospital Universitário. Anatomy Pathology Service
  • Verônica Sibre University of São Paulo. Hospital das Clínicas. Department of Pathology
  • Regina Schultz University of São Paulo. Faculty of Medicine. Department of Pathology - Instituto do Cancer de São Paulo. Anatomy Pathology Service
  • Ana Maria Andrello Gonçalves Pereira de Melo Melo University of São Paulo. Hospital Universitário. Pediatrics Division
  • Silvia Maria Ibidi University of São Paulo. Hospital Universitário. Pediatrics Division
  • Jackeline Della Torre University of São Paulo. Faculty of Medicine. Pediatrics Department

DOI:

https://doi.org/10.4322/acr.2015.027

Keywords:

Lymphangiectasia, pulmonary, congenital, Respiratory Insufficiency, Autopsy

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

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Published

2015-12-10

How to Cite

Ferreira, C. R., Sibre, V., Schultz, R., Melo, A. M. A. G. P. de M., Ibidi, S. M., & Della Torre, J. (2015). Congenital generalized lymphangiectasia: a rare developmental disorder for non-immune fetal hydrops. Autopsy and Case Reports, 5(4), 27-33. https://doi.org/10.4322/acr.2015.027

Issue

Section

Article / Autopsy Case Report