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Congenital generalized lymphangiectasia: a rare developmental disorder for non-immune fetal hydrops.
Autops Case Rep 2015 Oct-Dec; 5(4):27-33AC

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+Show Affiliations

Anatomy Pathology Service - Hospital Universitário - University of São Paulo, São Paulo/SP - Brazil .Department of Pathology - Hospital das Clínicas - University of São Paulo, São Paulo/SP - Brazil .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.Pediatrics Division - Hospital Universitário - University of São Paulo, São Paulo/SP - Brazil .Pediatrics Division - Hospital Universitário - University of São Paulo, São Paulo/SP - Brazil .Pediatrics Department - Faculty of Medicine - University of São Paulo, São Paulo/SP - Brazil .

Pub Type(s)

Case Reports

Language

eng

PubMed ID

26894043

Citation

Ferreira, Cristiane Rúbia, et al. "Congenital Generalized Lymphangiectasia: a Rare Developmental Disorder for Non-immune Fetal Hydrops." Autopsy & Case Reports, vol. 5, no. 4, 2015, pp. 27-33.
Ferreira CR, Sibre V, Schultz R, et al. Congenital generalized lymphangiectasia: a rare developmental disorder for non-immune fetal hydrops. Autops Case Rep. 2015;5(4):27-33.
Ferreira, C. R., Sibre, V., Schultz, R., de Melo, A. M., Ibidi, S. M., & Torre, J. D. (2015). Congenital generalized lymphangiectasia: a rare developmental disorder for non-immune fetal hydrops. Autopsy & Case Reports, 5(4), pp. 27-33. doi:10.4322/acr.2015.027.
Ferreira CR, et al. Congenital Generalized Lymphangiectasia: a Rare Developmental Disorder for Non-immune Fetal Hydrops. Autops Case Rep. 2015;5(4):27-33. PubMed PMID: 26894043.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Congenital generalized lymphangiectasia: a rare developmental disorder for non-immune fetal hydrops. AU - Ferreira,Cristiane Rúbia, AU - Sibre,Verônica, AU - Schultz,Regina, AU - de Melo,Ana Maria Andrello Gonçalves Pereira, AU - Ibidi,Silvia Maria, AU - Torre,Jackeline Della, Y1 - 2015/12/30/ PY - 2015/10/01/received PY - 2015/11/17/accepted PY - 2016/2/20/entrez PY - 2016/2/20/pubmed PY - 2016/2/20/medline KW - Autopsy KW - Lymphangiectasia, pulmonary, congenital KW - Respiratory Insufficiency SP - 27 EP - 33 JF - Autopsy & case reports JO - Autops Case Rep VL - 5 IS - 4 N2 - 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. SN - 2236-1960 UR - https://www.unboundmedicine.com/medline/citation/26894043/Congenital_generalized_lymphangiectasia:_a_rare_developmental_disorder_for_non-immune_fetal_hydrops L2 - http://dx.doi.org/10.4322/acr.2015.027 DB - PRIME DP - Unbound Medicine ER -