Document Detail

Autopsy case of congenital pulmonary lymphangiectasis.
MedLine Citation:
PMID:  15189509     Owner:  NLM     Status:  MEDLINE    
Congenital pulmonary lymphangiectasis (CPL) is a rare anomaly. We report a female infant born at 39 weeks of gestation who was found to have CPL. Cyanosis and tachypnea were noted immediately after birth, and, at room air, PaO2 was 30.7 mmHg, PaCO2 was 82.5 mmHg and pH was 7.12. The infant's symptoms did not improve even with the initiation of artificial ventilation. Chest X-ray film showed cotton-like infiltrates in both lungs and an air-leak surrounding the cardiac shadow. Echocardiography study showed no abnormality. The neonate died 3 days after birth due to hypoxemic cardiac failure. At autopsy, the pleural surface contained numerous dilated vessels that had the appearance of lymphatics. Microscopic features of the lungs were marked lymphatic dilatation of the perivascular, subpleural and interlobular areas. Lymphangiectasis was found in the liver, kidney, pancreas, thyroid and alimentary canals, such as the esophagus, stomach and rectum. Patients with lymphatic dilatations in extrapulmonary organs have mild pulmonary involvement and symptoms and a better prognosis. However, a few cases of CPL with lymphatic dilatations in extrapulmonary organs and an aggressive course, such as the present case, have been reported. The clinical behavior and prognosis of CPL depend on the extent of pulmonary involvement of the lymphatic dilatations regardless of systemic lymphatic dilations.
Hiroshi Hirano; Takashi Nishigami; Akira Okimura; Keiji Nakasho; Kunio Uematsu
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pathology international     Volume:  54     ISSN:  1320-5463     ISO Abbreviation:  Pathol. Int.     Publication Date:  2004 Jul 
Date Detail:
Created Date:  2004-06-10     Completed Date:  2004-12-23     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9431380     Medline TA:  Pathol Int     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  532-6     Citation Subset:  IM    
Second Department of Pathology, Hyogo College of Medicine, Hyogo, Japan.
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MeSH Terms
Fatal Outcome
Infant, Newborn
Lung / pathology*
Lung Diseases / congenital,  pathology*
Lymphangiectasis / congenital,  pathology*
Lymphatic System / pathology*
Radiography, Thoracic

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