Cystic disease / congenital anomalies

Pulmonary lymphangiectasis

Last author update: 1 August 2011
Last staff update: 20 July 2020

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PubMed search: pulmonary lymphangiectasis

Nat Pernick, M.D.
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Cite this page: Pernick N. Pulmonary lymphangiectasis. website. Accessed September 30th, 2023.
  • Classified by Noonan in 1970 into three groups (Am J Dis Child 1970;120:314):
    • Group 1: generalized lymphangiectasis, including lymphedema with intestinal lymphangiectasis
    • Group 2: secondary disorder due to pulmonary venous hypertension or obstruction associated with cardiovascular anomalies
    • Group 3: patients compromised by a primary developmental defect of the pulmonary lymphatics
  • In 2000, Faul proposed classifying these disorders as primary or secondary lymphangiectasis (Am J Respir Crit Care Med 2000;161:1037):
    • Primary cases: present in neonates with severe respitatory distress with pleural effusion, with generalized edema in some cases; usually require mechanical ventilation; historically, they were almost always fatal; thought to be caused by failure of pulmonary interstitial connective tissues to regress, leading to the dilation of pulmonary lymphatic capillaries
    • Secondary cases: due to various disorders that impair lymphatic drainage and increase lymph production, including surgery, radiation, infection, tumor, trauma
Clinical features
  • Rare disease characterized by abnormal dilatation of pulmonary lymphatics, but without lymphatic proliferation (J Korean Med Sci 2007;22:740)
  • Often fatal in early life, but aggressive neonatal therapies may improve outcome (Eur Respir J 2004;24:413), although in this study, 6 of their 9 cases had an apparently normal neonatal period, suggesting that they were actually secondary disease under the Faul classification
Case reports
Gross description
  • Heavy and noncompliant, with numerous dilated lymphatics or cystic spaces in visceral pleura
Microscopic (histologic) description
  • Widened interlobular septa, dilated / cystic lymphatic vessels
Microscopic (histologic) images

Case #196

12 day old premature male infant with bilateral milky white pleural effusions at autopsy

Same patient - D2-40 staining

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Various images

Differential diagnosis
  • Pulmonary interstitial emphysema: cystic spaces are connected with alveoli; keratin staining shows pneumocytes lining the intact alveolar wall
  • Congenital cystic adenomatoid malformation: entire lung is abnormally arranged and immature; cysts are lined by prominent epithelium with goblet cells
  • Lymphangiomatosis: increased number of complex, anastomosing lymphatic channels; dilation is secondary, not primary
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