Lung
Cystic disease / congenital anomalies
Pulmonary lymphangiectasis
Author: Nat Pernick, M.D.
Last author update: 1 August 2011
Last staff update: 20 July 2020
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed search: pulmonary lymphangiectasis
Table of Contents
Classification | Clinical features | Case reports | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosisCite this page: Pernick N. Pulmonary lymphangiectasis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungnontumorlymphangiectasis.html. Accessed April 2nd, 2023.
Classification
- 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
- Premature male with bilateral, pleural effusions (Case of the Week #196)
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
Images hosted on other servers:
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
