Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

Lung-nontumor

Cystic disease/congenital anomalies

Pulmonary lymphangiectasis


Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 25 August 2011, last major update August 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

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

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 (Orphanet Encylopedia December 2004); 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

Case Reports
=========================================================================

● Premature male with bilateral, pleural effusions (Case of Week #196)

Gross description
=========================================================================

● Heavy and noncompliant, with numerous dilated lymphatics or cystic spaces in visceral pleura

Micro description
=========================================================================

● Widened interlobular septa, dilated / cystic lymphatic vessels

Micro images
=========================================================================


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


Above patient - D2-40 staining

   
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 abormally 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

End of Lung-nontumor > Cystic disease/congenital anomalies > Pulmonary lymphangiectasis


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).