Cystic disease/congenital anomalies
Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 25 August 2011, last major update August 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.
● 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
● 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
● Premature male with bilateral, pleural effusions (Case of Week #196)
● Heavy and noncompliant, with numerous dilated lymphatics or cystic spaces in visceral pleura
● Widened interlobular septa, dilated / cystic lymphatic vessels
12 day old premature male infant with bilateral milky-white pleural effusions at autopsy
Above patient - D2-40 staining
● 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
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