Health Information

 

 

Congenital Pulmonary Lymphangiectasia

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Congenital Pulmonary Lymphangiectasia is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • CPL
  • PPL
  • primary pulmonary lymphangiectasia
  • pulmonary cystic lymphangiectasis

Disorder Subdivisions

  • None

General Discussion

Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder that is present at birth (congenital). Affected infants have abnormally widened (dilated) lymphatic vessels within the lungs. The lymphatic system helps the immune system in protecting the body against infection and disease. It consists of a network of tubular channels (lymph vessels) that drain a thin watery fluid known as lymph from different areas of the body into the bloodstream. Lymph accumulates in the tiny spaces between tissue cells and contains proteins, fats, and certain white blood cells known as lymphocytes.



Infants with CPL often develop severe, potentially life-threatening, respiratory distress shortly after birth. Affected infants may also develop cyanosis, a condition marked by abnormal bluish discoloration of the skin that occurs because of low levels of circulating oxygen in the blood. The exact cause of CPL is unknown.



CPL can occur as a primary or secondary disorder. Primary pulmonary lymphangiectasia can occur as isolated congenital defect within the lungs or as part of a generalized form of lymphatic vessel malformation (lymphangiectasia) that affects the entire body, usually associated with generalized lymphedema. Secondary CPL occurs secondary to a variety of heart (cardiac) abnormalities, and/or lymphatic obstructive forms.

Resources

National Lymphedema Network

116 New Montgomery Street

Suite 235

San Francisco, CA 94105

Tel: (415)908-3681

Fax: (415)908-3813

Tel: (800)541-3259

Email: nln@lymphnet.org

Internet: http://www.lymphnet.org



March of Dimes Birth Defects Foundation

1275 Mamaroneck Avenue

White Plains, NY 10605

Tel: (914)997-4488

Fax: (914)997-4763

Tel: (888)663-4637

Email: Askus@marchofdimes.com

Internet: http://www.marchofdimes.com



NIH/National Heart, Lung and Blood Institute

P.O. Box 30105

Bethesda, MD 20892-0105

Tel: (301)592-8573

Fax: (301)251-1223

Email: nhlbiinfo@rover.nhlbi.nih.gov

Internet: http://www.nhlbi.nih.gov/



Lymphatic Research Foundation

40 Garvies Point Road

Glen Cove, NY 11542

USA

Tel: (516)625-9675

Fax: (516)625-9410

Email: lrf@lymphaticresearch.org

Internet: http://www.lymphaticresearch.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



British Paediatric Orphan Lung Disease

Email: admin@bpold.co.uk

Internet: http://www.bpold.co.uk



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to MyD-H, the Dartmouth-Hitchcock patient portal. You must be a registered MyD-H user for the Lebanon, Manchester, or Nashua locations to access this site.

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  4/4/2012

Copyright  2008, 2012 National Organization for Rare Disorders, Inc.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use. How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.