What is the definition of Congenital Pulmonary Lymphangiectasia?

Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder that affects the lungs. It is present from birth and usually becomes apparent in the first few days of life with respiratory failure. It sometimes is apparent before birth with non-immune hydrops fetalis and pleural effusion (fluid in the lung). Infants with CPL often develop severe, potentially life-threatening, respiratory distress shortly after birth. They may also develop cyanosis caused by low oxygen levels in the blood, which causes the skin to have a bluish tint. Symptoms are due to abnormally wide (dilated) lymphatic vessels within the lungs. These vessels drain a fluid called lymph from different areas of the body. They are an important part of the lymphatic system, which helps the immune system protect the body against infection and disease.

The underlying cause of CPL is unknown. It can occur as a primary or secondary disorder (due to another underlying condition). Primary CPL occurs as an isolated defect or as part of a generalized form of lymphatic disease affecting the whole body. Secondary CPL can occur due to a variety of heart abnormalities or lymphatic obstruction. Some cases of CPL have been associated with genetic disorders.

Treatment aims to relieve the symptoms of the disorder and may include CPAP, intubation, and/or fluid drainage. While much of the older literature suggests a very high mortality rate, recent studies suggest that CPL does not have as poor an outlook.

What are the alternative names for Congenital Pulmonary Lymphangiectasia?

  • CPL
  • Lymphangiomatosis pulmonary
  • Pulmonary cystic lymphangiectasis
  • Lymphangiectasia pulmonary congenital

What is the outlook (prognosis) for Congenital Pulmonary Lymphangiectasia?

Conflicting data have been reported regarding the outcome for children with congenital pulmonary lymphangiectasia (CPL). The prognosis has previously been reported to be very poor, with mortality at about 100% before the 1990s. However, more recent reports suggest that CPL is not a uniformly fatal condition, and that in survivors, the condition improves. This might be due to advances in modern intensive care therapy and also to the fact that severity can vary among affected children. It has been suggested that children with generalized lymphangiectasia who have pulmonary involvement may have less severe CPL and a better prognosis; having a primary developmental defect of the pulmonary lymphatic vessels tends to be associated with a higher mortality. Those with CPL who do survive infancy often continue to have medical problems that are characteristic of chronic lung disease. Gastroesophageal reflux and poor growth are also not uncommon during the first year of life, especially between six and twelve months of age, and are closely related to chronic lung disease.

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