Pulmonary Lymphangiectasia
CONGENITAL PULMONARY LYMPHANGIECTASIA
DEFINITION:
A congenital disease involving the lung characterized by greatly dilated lymphatic ducts throughout the lung.
EPIDEMIOLOGY:
incidence: ?
age of onset:neonatal period and after the neonatal period
risk factors:Noonan's SyndromeTurner's Syndrome
TYPES (after Noonon (1970))*:
Group I
dilated pulmonary lymphatics are part of a generalized form of lymphangiectasis: thoracic and extrathoracic (intestinal) lymphangiectasiaassociated with lymphedemahemi hypertrophy diffuse angiomatosis in which bone is the most common site of involvementless severe form and has a much better prognosis than the other two forms2.
Group II
due to a cardiac lesiondilated pulmonary lymphatics are acquired in utero due to obstruction of the pulmonary venous flow secondary to a cardiac lesion which may interfere with the normal regression of the lymphatic tissue elements after the 16th week of fetal life
very poor prognosis
Group III
due to a defect in the primary development of the lungLawrence (1955) postulated that lymphangectasis is due to a developmental error in which the normal regression of connective tissue elements in the lung fails to occur after the 16th week of life. Subsequently the lymph vessels do not regress in size and remain enlarged in relation to the parenchyma of the lung (Noonan, 1970).
male predominance
may be associated with congenital malformations including cardiac lesions clinical picture characterized by over expanded lungs with alveolar hypoventilation and periodic wheezing and a relapsing course
dilated lymphatics may result in marked restriction of respiratory movements
poor prognosis
*first report of CPL by Virchow (1856) and first named by Lawarence in 1955
CLINICAL FEATURES:
presents in two forms:
1. Neonatal Period presents with respiratory distress and cyanosis at birth(usually term) with rapid demisecan also present as a stillbirth
2. Post Neonatal Period presents with respiratory difficultiesprogressive relapsing courseassociated with chylopericardium, chylothorax, chronic cough, congestive heart failure
3. Associated Abnormalities congenital abnormalities in 50% of cases:
1. CardiacTAPVR, mitral valve atresia, hypoplastic left heart, aortic coarctation, VSD, PDA, ASD
2. Otherscystic hygromas, diffuse angiomatosis (bones), pneumothorax
INVESTIGATIONS:
A. Diagnostic
1. Lung Biopsycystic dilation of the lymphatics in the subpleural and intralobular regions and perivascular spaces give the lungs a subpleural reticulated pattern of fine interconnecting white lines with air-filled cystic areas
B. Imaging Studies
1. Chest X-Ray
hyperexpansion
diffuse reticular pattern
MANAGEMENT:
supportive
. . . . . . .
See Also
Pulmonary Lymphangiectasia
Lymphedema People
DEFINITION:
A congenital disease involving the lung characterized by greatly dilated lymphatic ducts throughout the lung.
EPIDEMIOLOGY:
incidence: ?
age of onset:neonatal period and after the neonatal period
risk factors:Noonan's SyndromeTurner's Syndrome
TYPES (after Noonon (1970))*:
Group I
dilated pulmonary lymphatics are part of a generalized form of lymphangiectasis: thoracic and extrathoracic (intestinal) lymphangiectasiaassociated with lymphedemahemi hypertrophy diffuse angiomatosis in which bone is the most common site of involvementless severe form and has a much better prognosis than the other two forms2.
Group II
due to a cardiac lesiondilated pulmonary lymphatics are acquired in utero due to obstruction of the pulmonary venous flow secondary to a cardiac lesion which may interfere with the normal regression of the lymphatic tissue elements after the 16th week of fetal life
very poor prognosis
Group III
due to a defect in the primary development of the lungLawrence (1955) postulated that lymphangectasis is due to a developmental error in which the normal regression of connective tissue elements in the lung fails to occur after the 16th week of life. Subsequently the lymph vessels do not regress in size and remain enlarged in relation to the parenchyma of the lung (Noonan, 1970).
male predominance
may be associated with congenital malformations including cardiac lesions clinical picture characterized by over expanded lungs with alveolar hypoventilation and periodic wheezing and a relapsing course
dilated lymphatics may result in marked restriction of respiratory movements
poor prognosis
*first report of CPL by Virchow (1856) and first named by Lawarence in 1955
CLINICAL FEATURES:
presents in two forms:
1. Neonatal Period presents with respiratory distress and cyanosis at birth(usually term) with rapid demisecan also present as a stillbirth
2. Post Neonatal Period presents with respiratory difficultiesprogressive relapsing courseassociated with chylopericardium, chylothorax, chronic cough, congestive heart failure
3. Associated Abnormalities congenital abnormalities in 50% of cases:
1. CardiacTAPVR, mitral valve atresia, hypoplastic left heart, aortic coarctation, VSD, PDA, ASD
2. Otherscystic hygromas, diffuse angiomatosis (bones), pneumothorax
INVESTIGATIONS:
A. Diagnostic
1. Lung Biopsycystic dilation of the lymphatics in the subpleural and intralobular regions and perivascular spaces give the lungs a subpleural reticulated pattern of fine interconnecting white lines with air-filled cystic areas
B. Imaging Studies
1. Chest X-Ray
hyperexpansion
diffuse reticular pattern
MANAGEMENT:
supportive
. . . . . . .
See Also
Pulmonary Lymphangiectasia
Lymphedema People
Intestinal Lymphangiectasia - Waldmann Disease
All About Lymphangiectasia Yahoo Support Group
Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.
Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com
posted by Pat O'Connor @ 2:13 AM
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