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Detailed information of CONGENITAL PULMONARY LYMPHANGECTASIA
CONGENITAL PULMONARY LYMPHANGECTASIA
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 Syndrome
- Turner's Syndrome
TYPES (after Noonon (1970))*:
- dilated pulmonary lymphatics are part of a generalized form
of lymphangiectasis: thoracic and extrathoracic (intestinal)
lymphangiectasia
- associated with
- lymphedema
- hemihypertrophy
- diffuse angiomatosis in which bone is the most common site
of involvement
- less severe form and has a much better prognosis than the
other two forms
2. Group II
- due to a cardiac lesion
- dilated 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
3. Group III
- due to a defect in the primary development of the lung
- Lawrence (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 enlargened 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 overexpanded lungs with
alveolar hypoventilation and periodic wheezing and a relapsing
course
- dilated lymphatics may result in marked restriction of
respiratory movements
- poor prognosis
CLINICAL FEATURES:
- presents with respiratory distress and cyanosis at birth
- (usually term) with rapid demise
- can also present as a stillbirth
2. Post Neonatal Period
- presents with respiratory difficulties
- progressive relapsing course
- associated with chylopericardium, chylothorax, chronic
cough, congestive heart failure
3. Associated Abnormalities
- congenital abnormalities in 50% of cases:
- TAPVR, mitral valve atresia, hypoplastic left heart,
aortic coarctation, VSD, PDA, ASD
2. Others
- cystic hygromas, diffuse angiomatosis (bones),
pneumothorax
INVESTIGATIONS:
- cystic 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
2. Imaging Studies
- hyperexpansion
- diffuse reticular pattern
MANAGEMENT:
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Pediatric Database - CONGENITAL PULMONARY LYMPHANGECTASIA
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