****LYMPHATIECTASIA****

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Fri Oct 25 09:24:39 EST 1996


M. McDermott wrote:
> 
> If you have any information regarding this malady, it is desperately
> needed.  A friends baby is suffering from this and they are desperate
> for information re: treatments, research, names of doctors involved in
> research etc..... If you have access to this information please help
> me..Can you post and/or email any info to Scubadyver at aol.com  Thank you

Before I send you off on a wild goose chase, I should mention a
pulmonary form of this illness in newborns.  Here is a brief
outline:
Pulmonary Lymphangiectasia

Clinical Presentation:

Respiratory distress in a newborn. 

Etiology/Pathophysiology:

Complete pulmonary venous atresia leads to pulmonary venous obstruction
which results in engorged pulmonary lymphatics
and capillaries. Heart size is normal since flow through the left side
of the heart is decreased. Usually is fatal. Classified into 3
types: (1) Pulmonary lymphangiectasia associated with congenital heart
lesions characterized by obstruction of pulmonary
venous return [totally anomalous venous return below the diaphragm,
pulmonary venous atresia, mitral atresia, hypoplastic left
heart] (2) Pulmonary lymphangiectasia associated with systemic
lymphangiectasia (3) Isolated pulmonary lymphangiectasia.
Type (1) is most common, Type (3) is rarest. 

Pathology:

Absence of the pulmonary venous structures, prominence of the pulmonary
lymphatics and capillaries. 

Imaging Findings:

Prominent bilateral, symmetrical interstitial distention of lymphatics
with a general branching pattern radiating from the hila that
extends to the periphery with prominence of Kerley B lines.  Diffuse
pulmonary hyperinflation is also seen. Less
commonly a ground glass pattern can be seen. 

DDX:

     Transient Tachypnea of the Newborn  

References:

See References Chapter. 


sandro at lhc.nlm.nih.gov

librarian at vh.radiology.uiowa.edu 

Electric Differential Multimedia Lab



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