[Primary intestinal lymphangiectasia, chronic hepatitis and australia antigen].
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Congenital anomalies of the lung have been recognized relatively recently, and as a group are less well understood than the birth defects in most other systems. From case reports and reviews, it appears that the vast majority of pulmonary malformations are not familial, although many occur in association with other anomalies.
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A case of intestinal lymphangiectasia, limited to a small segment of jejunum, is reported. This finding reflects part of the spectrum of generalized dysplasia of the lymphatic system. A discussion of the pathogenesis, pathophysiology and roentgen features of intestinal lymphangiectasia as part of a systemic disorder is presented.
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The mechanism responsible for persistent regional pulmonary interstitial emphysema under tension, which occasionally complicates assisted ventilation, has not been explained. It is difficult to comprehend how interstitial air may cause lobar hyperinflation and not escape to the mediastinum or pleura. Histologic examination of resected emphysematous lung in two neonates with this type of interstitial emphysema revealed air dissecting from the interstitial connective tissue into markedly dilated lymphatics. It is proposed that intralymphatic air dissection causing emphysematous lymphangiectasia may be responsible for fixation of the lung in the emphysematous state.
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