Abstract
We report the case of 2-week-old female infant with cystic lung disease who presented
with mild tachypnea and had no history of mechanical ventilation. Chest CT demonstrated
multiple air-filled cystic lesions in right upper lobe, and the patient subsequently
underwent a right upper lobectomy. Histology revealed cystic lesions located in the
pulmonary parenchyma and showed that the lesions were lined by lymphatic endothelium
and were communicating with dilated lymphatic vessels in the interstitium. Additionally,
multinucleated foreign body giant cells were attached to the lumen of the cyst. On
the basis of these findings, we considered this a case of persistent interstitial
pulmonary emphysema (PIPE) with massive pneumatic expansion of the lymphatic vessels,
resulting in cystic lesions with lymphatic endothelium in the pulmonary parenchyma.
While PIPE is extremely rare in term non-ventilated infants, our case demonstrated
that this disease should be added to the differential diagnosis of cystic lung diseases
with lymphatic endothelium even in infants without mechanical ventilation. When cystic
lesions and symptoms persist despite conservative treatment, open or thoracoscopic
resection is an appropriate option for diagnosis and treatment.
Key words
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Article info
Publication history
Accepted:
September 11,
2012
Received in revised form:
August 6,
2012
Received:
May 19,
2012
Identification
Copyright
© 2012 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
