Congenital pulmonary lymphangiectasis.
BACKGROUND
Congenital pulmonary lymphangiectasis (CPL) characterized by dilatation of pulmonary lymphatic vessels occurs as a congenital anomaly. With poor prognosis, neonatal presentation of bilateral CPL is associated with the severe compromise of pulmonary gas exchange and high mortality.


METHODS
A male infant born at 39 weeks of gestation was found to have CPL. Cyanosis and cardiac arrest occurred a few minutes after birth, and the symptoms remained after artificial ventilation. The infant died of hypoxemic cardiac failure 45 minutes after birth. Autopsy showed neither pleural effusion nor valvular abnormalities.


RESULTS
Microscopically dilated vessels with lymphatics were seen in the lung of the infant. Atelectasis, CPL, inhalation of amniotic fluid, partial hydropic degeneration of hepatic cells, and scrotal edema were diagnosed.


CONCLUSIONS
With regard to treatment and prognosis, CPL must be distinguished from interstitial emphysema and other diseases.
Xiao
Zuo-Yuan
ZY
Department of General Pediatrics, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China. xiaozy2@126.com
Tao
Yu
Y
Tang
Xin-Yi
XY
Chen
Guo-Juan
GJ
Guo
Lei
L
D001344:Autopsy; D017809:Fatal Outcome; D006801:Humans; D007231:Infant, Newborn; D008171:Lung Diseases / Q000151:congenital* / Q000473:pathology / Q000628:therapy; D008200:Lymphangiectasis / Q000151:congenital* / Q000473:pathology / Q000628:therapy; D008297:Male; D012121:Respiration, Artificial