Guangzhou, China

Author Affiliations: Department of General Pediatrics, The Third Affiliated Hospital of Sun Yat-sen University (Xiao ZY, Tang XY, Chen GJ, Guo L); Department of Pathology, The First Affiliated Hospital of Sun Yat-sen University (Tao Y), Guangzhou, China

Corresponding Author: Zuo-Yuan Xiao, Department of General Pediatrics, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, China (Tel: 86-20-85253098; Email: xiaozy2@126.com)

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.

Conclusion: With regard to treatment and prognosis, CPL must be distinguished from interstitial emphysema and other diseases.

Key words: congenital disease; neonate; pulmonary lymphangiectasis

                   World J Pediatr 2009;5(1):68-70