目的:报告3例肺静脉共同腔闭锁均合并肺淋巴管扩张症,二者均系少见畸形,后者易被误诊。方法:3例中男性2例.女性1例,均为足月出生婴儿,分别于生后2小时、6小时和16小时死亡,尸俭后观察。结果:前2例为完全性肺静脉共同腔闭锁,例3为不完全性。例1为孤立性肺静脉共同腔闭锁,后2例均合并心房迷走位伴无脾症。3例均有肺膨大和肺淋巴管囊性变,而且以例3右上肺叶尖部为最显著。结论:肺淋巴管扩张的发生机制除与肺静脉高压有关外,与肺淋巴管发生缺陷也有联系。 PURPOSE: To report 3 cases of pulmonary venous common cavity atresia with pulmonary lymphangiectasis, both of which are rare deformities, the latter being easily misdiagnosed. Methods: There were 2 males in 3 cases and 1 female females, all of whom were full-term infants who died at 2 hours, 6 hours and 16 hours after birth, respectively. Results: The first two cases were complete pulmonary vein common cavity atresia. Case 3 was incomplete. Example 1 solitary pulmonary vein common cavity atresia, 2 cases were combined with atrial vagus position without spleen disease. 3 cases had lung enlargement and pulmonary lymphatic cystic degeneration, and to the top right lung lobes in Example 3 is the most significant. CONCLUSION: The mechanism of pulmonary lymphangiectasis is related to pulmonary venous hypertension and pulmonary lymphangiogenesis defects.