先天性肺淋巴管扩张是一罕见疾病,首先由Virchow描述.患儿通常是足月的.生后即有呼吸窘迫,大多数死于新生儿期.乳糜胸是本病的少见症状.本组一例,现已超过两岁,出生时就有持续性呼吸困难,因出现乳糜胸而确诊.患儿男性,妊娠34星期,自然分娩,出生体重2040g,生后一小时之内,呼吸率为每分钟80～90次,胸部X-线检查显示弥漫性间质性浸润,无胸膜渗出征.PaO_2 54 torr(未用O_2时),用头罩给O_2几天,呼吸改善,胸部X线检查无改变.10天时始听到持续性心杂音,而诊断为继发于动脉导管未闭的心 Congenital pulmonary lymphangiectasis is a rare condition, first described by Virchow. Children are usually term, with respiratory distress after birth, most of whom die in the neonatal period. Chylothorax is a rare symptom of the disease. One case, now over two years old, had persistent dyspnea at birth and was diagnosed due to the presence of chylothorax.Pregnant children, 34 weeks of gestation, had a spontaneous childbirth with a birth weight of 2040g, within one hour of birth, the rate of respiration was Minutes 80 to 90 times, chest X-ray examination showed diffuse interstitial infiltration, no signs of pleural exudation .PaO_2 54 torr (without O_2), with a hood to O_2 a few days to improve breathing, chest X-ray examination No change was noted, and persistent heart murmur was heard ten days later, with a diagnosis of secondary to patent ductus arteriosus