CAJ | 학술논문

目的：探讨先天性肺淋巴管扩张的临床及预后。方法回顾性分析1例表现为胎儿双侧胸腔积液的先天性肺淋巴管扩张症患儿的临床资料。结果患儿于胎儿期就表现为双侧胸腔积液，生后呼吸困难，双侧大量气胸、乳糜胸，低蛋白血症，持续性肺动脉高压。给予肺泡表面活性物质、NO吸入、高频机械通气、反复白蛋白输注，治疗3个月后出院。出院后第10天又以“重症肺炎、慢性肺病”收治入院，经机械通气、抗感染治疗2个月余，仍不能离氧，存活半年死亡。结论先天性肺淋巴管扩张症罕见，产前诊断困难。对于生后呼吸困难严重、有明显间质性肺气肿表现的患儿，应适时给予肺组织活检或核素淋巴管显影扫描以明确诊断。
목적：탐토선천성폐림파관확장적림상급예후。방법회고성분석1례표현위태인쌍측흉강적액적선천성폐림파관확장증환인적림상자료。결과환인우태인기취표현위쌍측흉강적액，생후호흡곤난，쌍측대량기흉、유미흉，저단백혈증，지속성폐동맥고압。급여폐포표면활성물질、NO흡입、고빈궤계통기、반복백단백수주，치료3개월후출원。출원후제10천우이“중증폐염、만성폐병”수치입원，경궤계통기、항감염치료2개월여，잉불능리양，존활반년사망。결론선천성폐림파관확장증한견，산전진단곤난。대우생후호흡곤난엄중、유명현간질성폐기종표현적환인，응괄시급여폐조직활검혹핵소림파관현영소묘이명학진단。
ObjectiveTo summarize and review the clinical characteristics of congenital pulmonary lymphangiectasia with fetal bilateral pleural effusions.MethodsThe clinical data of a newborn diagnosed as congenital pulmonary lymphangiec-tasia with bilateral pleural effusions in Obstetrics and Gynecology Hospital, was summarized. The clinical, radiographic features, treatment and prognosis of this case are discussed in the context of the literature review.ResultsThe premature infants present-ed with rapid progression bilateral pleural effusions, respiratory distress, chylothorax, hypoalbuminemia and persistent pulmo-nary hypertension at birth. The pulmonary surfactant was given and mechanical ventilation was used for respiratory support. NO was inhaled, high-frequency mechanical ventilation was applied and albumin was repeatedly administered. After treatment for 3 months in the NICU, the patient was discharged. After 10 days, the patient was administered to the PICU with "severe pneumo-nia, chronic lung disease, mechanical ventilation" and anti-infection treatments were applied for 2 months. After living for six months, the baby died.ConclusionsCongenital pulmonary lymphangiectasia is extremely rare and prenatal diagnosis was dif-ifcult. The disease should be considered in patients presented with progressive dyspnea, interstitial emphysema after birth. Lung tissue biopsy and radioisotope scanning should be performed in time to get diagnosis.