Outcomes of Cases of Prenatally-Diagnosed Congenital Pulmonary Airway Malformation

Abstract Objective To evaluate the outcomes of cases of prenatally-diagnosed congenital pulmonary airway malformation (CPAM). Methods We retrospectively evaluated cases of prenatally-diagnosed CPAM between 2004 and 2018. Ultrasonographic features such as visualization of a fetal lung mass and heterogeneous pulmonary parenchyma were used for CPAM diagnosis. Prenatal and postnatal findings were compared in terms of accuracy regarding the CPAM diagnosis. Results The sample consisted of 27 cases. There were four cases in which the patients opted for the termination of pregnancy due to the severity of the lesion. A total of 23 neonates were delivered, and CPAM was confirmed in 15 cases. Themedian gestational age at delivery was 37 weeks (28-40 weeks) and the mean birth weight was 2,776 g. There were two neonatal deaths, one due to pneumothorax, and the other due to hypoplastic left heart syndrome (HLHS). A total of five patients with respiratory problems were operated in the postpartum period. There were eight misdiagnosis: bronchopulmonary sequestration (five cases), congenital lobar emphysema (two cases), and congenital diaphragm hernia (one case). Conclusion A precise postnatal diagnosis is very important to organize the proper management of the pregnancies with fetuses with CPAM. The positive predictive value of the prenatal diagnosis of CPAM via ultrasonography is of 70.3%. The differential diagnosis of CPAM may be prolonged to the postpartum period in some cases.

摘要 研究目的：评估产前诊断先天性肺气道畸形（congenital pulmonary airway malformation, CPAM）病例的临床结局。方法：回顾性分析2004年至2018年间收治的产前诊断CPAM病例。以胎儿肺肿块可视化、肺实质不均质等超声影像学特征作为CPAM的诊断依据，对比产前与产后检查结果的CPAM诊断准确性。结果：本研究共纳入27例病例。其中4例因病变严重程度选择终止妊娠。共分娩23例新生儿，其中15例经确诊为CPAM。分娩时中位孕周为37周（范围28~40周），平均出生体重为2776g。共发生2例新生儿死亡，1例因气胸，另1例因左心发育不良综合征（hypoplastic left heart syndrome, HLHS）。共5例存在呼吸系统问题的患儿于产后接受手术治疗。共出现8例误诊：支气管肺隔离症5例、先天性肺叶气肿2例、先天性膈疝1例。结论：精准的产后诊断对于制定CPAM胎儿妊娠的合理管理方案至关重要。超声产前诊断CPAM的阳性预测值为70.3%。部分病例的CPAM鉴别诊断需延长至产后阶段方可明确。