조기수술이 필요한 폐 선천성 낭포성 샘 모양 기형아의 특성과 수술 전 중재적 시술
Neonatal Characteristic of Congenital Cystic Adenomatoid Malformation of the Lung Requiring Early Operation and Preoperative Intervention
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목적: 폐 선천성 낭포성 샘 모양 기형(congenital cystic adenomatoid malformation, CCAM)에서 출생 후 증상을 보여 조기수술을 하게 되는 환자와 연관된 인자를 알아보고, 수술 전 호흡 기능의 안정화를 위한 중재적 시술 방법에 대해 소개하고자 하였다. 방법: 서울 아산병원 소아청소년병원 신생아중환자실 혹은 신생아실로 2001년 1월부터 2011년 12월까지 지난 11년간 본원에서 산전초음파로 CCAM 진단받고 입원 후 폐 절제 수술을 받은 환아들을 대상으로 후향적 연구를 하였다. 출생 직후 증상을 보인 조기수술군과 증상이 없었던 계획수술 군으로 나누어 환아와 산모의 특성, 조기수술과 연관된 인자를 조사하여 통계학적 방법으로 비교하였다. 아울러 산전 중재적 시술과 출생 후 수술 전 중재적 시술을 받았던 환아들의 임상 경과도 살펴보았다. 결과: 총 60명의 환자들이 등록되었고 수술 시기에 따라 조기수술 군과 계획수술 군으로 나누었다. 수술 시기는 조기수술군(n=12, 20%) 이 중간값 5.5일, 계획수술군(n=48, 80%)이 504일이었다. 나이, 조산, 양수과소증을 포함한 산모의 특성은 두 군간 비슷하였다. 양수과다증이 있었거나(OR 23, P=0.001), 산전 중재적 시술을 받았거나(OR 47,P=0.001),, 산전 CCAM의 크기 증가를 보이면서 출생 시 낮은 아프가 점수를 보였던 환아가 조기수술과 연관된 인자들로 조사되었다. 출생 후 호흡기증상이 발생하고 생체징후가 불안전하여 응급수술을 시행하지 못한 3명의 환아가 수술 전 중재적 시술을 받았고 이후 성공적으로 폐 절제수술 시행이 가능하였다. 결론: 대부분의 CCAM을 지닌 신생아는 증상이 없어 계획적 수술이 가능하였지만 신생아 시기에 조기수술이 약 20%에서필요하였다. 조기수술과 관련된 인자로 양수과다증, 산전 중재적 시술, 산전 CCAM의 크기 증가, 낮은 아프가 점수를 보인 신생아로 조사되었고 이들에게 적극적인 산전 중재적 시술, 수술 전 중재적 시술과 궁극적으로 폐 절제 조기수술을 시행함으로써 단 한 건의 사망 없이 생존율을 향상시킬 수 있었다.
Abstract
Purpose: To identify neonatal characteristics associated with congenital cystic adenomatoid malformation (CCAM) who required early operations and to introduce preoperative interventions to delay definitive operations until stabilized. Methods: A retrospective review of dataset was performed from January 2000 to December 2011 for neonates admitted to NICU at Asan Medical Center with prenatally diagnosed CCAM. Variable prenatal and postnatal factors were compared for those who required early operations to those asymptomatic neonates who required elective operations at later age. Results: A total of 60 patients were enrolled and patients were divided into 2 groups according to time of operation. Median time of surgery for group 1 (n=12, 20%) and group 2 (n=48, 80%) was 5.5 days and 504 days, respectively. Maternal characteristics including age, parity, preterm labor, oligohydramnios were similar in between two groups. Factors associated with early operation included prenatal history of polyhydramnios (OR 23, P=0.001), who had undergone fetal interventions (OR 47, P=0.001), low 1 and 5 minute Apgar scores and increasing fetal fluid-filled cystic sizes (OR 26, P=0.013). Of those 3 neonates who required preoperative interventions to decrease air-filled cysts to relieve initial respiratory symptoms were successful and all underwent for definitive operations during NICU hospitalizations. All survived. Conclusion: Most neonates with CCAM undergo elective operations during infancy. However, about 20% neonates with polyhydramnios, fetal intervention, increasing fetal cystic mass and born with low Apgar scores required early operations for whom prenatal and postnatal interventions prior to definitive surgery can improve survival rate.
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