Progress in diagnosis and treatment of congenital cystic adenomatoid malformation of the lung in children / 国际儿科学杂志

Congenital cystic adenomatoid malformation of the lung is a rare clinical lung disease.It is the deformity formed by abnormal embryo development of lung tissue.Its pathological morphology is very different from other types of pulmonary cyst.The main pathogenesis,clinical manifestation,diagnosis,differential diagnosis and treatment are described.

Neonatal Characteristic of Congenital Cystic Adenomatoid Malformation of the Lung Requiring Early Operation and Preoperative Intervention

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.

Ultrasonographic diagnosis and clinical prognosis of fetal congenital cystic adenomatoid malformation of the lung / 中国医学影像技术

Objective To explore the diagnostic value of ultrasonography of fetal congenital cystic adenomatoid malformation of the lung (CCAM), and to predict the prognosis according to ultrasonographic findings. Methods The chest of 19 fetus with CCAM was multi-sectionally scanned with two-dimensional ultrasonography. The position, appearance and size of mass were observed, and complications were continuously followed. Results CCAM was pathologically confirmed in 13 fetus after induced abortion. One neonatal died, while CCAM in other 5 fetus disappeared before 36 weeks. The mass of typeⅠCCAM became smaller and smaller, and eventually disappeared. The echo-free spaces in typeⅡbecame smaller and fewer, and the strong echo weakened to the same level as normal lung. For type Ⅲ, the echo of solid mass weakened to the same level as normal lung, or transformed to typeⅡ gradually, and finally recovered to normal echo of lung as the gestational age increased. During follow up, there were 10 fetus (10/19, 52.63%) that lung adenoma cyst increased with the gestational age increased, and the heart, mediastinal shifted, pleural effusion, and (or) fetal edema were observed. Conclusion Ultrasound examination is a reliable method for the diagnosis of CCAM, and enable to predict the prognosis of the affected fetuses. If heart and mediustinum displacement, pleural effusion, hydrops fetal or other abnormalities exist, it's necessary to terminate the pregnancy.