Fetal Congenital Cystic Adenomatoid Malformation And Bronchopulmonary Sequestration: A Clinical Restrospective Study And A Systematic Review

OBJECTIVE: To review the intrauterine diagnosis of chromosomal karyotypes and perinatal outcomes after prenatal maternal steroid therapy on congenital cystic adenomatoid malformation(CCAM)and bronchopulmonary sequestration(BPS)through ultrasonography and to put forward the actual clinical recommendations for aristogenesis and genetic counseling.METHOD: A retrospective research was performed on 17 singleton pregnancies for fetal CCAM and BPS in our hospital's prenatal and genetic disease diagnosis center from Jan.2006 to Dec.2016.Then 7 high-risk fetal cases were collected from the continued pregnancy group and treated with prenatal steroids.A follow-up and analysis were done on the chromosomal karyotype,therapy situation and outcome of pregnancy and newborns.All articles that telling the association between CCAM and BPS and steroid therapy published on PubMed,Embase,CKNI and WanFang Data were searched based on the established strategy.The basics of identified studies were extracted independently and analyzed to assess the efficacy of steroid on CCAM and BPS.RESULT: In 17 cases of patients with CCAM and BPS,2 cases were checked for chromosome abnormalities that one was found having pericentric inversion of chromosome 9(continue the pregnancy).Among the 15 cases with normal chromosomal karyotypes,1 case was lost of follow-up and 2 cases chose to terminate pregnancy since they aren't willing to take the risks involved.For the 13 cases continuing the pregnancy,6 cases without hydropic or other malformation delivered at term.Among the 7 high-risk fetal cases accepted maternal dexamethasone therapy,5 cases delivered at term and the survival rate was 71.43%.Moreover,in the choice of delivery mode,4 babies were born vaginally and one was arrived by Caesarean-section.In the part of systematic review,8 studies in totally with 97 cases were adopted.The overall survival rate was 53% ~ 100% and a reduction in lesion size demonstrated in 62% ~ 100% of patients after maternal betamethasone therapy.After single or multiple course of steroids,hydrops relieved in 54%~100% of the patients and 46~80% survived to delivery and neonatal discharge.In the time and ways of delivery,there were 9%~27%(18.4%)infants were born prematurely and the rate of caesarean delivery was 18% ~ 60%(28.9%).In the subgroup analysis,no significant difference was seen between single and multiple course,including the overall survival and resolution of hydrops.CONCLUSIONS: We suggest the patients for fetal CCAM and BPS accept the karyotype analysis since the congenital dissection textural anomaly.In our study,we found that the prognosis for high-risk fetuses has been greatly improved owing to prenatal maternal steroid therapy,and the rate of caesarean delivery was no significant difference in the recent years' rate.However,further research is needed for the association between preterm and CCAM and BPS.Our results also suggested that there is no difference in the effect between singleand multiple course,so the research for the fetuses who has no response to single steroid therapy needs to be studied further.