| Birth defect is a public health problem which is concerned by the government and society.Improving the ability of early diagnosis,intervention and treatment of birth defects can provide medical technical support for the implementation of the current three-children policy,which is of great positive significance.Congenital diaphragmatic hernia(CDH)is a serious birth defect,which is a hot topic of research on birth defect diseases in the world.It is mainly caused by dysplasia of fetal diaphragm,which makes the abdominal viscera enter the chest cavity and compress the lung.Genetic factors account for 10%of CDH morbidity,and more than 80%have not found clear etiology.In the past,due to insufficient understanding of CDH and limited treatment methods such as neonatal surgery,the mortality of CDH was over 30%,the mortality of severe CDH was up to 70%,and the induced labor rate was as high as 61.54%.In recent years,with the promotion of prenatal diagnosis and genetic screening technology and the progress of intensive care technology and surgical methods,the prognosis of children with CDH has been greatly improved.In clinical practice,the long-term prognosis of children with successful congenital diaphragm repair can be similar to that of healthy children of the same age after postoperative respiratory exercise and long-term follow-up.The main pathophysiological changes of CDH were fetal lung dysplasia.During fetal development,the abdominal viscera herniated into the thoracic cavity and compressed the lungs,affecting the growth of bronchial branches,reducing the number of small arteries and thickening the blood vessel wall,resulting in lung hypoplasia and abnormal development of pulmonary blood vessels.The final manifestations are hypoxemia,hypercapnia and persistent pulmonary hypertension of the newborn(PPHN).The systemic circulation blood flows through the unclosed arterial duct and foramen ovale to form right-left shunt,which causes the newborn to remain in the fetal circulation state,and eventually causes hypoxemia,respiratory circulation failure,and death of the child.Therefore,early and accurate diagnostic evaluation,timely intervention and treatment of children with CDH will bring more chances of survival for this population.Ex utero intrapartum treatment(EXIT)is a technique used to provide cardiopulmonary support to newborns during delivery by maintaining placental circulation.EXIT procedure has the advantage that the placental circulation support let physicians have enough time to perform some action to save lives,such as bronchoscopy,laryngoscopy,endotracheal intubation and tracheotomy,even ECMO treatment,giving children a safe airway protection during childbirth,and reduce the risks associated with hypoxia,acidosis,and even of ischemic encephalopathy.International studies have supported the safe and feasible application of EXIT in the treatment of children with CDH.The study by Foglia’s team at the Children’s Hospital of Philadelphia showed that the children in EXIT group had better short-term indicators such as the hemoglobin content measured at the first time and the mean arterial pressure one hour after birth than the control group.But there have been no studies on whether EXIT-to-Airway can improve the survival rate and recurrence rate of children with CDH.The effect of EXIT-to-Airway treatment on the prognosis of neonatal diaphragmatic hernia was first evaluated in our center(Part 1).From January 1,2011 to December 31,2020,a retrospective review was performed for 95 CDH neonates.Among them,22 cases with severe disease and postnatal dyspnea received ex utero intrapartum treatment(EXIT),21 were operated and 19 survived with a postoperative survival rate of 90.5%.Clinical characteristics,surgical parameters and outcomes were compared.There were 73 cases in non-EXIT group,60 cases were operated and 43 cases survived with a postoperative survival rate of 71.7%.Logistic regression analysis showed that EXIT during labor(OR=0.104,[95%CI:0.022-0.493]),gestational age at prenatal diagnosis(OR=0.863,[95%C/:0.783-0.952])and liver herniation into chest(OR=9.657,[95%CI:3.094-30.137])were independent prognostic factors for CDH children.According to the receiver operating characteristic(ROC)curve,the cutoff value of maximizing the diagnostic efficiency of prenatal diagnostic age was 24.5 weeks.The final conclusion is that both safe and feasible,EXIT may improve the prognosis of neonatal CDH effectively.EXIT,gestational age of prenatal diagnosis and liver herniation into thoracic cavity are independent prognostic factors for CDH neonates.Since selection bias might exist due to the significant difference in disease severity,here,we applied propensity score matching(PSM)to minimise the effect of other confounding factors for prognosis,and simulate the research conditions of an RCT.To further explore the influence of EXIT on the prognosis of CDH neonates.Clinical data of 110 children with CDH treated in Children’s Hospital of Capital Institute of Pediatrics from 2012 to 2021 were retrospectively analyzed(Part 2).Children were divided into EXIT group and non-EXIT group according to whether EXIT was performed at birth.The outcomes of the two groups were compared,applying propensity score matching.Logistic regression analysis was carried out to explore the efficacy of EXIT on survival.During the study period 30 of 116 children received EXIT.After PSM,the survival rates of the EXIT group and the non-EXIT group were 82.76%(24/29)and 48.28%(14/29),respectively(P=0.006).EXIT(OR=0.083,95%CI=0.013-0.525,P=0.008),liver herniation(OR=16.955,95%CI=2.342-122.767,P=0.005),and gestational age at diagnosis(OR=0.662,95%CI=0.497-0.881,P=0.005)were independent mortality related risk factors of all children with CDH.Ninety-nine of 116 children underwent surgery.After PSM,the postoperative survival rates of the EXIT group and non-EXIT group were 84.6%(22/26)and 76.9%(20/26),respectively(P=0.754).Liver herniation(OR=10.451,95%CI=1.641-66.544,P=0.013]and gestational age at diagnosis(OR=0.136,95%CI=0.577-0.938,P=0.013)were independent mortality related risk factors of children after surgery.The final conclusion is that,for selected neonates with CDH,EXIT is safe and feasible with potentially better survival than traditional delivery mode,and does not cause more complications than traditional cesarean section.Over the past three years,the COVID-19 pandemic has ravaged the world and is the most important issue for the world right now.Concerns about the potential risk of infection and the strict implementation of containment measures can lead to delays in routine diagnosis and treatment.How pediatric surgeons should respond to the impact of the pandemic on health care remains a huge challenge.To investigate the impact of COVID-19 on the treatment of children with congenital diaphragmatic hernia(CDH),we retrospectively collected and compared the data of patients with CDH admitted between January 1,2020 and December 31,2021(study group)with the CDH patients admitted before the pandemic between January 1,2018 and December 31,2019(control group)(Part 3).During the pandemic,41 patients with CDH diagnosed prenatally were transferred to our hospital,and 40 underwent surgical repair.The number of patients treated in our hospital increased by 24.2%compared with the 33 patients before the pandemic.During the pandemic,the overall survival rate,postoperative survival rate and recurrence rate were 85.4%,87.5%and 7.3%,respectively,and there were no significant differences compared with the control group(75.8%,83.3%and 9.1%,respectively).The average length of hospital stay in patients admitted during the pandemic was longer than that in the control group(31 days vs.16 days,P<0.001),and the incidence of nosocomial infection was higher than that in the control group(19.5%vs.3%,P=0.037).We came to the conclusion that CDH patients confirmed to be SARS-CoV-2 infection-free can receive routine treatment.Our data indicate that the implementation of protective measures during the COVID-19 pandemic,along with appropriate screening and case evaluation,do not have a negative impact on the prognosis of children.We hope to offer some advice to pediatric surgeons around the world who are on the front lines of fighting the epidemic. |
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