新生儿膈膨升22例病例系列报告

Twenty-two neonates with diaphragmatic eventration: A case series report

目的探讨新生儿膈膨升的临床特点、手术治疗和预后。方法回顾性分析2016年6月1日至2020年5月31日复旦大学附属儿科医院收治的新生儿期诊断膈膨升患儿的临床资料,包括患儿性别、胎龄、体重、患侧、先天性/获得性、膈膨升程度、是否合并其他先天疾病、是否手术、手术方式、术前/术后呼吸支持和预后等。比较手术病例和未手术存活病例的临床特点。结果22例膈膨升,男12例,女10例;胎龄38(33,39)周,早产儿8例,足月儿14例;出生体重2730(1940,3110)g。膈膨升均为单侧,以右侧为主(20例)。先天性膈膨升20例;继发性膈膨升2例,均继发于产伤。膈膨升高度为1.8(1.5,2.0)cm。手术9例,手术时最小体重2.3 kg,手术指征:不能维持正常呼吸8例、发作性青紫1例。手术和非手术存活病例比较,膈肌抬高更明显[2.0(1.65,2.4)vs 1.6(1.0,1.8)cm,P=0.015],呼吸症状为首发症状比例更高(100%vs 50%,P=0.029)。先天性膈膨升合并的常见先天性疾病包括先天性肌张力低下、先天性心脏病、气道异常、隐睾等。死亡5例,均合并其他先天异常,包括先天性肌病4例,复杂先天性心脏病1例。结论难以撤离呼吸支持是新生儿期诊断的膈膨升手术的主要指征。合并严重先天疾病,如先天性肌病等,是影响先天性膈膨升预后的主要因素。

Objective:To explore the clinical features,surgical treatment and prognosis of neonates with diaphragmatic eventration.Methods:Twenty-two neonates with diaphragmatic eventration admitted from June 1st,2016 to May 31st,2020 were recruited in the study.Clinical data was analyzed including gender,gestational age,weight,sides,being congenital or acquired,degree of diaphragmatic eventration,combined congenital diseases,treatment,preoperative/postoperative respiratory support,prognosis,et al.Clinical characteristics of surgical cases and survived cases without surgery were compared as well.Results:The median gestational age and birth weight of 22 neonates,including 12 males and 8 preterm infants,were 38(33,39)weeks and 2,730(1,940,3,110)g respectively.All cases were unilateral,with 20 cases on the right side.Twenty cases were congenital while 2 cases were secondary to birth injury.The median elevation of diaphragmatic eventration was 1.75(1.50,2.00)cm.Nine cases had operation.The lowest weight of operated cases was 2.3 kg at the time of operation.The main indications for surgery were failure to maintain normal breathing(8 cases),and repeated cyanosis(1 case).Compared with non-surgical survival,the diaphragm elevation[2.0(1.65,2.4)vs 1.60(1.0,1.80)cm,P=0.015],and the proportion of respiratory symptoms as the chief complaint for admission(100%vs 50%,P=0.029)were higher in surgical cases.Co-existing congenital diseases in recruited cases were common,including congenital hypotonia,congenital heart disease,airway anomalies,cryptorchidism,et al.All 5 deaths were combined with other congenital abnormalities,including 4 cases of congenital hypotonia and 1 case of complicated congenital heart disease.Conclusion:The main indication for operation in neonatal diaphragmatic eventration cases was failure to maintain normal breathing.The poor prognosis was mainly determined by combined critical congenital anomalies,such as congenital neuromuscular disease.