儿童迟发性先天性膈疝患儿的临床诊治特点分析

Clinical diagnosis and treatment of children with late-onset congenital diaphragmatic hernia

目的探讨儿童迟发性先天性膈疝(CDH)临床表现及诊治特点。方法选择2013年1月至2023年12月于本院小儿普通外科、胸外科诊治的74例迟发性CDH患儿为研究对象,纳入研究组,其平均年龄为3.2岁(1个月至14岁);Bochdalek疝、Morgagni疝及食管裂孔疝(EHH)患儿各为27、2及45例。选择同期在同一家医院治疗的67例平均年龄为1.3 d(0~28 d)CDH患儿作为对照,纳入对照组,Bochdalek疝、Morgagni疝及EHH患儿各为50、6及11例。采用回顾性分析法,采集研究组患儿临床资料,包括临床表现、CDH类型、诊治特点等,并采用独立样本t检验、χ^(2)检验或连续性校正χ^(2)检验进行统计学分析。由于本研究对EHH与Bochdalek疝及Morgagni疝患儿采取的检查及治疗方式不同,因此将研究组与对照组按照纳入对象为EHH患儿与Bochdalek疝及Morgagni疝患儿,进一步分为EHH亚组1(n=45)及BM亚组1(n=29),EHH亚组2(n=11)及BM亚组2(n=56)。本研究遵循的程序经本院伦理委员会批准(审批文号:2018PS391K),所有患儿监护人对本研究知情同意,并签署临床研究知情同意书。结果①研究组迟发性CDH患儿CDH类型:27例(36.5%)为Bochdalek疝[位于右外侧为2例(2.7%),左外侧为25例(33.8%)];2例(2.7%)为Morgagni疝;45例(60.8%)为EHH。临床症状:18例(24.3%)以呼吸系统症状为主要表现,46例(62.2%)以消化道症状为主要表现[7例(9.5%)被明确诊断为伴有生长发育迟缓或贫血],10例(13.5%)无CDH相关临床症状,为体检时被诊断。治疗方式:采取微创手术治疗者为31例(41.9%),采取微创手术+中转开放手术治疗者为3例(4.1%),采取开放手术治疗者为40例(54.0%)。诊断方式:对74例患儿均采取胸部或腹部数字X射线摄片、胸部或腹部CT检查被确诊。61例(82.4%)患儿同时采取上消化道X射线造影,2例(2.7%)完善X射线钡灌肠检查,1例(1.4%)仅进行X射线钡灌肠检查。6例(8.1%)进行胸部增强CT检查辅助判断疝内容物活性。②EHH亚组1及EHH亚组2患儿性别构成比、手术时长及术后进食时间等比较,差异均无统计学意义(P>0.05),而EHH亚组1患儿膈肌缺损直径大于EHH亚组2,并且差异有统计学意义(t=-2.71、P=0.009)。BM亚组1与BM亚组2患儿性别构成比、膈肌缺损直径、有无疝囊构成比比较,差异均无统计学意义(P>0.05);但是BM亚组1补片使用率,较BM亚组2低,手术时长、术后呼吸机辅助通气时间及术后进食时间,均短于BM亚组2,并且差异均有统计学意义(χ^(2)=4.08、P=0.043,t=3.36、P=0.001,t=2.85、P=0.006,t=5.51、P<0.001)。结论迟发性CDH患儿由于缺乏典型临床表现,常导致该病被临床延迟诊断。临床对呼吸窘迫和胃肠道紊乱患儿进行鉴别诊断时,应充分考虑患儿迟发性CDH可能。影像学检查在迟发性CDH患儿的鉴别诊断中是必要的。

Objective To investigate clinical manifestations,clinical diagnosis and treatment of children with late-onset congenital diaphragmatic hernia(CDH).Methods A total of 74 children with late-onset CDH who were treated in the Department of Pediatric General Surgery and Thoracic Surgery,Shengjing Hospital Affiliated to China Medical University from January 2013 to December 2023 were selected as study subjects,and were included in study group,their mean age was 3.2 years old(1 month to 14 years old);27,2,and 45 children with Bochdalek hernia,Morgagni hernia,and esophageal hiatal hernia(EHH),respectively.And another 67 neonates with CDH treated in the same hospital during the same period were selected as control and included in control group,their mean age was 1.3 d(0-28 d);50,6,and 11 children with Bochdalek hernia,Morgagni hernia,and EHH,respectively.Clinical data of children between study group and control group,including clinical manifestations,CDH types,diagnosis and treatment characteristics were retrospectively collected and were compared by independent-samples t test,or chi-square test or correction for continuity chi-square test.As examination and treatment methods of EHH,Bochdalek hernia and Morgagni hernia were different,study group and control group were further divided into EHH subgroup 1(n=45)and BM subgroup 1(n=29),EHH subgroup 2(n=11)and BM subgroup 2(n=56)based on EHH children,Bochdalek hernia and Morgagni hernia children,respectively.The procedures followed in this study were approved by the Ethics Committee of our hospital(Approval No.2018PS391K),and all guardians of children signed informed consent for clinical study.Results①CDH type of children with late-onset CDH:bochdalek hernia in 27 cases(36.5%)[right lateral hernia in 2 cases(2.7%),left lateral hernia in 25 cases(33.8%)];2 cases(2.7%)were Morgagni hernias,and 45 cases(60.8%)were EHH.Clinical manifestations:18 cases(24.3%)had respiratory symptoms as the main manifestation,46 cases(62.2%)had digestive tract symptoms as the main manifestation[7 cases(9.5%)were diagnosed with growth retardation or anemia],and 10 cases(13.5%)had no clinical symptoms related to CDH and were diagnosed during physical examination.Treatment methods:31 cases(41.9%)underwent minimally invasive surgery,3 cases(4.1%)underwent minimally invasive surgery+transfer to open surgery,and 40 cases(54.0%)underwent open surgery.Diagnosis methods:all 74 cases were diagnosed by digital X-ray of chest or abdomen and CT of chest or abdomen.And 61 cases(82.4%)also received upper digestive tract X-ray angiography,2 cases(2.7%)underwent complete X-ray barium enema examination,and 1 case(1.4%)underwent only X-ray b arium enema examination.6 cases(8.1%)underwent chest enhanced CT to assist in judging the activity of hernia contents.②There were no statistically significant differences in gender ratio,operation duration and postoperative feeding time between EHH subgroup 1 and EHH subgroup 2(P>0.05).Diameter of diaphragm defect in EHH subgroup 1 was larger than that in EHH subgroup 2,and the difference was statistically significant(t=-2.71,P=0.009).There were no statistically significant differences in gender ratio,diaphragm defect diameter and hernia sac between BM subgroup 1 and BM subgroup 2(P>0.05).However,rate of patch usage in BM subgroup 1 was lower than that in BM subgroup 2,and the operation duration,postoperative ventilator assisted ventilation duration and postoperative feeding time in BM subgroup 2 were shorter than those in BM subgroup 2,and all the differences were statistically significant(χ^(2)=4.08,P=0.043;t=3.36,P=0.001;t=2.85,P=0.006;t=5.51,P<0.001).Conclusions The lack of typical clinical manifestations in children with late-onset CDH often leads to delayed clinical diagnosis of the disease.The possibility of late-onset CDH should be fully considered in the differential diagnosis of children with respiratory d istress and gastrointestinal disorders.Imageological examinations are necessary in the differential diagnosis of every child with late-onset CDH.