赵媛 杨光 刘克战 张新华
[摘要] 目的 探討先天性食管闭锁(Esophageal atresia,EA)的诊断路径,提高早期诊断能力,为手术赢得时间。 方法 收集2016年5月~2018年5月本院新生儿内科收治的28例确诊EA的新生儿临床资料,采用回顾性研究方法,分析患儿诊治资料。 结果 所有患儿产前超声提示羊水多者占50.0%,可疑EA者7.1%。足月儿组和早产儿组两组主要症状相比有显著差异(χ2=15.857,P=0.000)。足月儿组主要症状以吐沫最常见,占47.8%,与早产儿组吐沫发生率相比有显著差异(χ2=3.939,P=0.047);其次为呕吐及喉中痰鸣,各占21.7%;而早产儿组主要症状以早产为主,与足月儿组早产发生率相比较有显著统计学差异(χ2=15.456,P=0.003)。80.0%早产儿为小于胎龄儿,早产儿宫内生长受限几率明显高于足月儿(χ2=0.431,P<0.05),早产儿内科住院时间亦明显延长(t=2.099,P<0.05)。 结论产前超声提示羊水多者多次行超声检查,产后应根据不同胎龄主要症状判定EA,可疑EA者早期置胃管、口服造影剂床旁摄胸腹片,合并多发畸形者做染色体及基因检测,可提高手术成功率。
[关键词] 先天性食管闭锁;新生儿;出生缺陷;消化系统疾病
[中图分类号] R726.1 [文献标识码] A [文章编号] 1673-9701(2019)26-0017-04
[Abstract] Objective To investigate the diagnostic path of congenital esophageal atresia (EA), improve early diagnosis ability and win time for surgery. Methods The clinical data of 28 neonates with confirmed EA who were admitted to the department of neonatology in our hospital from May 2016 to May 2018 were collected. A retrospective study was conducted to analyze the diagnosis and treatment data of the children patients. Results The prenatal ultrasound for all children patients showed that there were 50.0% of excessive amniotic fluid and 7.1% of suspected EA. There was a significant difference in main symptoms between the two groups of full-term infant group and the premature infant group (χ2=15.857, P=0.000). The main symptoms in the full-term infant group were spittle, which accounted for 47.8%. There was a significant difference in the incidence rate of spittle compared with the premature infant group(χ2=3.939, P=0.047); the second main symptoms was vomiting and snoring in the throat, each accounting for 21.7%; the main symptoms in the premature infant group were prematurity, which was significantly different from the incidence rate of prematurity in the full-term infant group(χ2=15.456, P=0.003). 80.0% of the premature infants were small for gestational age, and the probability of intrauterine growth restriction in premature infants was significantly higher than that of full-term infants(χ2=0.431, P<0.05). The length of hospital stay in premature infants was also significantly longer(t=2.099, P<0.05). Conclusion Prenatal ultrasound suggests that excessive amniotic fluid should be examined by ultrasound several times. After birth, EA should be determined according to the main symptoms of different gestational age. Suspected EA patients should be given early gastric tube, oral administration of contrast agent for bedside chest and abdominal imaging, and patients combined with multiple malformations should be given chromosome and genetic testing, which can improve the success rate of surgery.
[Key words] Congenital esophageal atresia (EA); Neonates; Birth defect; Digestive system disease
先天性食管闭锁(Esophageal atresia,EA)是新生儿期最具挑战的先天性解剖畸形之一,以食管发育不连续为主要特征,伴或不伴食管气管瘘(Tracheo-esophageal Fistula,TEF),发病率约2.43/10000[1]。如不经手术治疗,数日内死亡。近年来随着产前诊断、危重新生儿救治及外科手术水平的不断提高,包括早产儿在内的EA患儿成活率有所改善[2]。本文通过回顾性分析我院新生儿内科收治的28例EA患儿生后早期内科诊治的临床资料,旨在探讨早期诊断路径,提高早期诊断能力,为手术治疗赢得时间,现报道如下。
1 资料与方法
1.1 一般资料
选取2016年5月~2018年5月我院新生儿内科收治的确诊EA者28例,按照胎龄是否≤37周分为早产儿组和足月儿组。诊断依据[3]:(1)临床表现:生后有唾液过多、饮奶出现呛咳、发绀,胃管不能插入或折返。(2)X线表现:经胃管注入0.5~1 mL非离子型造影剂,胸部正侧位片发现食管近段盲端。EA分型[4]:按照Gross法分五型,即A型(EA不合并TEF);B型(EA合并近端TEF);C型(EA合并远端TEF);D型(EA合并近端+远端TEF);E型(TEF无闭锁或H型TEF)。
1.2 方法
采用凯华文大联合实验室开发的病案管理及数字化系统查阅满足标准的新生儿。记录一般情况:胎龄、出生体重、入院日龄、住院时间、围产期情况(母孕期疾病史、分娩方式、羊水情况、宫内窘迫及生后窒息史等)、临床表现、合并症及转归等,采用回顾性研究方法,分析患儿诊断过程。
1.3 统计学处理
正态分布的计数资料以均数±标准差(x±s)表示,两样本均数比较采用t检验,率的比较采用χ2检验或Fisher确切概率法,P<0.05为差异有统计学意义。非正态分布计量资料采用中位数(M)和百分位数间距(P25,P75)表示。
2 结果
2.1 一般情况
研究期间共收治新生儿总数16 146例,确诊EA者28例,占总住院人数的1.73‰。其中男22例,女6例,男女比例3.7:1;足月儿23例(占82.1%),早产儿5例;胎龄32~40周,平均胎龄(38.1±2.0)周;出生体重1300~4000 g,平均(2769±215)g;小于胎龄儿(SGA)10例,占35.7%,其中80.0%早产儿为SGA,早产儿的SGA发生率明显高于足月儿,两两比较差异有统计学意义。剖宫产分娩13例,占46.4%;母孕期有并发症者(仅为妊娠期高血压及糖尿病)共6例,占21.4%;产前超声提示羊水多者14例,占50.0%;产前超声可疑EA者2例,占7.1%。所有患儿均于48 h内入院,平均入院日龄5 h(中位数);内科住院时间6~46 h,平均(32±23)h,早产儿内科住院时间较足月儿长,两两相比有显著差异。其中1例早产儿因症状不典型用机械通气治疗,住院9 d方确诊转入外科治疗。见表1。
2.2 临床特点
28例患儿中,C型EA共26例,占92.9%,A型EA 1例,E型EA 1例。足月儿组和早产儿组两组主要症状相比有显著差异(χ2=15.857,P=0.000)。足月儿组主要症状以吐沫最常见,占47.8%,与早产儿组吐沫发生率相比有显著差异(χ2=3.939,P=0.047);其次为呕吐及喉中痰鸣,各占21.7%;而早产儿组主要症状以早产为主,与足月儿组早产发生率相比较有显著统计学差异(χ2=15.456,P=0.003);1例为可疑EA,1例呼吸促。见表2。足月儿和早产儿两组症状比较所有患儿中,9例(32.1%)生后早期于外院开奶,其中3例产前超声提示羊水多。8例开奶后出现上述症状,置胃管受阻确诊,1例摄胸片发现胃管于上纵隔内折返确诊。余19例患儿生后均未开奶,16例开奶前洗胃或吸痰时置导管有阻力或吐沫明显未开奶;2例产前超声可疑EA未开奶,1例早产儿生后出现呼吸促并逐渐加重,无创呼吸机辅助通气下低氧血症难以缓解,予机械通气呼吸支持后,唾液过多、饮奶呛咳等常见症状未出现,撤机后方明确诊断。研究发现,所有患儿肺部均可闻及不同程度的肺部啰音,清理呼吸道分泌物后啰音减轻或消失,肺部体征时轻时重。
2.3 合并症及转归
合并症包括先天性心脏病(房间隔缺损、室间隔缺损、动脉导管未闭)、指畸形(并指、多指)、胸椎畸形、低血糖、附耳、肛门闭锁、血管瘤、隐睾、肾积水,其中合并先天性心脏病最常见,占60.7%,其中早产儿组和足月儿组相比,合并症并无统计学差异(χ2=0.067,P=1.000)。见表3。10例因各种原因放弃转科,18例转入外科手术治疗存活,占64.2%。
3 讨论
欧洲研究显示EA更易发生于男性、白种人、初孕者、兄弟姐妹曾患EA者、高龄产妇,一半病例合并其他畸形,10%病例合并染色体畸形或单基因病[5]。本研究亦发现男女比例3.7:1,男性明显高于女性,合并先天性心脏病者60.7%,均与国外数据相似,未发现双胎发病者,可能与本研究例数少有关。有研究发现产前可疑EA者自然分娩率为42%,与未怀疑EA者相比并无差异,选择剖宫产常因分娩中的异常因素所决定而并非EA本身[6]。本研究中EA患儿自然分娩率为57.6%,2例可疑EA者均为自然分娩,故分娩方式与EA发生并无关联。
产前诊断影响EA患儿生存率,产前超声提示羊水多可疑EA时,宫内减羊水处理可降低早产风险。也有助于产妇选择有外科手术条件的医院分娩,可避免转运风险。产前超声检测EA有一定难度,国外报道产前超声提示EA者约10%~50%,多中心研究很少,仅有一些地域性报道[7-8]。超声提示小或无胃泡是EA最敏感指标[9],但其作为预测值亦有限,预测范围在44%~56%之间,因为当羊水通过瘘管填满胃泡时,小胃泡及羊水过多均不易判定。所以几乎所有A型EA(单纯EA不伴TEF)者超声提示为羊水多、小或无胃泡,而其他类型者仅有46.3%有此表现。法国一项研究表明,A型EA不伴TEF者超声提示羊水多者占86%,伴TEF者仅占12%,但临床所见EA合并TEF是最常见类型,这也就是产前诊断EA少的原因[10,11]。产前超声提示羊水多时生后应早期下胃管检测,即使下胃管顺利,仍有极少数患儿胃管于盲端附近折返不易察觉,摄X线胸腹片即可发现。如高度怀疑EA时口服造影剂并摄X线胸腹片简單有效,可显示食管盲端于上纵隔内可以确诊,如胃肠道含气提示存在食管远端TEF。极少情况下,胃管可能从食管到气管,再从食管气管瘘到达胃内,即使胸腹片提示胃管已达胃内仍不能完全除外EA。本研究中产前超声提示EA者仅7.1%,低于国外报道,产前超声提示羊水多的患儿中仍有1/3生后早期给予开奶,出现吐沫、呕吐、胃管折返表现最终确诊。故临床工作中应关注羊水情况,产前未能明确食管闭锁,但提示羊水多者应在孕检中多次随访超声检测,必要可做胎儿磁共振检查,期待明确诊断;未明确诊断者宜早期置胃管检测,并注意观察胃管有无折返,无造影条件的基层医院可留置胃管注入造影剂同时摄胸腹片观察造影剂情况,避免喂养引起吸入性肺炎,延误手术时机。即使未提供羊水多者,亦有发生EA可能。
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(收稿日期:2019-05-24)