摘要
目的:总结以呛咳起病的先天性喉裂新生儿病例的临床特点和预后,提高新生儿专科医护人员对先天性喉裂的诊治水平。方法:本研究为单中心回顾性病例系列报告。以"呛咳"为关键词,结合病案首页信息,检索电子病历系统,总结最终确诊为先天性喉裂新生儿的临床特点及预后资料。研究时限为2017年1月至2021年2月。结果:共7例患儿确诊为先天性喉裂,占同期因呛咳入院患儿总数的2.2%,其中男婴6例,胎龄38(36,39)周,出生体重2 820(2 255,3 420)g,入院日龄18(5,20)d,住院时间26(6,45)d。常见临床症状为7例患儿呛咳和5例患儿青紫。7例先天性喉裂患儿中6例可明确分型,分别为Ⅰ型2例、Ⅲ型3例、Ⅳ型1例。7例患儿均同时伴有其他部位/气管畸形或先天异常,其中4例为VACTERL综合征。3例患儿接受了喉裂修补术(2例Ⅲ型,1例Ⅳ型),均术后不久死亡。另4例患儿未接受喉裂修补术(2例Ⅰ型,1例Ⅲ型,1例分型不详),其中1例Ⅰ型喉裂合并气管食管瘘患儿接受了气管食管瘘修补术,随访至2岁6个月,能正常饮食,1例Ⅰ型喉裂患儿带胃管出院,随访至1岁8个月,可正常饮食,这2例患儿生长发育与同龄儿无明显差异;1例Ⅲ型喉裂患儿失访;1例分型不详患儿随访至40 d,仍不能经口喂养,体格发育明显落后于同龄儿。结论:先天性喉裂罕见,呛咳是其主要症状,多合并其他畸形,诊断和分型需借助纤维喉镜及全麻下支撑喉镜联合支气管镜检查,治疗难度大,Ⅰ型预后较好,Ⅲ型及Ⅳ型预后差。
Objective To summarize the clinical features and prognosis of neonates with congenital fissure of larynx due to choking,and improve the diagnosis and treatment of congenital fissure of larynx by neonatal medical professionals.Methods This study was a single-center retrospective case series report.With"choking"as the key word,combined with the information from the first page of the medical record,we searched in the electronic medical record system,and summarized the clinical characteristics and prognosis of neonates diagnosed as congenital fissure of larynx.The study period was from January 2017 to February 2021.Results A total of seven cases were diagnosed as congenital fissure of larynx,accounting for 2.2%of the total number of hospitalized children due to choking during the same period.Among them,six cases were male,gestational age was 38(36,39)weeks,birth weight was 2820(2255,3420)g,admission age was 18(5,20)days and hospitalization duration was 26(6,45)days.The common clinical symptoms were choking(7/7)and cyanosis(5/7).Six of the seven children with fissure of larynx were clearly classified as typeⅠ(two cases),typeⅢ(three cases),and typeⅣ(one case).All the seven cases were accompanied by other site/tracheal malformations or congenital abnormalities,among which four(4/7)cases had VACTERL syndrome.A total of three children(two cases of typeⅢand one case of typeⅣ)underwent laryngeal cleft repair operation,and all died shortly after surgery.Four cases didn′t receive laryngeal cleft repair operation(two cases of typeⅠ,one case of typeⅢ,one case of parting was unknown),one case with typeⅠfissure of larynx accepted the tracheoesophageal fistula repair operation only,who was followed up until the age of two years and six months,and he could have normal diet,one case of typeⅠfissure of larynx with gastric tube discharge,who was followed up to one year and eight months old,could be normal diet too,the two cases of children with no difference between the growth with their peers;one case with typeⅢfissure of larynx was lost to follow-up;One case,whose classification was unknown,was followed up until he was 40 days old,and still could be fed orally.The physical development of the child was significantly behind that of the same age.Conclusion Congenital fissure of larynx is rare,with choking as its main symptom and other malformations.Diagnosis and classification require fiberlaryngoscope and bolting laryngoscope under general anesthesia combined with bronchoscopy,which is difficult to treat.TypeⅠhas a good prognosis,while typeⅢandⅣhave poor prognosis.
作者
沈艳华
靳绯
齐宇洁
吴海兰
陈璐
郑旭
张杰
黑明燕
Shen Yanhua;Jin Fei;Qi Yujie;Wu Hailan;Chen Lu;Zheng Xu;Zhang Jie;Hei Mingyan(Department of Neonatal Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)
出处
《中国小儿急救医学》
CAS
2022年第8期626-630,共5页
Chinese Pediatric Emergency Medicine
基金
北京市医院管理中心儿科协同发展中心儿科专项创新推广项目(XTCX201816)。
关键词
呛咳
先天畸形
罕见病
喉裂
临床特点
预后
病例系列
Choking
Congenital malformation
Rare diseases
Fissure of larynx
Clinical features
Prognosis
Case series
