摘要：
Objectives To provide the experience about the diagnostic process and following management,and to discuss the outcome and predictors in children with laryngeal cleft (LC).Methods A retrospective case study was conducted at an academic children's hospital.Thirty children were diagnosed as laryngeal cleft between January 2016 and April 2017.Airway evaluations were performed using both flexible and rigid endoscopy,and swallowing evaluations were performed using fiberoptic endoscopic examination of swallowing or modified barium swallow.Results Of 30 cases,18 were male and 12 were female,ranging in age from birth to 8 years.Two cases were diagnosed as type 0 LC,and they were offered thickened liquid without medication.Throughout follow-up,they remained asymptomatic and showed no respiratory complications.Nineteen children were diagnosed as type Ⅰ LC.Six of them were significantly improved by anti-reflux therapy and feeding instructions.Four children were concomitant with swallowing dysfunction and/or neuromuscular disorders,and they were given a tracheotomy and routine management.Another 4 children were submitted surgical repair when routine treatment failed,and their symptoms were relieved.Five children were concomitant with larygomalacia,and their symptoms were totally ameliorated by supraglottoplasty.Three children were diagnosed as type Ⅱ LC.Two of them received surgical repair and clinically improved,and the rest one was treated by anti-reflux therapy and still under follow-up.Three children were diagnosed as type Ⅲ LC.One of them was underwent surgical repair and clinically improved.Two children were tracheotomized and treated by anti-reflux therapy.Three cases were diagnosed as type Ⅳ LC at birth and no one survived.Conclusions Laryngeal cleft is a rare congenital anomaly manifesting with a variety of symptoms,including swallowing disorder,aspirations,dyspnea,stridor and hoarseness.Diagnosis and treatment of laryngeal clefts is a challenge.The best way to evaluate the LC is FEES by laryngeal endoscopy combined with MLB.Cases with type 0-Ⅰ mostly were signifieantly improved by antireflux therapy and feeding instructions.When routine treatment failed,surgical repair is needed.All the cases with LC type Ⅱ-Ⅲ need surgical repair as soon as possible.For type Ⅳ cases,early diagnosis,appropriate treatment and management help to reduce mortality and morbidity.%目的 回顾性分析在复旦大学附属儿科医院耳鼻咽喉头颈外科诊治的30例先天性喉裂患者的病历资料,通过对其诊断和评估方法的研究以及疗效的分析,制定出有效的评估方法和治疗方案.方法 分析2016年1月至2017年5月喉裂患者30例,男18例,女12例;年龄范围1d至8岁,中位年龄3.5个月.喉裂评估方法:清醒状态下电子纤维喉镜检查、功能性内镜下吞咽功能评估、全麻下保留自主呼吸的显微喉气管硬镜检查、气管内插管透气实验,以及核素的吞咽正向肺吸入检查,比较各种客观检查方法的优势.根据不同的喉裂分型以及合并的畸形、功能异常等给出相应的治疗方案.术后1～3个月行症状评分和客观检查评估.结果 0型喉裂2例,Ⅰ型喉裂19例,Ⅱ型喉裂3例,Ⅲ型喉裂3例,Ⅳ型喉裂3例.其中合并吞咽功能异常4例,合并声门下狭窄6例,其中误吸阳性7例.治疗方案:2例0型喉裂患者予以非手术治疗;19例Ⅰ型喉裂患者中6例予以非手术治疗,4例合并吞咽功能异常者予以气管切开,4例予以非手术治疗后无效行喉裂修补,5例合并喉软化症无误吸者,行声门上成形术;Ⅱ型喉裂3例,2例行喉裂修补,1例行喉裂修补合并气管切开;3例Ⅲ型喉裂中1例行开放性喉裂修补,2例行气管切开;3例Ⅳ型喉裂因合并多发畸形,死亡.治疗有效率:0型为100％;Ⅰ型为94.74％;Ⅱ型为100％;Ⅲ型为66.67％;Ⅳ型无治疗经验.结论 显微喉气管硬镜检查合并气管内插管透气实验联合吞咽功能评估是对喉裂分级、吞咽功能以及相关气管畸形等为最有效的评估手段.对于0～Ⅰ型喉裂不合并其他畸形和吞咽功能异常者,多数经非手术治疗有效,无效时行修补手术,Ⅱ～Ⅲ型喉裂需通过手术修补;Ⅳ型喉裂多合并多发畸形,死亡率极高.
