Objective: To investigate the clinical diagnosis and treatment of congenital laryngotracheoesophageal cleft (LTEC) in children. Methods: The clinical data of 8 children (including 7 males and 1 female)with congenital laryngotracheoesophageal cleft from January 2016 to June 2020 were retrospectively analyzed. The median diagnosing age was 3.75 months (5 days to 12 months). According to the modified Benjamin Inglis classification proposed by Sandu in 2006,there were 3 cases of type Ⅱ, 3 cases of type Ⅲa, 1 case of type Ⅲb and 1 case of type Ⅳa. All children were followed up regularly. Results: Six patients were treated for recurrent bronchopneumonia and aspiration during feeding. The patients were first treated in the pneumology departmentt or intensive care unit. Six patients combined with other malformations. Endoscopic repair operations were performed in 6 cases (3 cases of type Ⅱ, 3 cases of type Ⅲ a), 1 case of LTEC was operated through cervical approach, and 1 case of type IVa LTEC associated with VACTERL was repaired under thoracoscope combined with suspension laryngoscope. Seven patients underwent tracheotomy before or during the repair operations. Gastrostomy was performed in 2 children. The operations were successfully performed in all cases. Three children with type Ⅱ LTEC recovered well and decannulated. One case of type Ⅲa was followed up for 5 months with occasionally choking while feeding. Two cases of type Ⅲa, 1 case of type Ⅲb and 1 case of type Ⅳa died due to severe reflux, tracheomalacia or respiratory failure. Conclusions: Congenital LTEC is a rare congenital malformation which is difficult to diagnose for the poor specificity of clinical manifestations. LTEC needs to be classified by endoscopy examination under general anesthesia. Severe cases of LTEC have poorer outcomes than the mild cases, and the perioperative managements need multi-disciplinary cooperation to reduce the mortality.
目的： 探讨儿童先天性喉气管食管裂（laryngotracheoesophageal cleft，LTEC）的临床诊断与治疗方法。 方法： 回顾性收集首都医科大学附属北京儿童医院2016年1月至2020年6月确诊Ⅱ~Ⅳ型LTEC的8例患儿临床资料，并进行分析。8例患儿中，男7例，女1例，诊断时年龄5 d~12个月，中位年龄为3.75个月。根据2006年Sandu提出的改良Benjamin-Inglis 分型：Ⅱ型3例，Ⅲa型3例，Ⅲb型1例，Ⅳa型1例。所有患儿常规随访。 结果： 6例患儿因反复支气管肺炎和喂养时呛咳症状就诊，首诊科室为呼吸内科、重症监护病房等，6例伴其他畸形。8例患儿的治疗：内镜下喉气管食管裂修复术6例（3例Ⅱ型，3例Ⅲa型），1例（Ⅲb型）经颈部正中入路LTEC修复术，1例（Ⅳa型）新生儿合并VACTERL［V：脊柱（vertebral），A：肛门（anal），C：心脏（cardiac），TE：气管和食管（tracheoesophaqeal），R：肾脏（renal），L：肢体（limb）］联合畸形行胸腔镜联合支撑喉镜下喉内镜修复手术。8例患儿中有7例行气管切开，2例行胃造瘘术。所有患儿均成功完成修补手术，随访时间为5个月至3年，其中4例存活（3例Ⅱ型患儿痊愈，1例Ⅲa型患儿术后5个月喂养有呛咳），4例（2例Ⅲa型、1例Ⅲb型、1例Ⅳa型）因严重反流、气管软化、呼吸衰竭死亡。 结论： 先天性LTEC的临床表现特异性差、确诊困难，需要通过内镜确诊分型。患儿确诊后需要行喉气管食管裂修复手术，但重型（Ⅲ型和Ⅳ型）患儿预后较差，围手术期管理需要多学科协作。.