先天性气管软骨环缺失4例

Four cases of congenital absence of tracheal cartilage ring

对2017年11月至2019年8月山东大学齐鲁儿童医院收治的4例先天性气管软骨环缺失患儿的临床资料进行回顾性分析。4例患儿确诊年龄为2个月~1岁。1例出生后无明显症状,之后反复喘息发作3次;余3例患儿出生后即有症状,表现为喉鸣、反复咳喘。4例患儿支气管镜检查均见气管局部管腔呈类圆形、明显向心性狭窄,但镜身可顺利通过,管壁未见软骨结构。其中3例行胸部增强CT检查均表现为气管局限性狭窄,呈沙漏样,不伴血管环畸形。4例患儿均手术治疗,其中3例恢复良好,1例术后死于重症感染。

The clinical data of 4 children with congenital absence of tracheal cartilage ring in Qilu Children′s Hospital of Shandong University from November 2017 to August 2019 were retrospectively analyzed.The age of the 4 cases was from 2 months to 1 year.One case had no obvious symptoms after birth,and then had repeated wheezing attacks for 3 times.Another 3 cases had symptoms after birth,such as throat ringing,repeated cough and asthma.Bronchoscopy in 4 cases indicated that the local lumen of trachea was round and centripetal stenosis,but the body of bronchoscope could pass smoothly,and no cartilage structure was found in the wall of trachea.Among them,3 cases of chest enhanced CT examination displayed local and hourglass-like tracheal stenosis,without vascular ring malformation.All the 4 cases were treated by operation,of which 3 cases recovered well and 1 case died of severe infection after operation.