laryngotracheoesophageal cleft

喉气管食管裂
  • 文章类型: Journal Article
    目的:描述通过前入路手术修复III型或IV型喉气管食管裂(LTEC)后软骨的临床结果。
    方法:在2017年5月至2022年5月期间,对III型或IV型LTEC患者进行了图表视图。收集并分析人口统计学特征和手术结果。
    结果:最终纳入7例患者。五名患者被诊断为III型LTEC,两名患者被诊断为IV型LTEC。除一名患者外,所有患者都幸存下来并蓬勃发展。四名患者能够以可接受的声音成功拔管,两名患者进行了气管造口术。五名患者被认为对所有一致性食物都是安全的,一名患者对增稠食物是安全的。平均随访49个月(18-83个月)后,未观察到并发症或复发.
    结论:喉裂前入路联合后路软骨移植修复裂隙是治疗III型或IV型LTEC的有效且安全的方法,可以闭合LTEC和重建环状软骨板,以避免术后形成气管食管瘘或声门下狭窄。严重的气管软化和GERD是手术失败的两个主要原因。
    OBJECTIVE: To describe the clinical outcomes of patients who underwent surgical repair through an anterior approach that involved interposition a posterior cartilage for Type III or Type IV laryngotracheoesophageal cleft (LTEC).
    METHODS: A chart view was performed on patients with Type III or Type IV LTEC between May 2017 and May 2022. Demographic features and surgical outcomes were collected and analyzed.
    RESULTS: Seven patients were finally included. Five patients were diagnosed with Type III LTEC and two patients were diagnosed with Type IV LTEC. All but one patients survived and thrived. Four patients were able to successfully extubate with acceptable voice, and two patients were tracheostomized. Five patients were deemed safe for all consistencies food and one was safe for thickened food. After a mean follow-up of 49 months (18-83 months), neither complications nor recurrences were observed.
    CONCLUSIONS: An anterior laryngofissure approach to the cleft repair with a posterior cartilage grafting is an effective and safe treatment for Type III or IV LTEC, which enables closure of LTEC and reconstruction of cricoid plate in order to avoid tracheoesophageal fistula formation or subglottic stenosis postoperatively. Severe tracheomalacia and GERD are two main causes for surgical failure.
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  • 文章类型: English Abstract
    Congenital laryngeal cleft is a rare airway malformation, mainly manifested as choking, feeding difficulties, which affects the growth and development of children. Patients with a severe laryngeal cleft may have recurrent aspiration, leading to cyanotic spells, or even death. Advances in development of endoscopic techniques have made early diagnosis possible. Depending on the degree of cleft, management may involve a variety of approaches ranging from medical management alone to open repair. Therefore, it is important for pediatric ENT doctors to diagnose and evaluate in clinical practice. This consensus statement, developed by the Pediatric otorhinolaryngology Professional Committee of the Pediatrician Branch of the Chinese Medical Doctor Association, provides comprehensive recommendations and standardized guidance on diagnosis and management of laryngeal cleft, based on symptomatology, physical examinations, and laboratory tests.
    摘要: 先天性喉裂是一个罕见的气道畸形,主要表现为呛咳、喂养困难等,影响患儿的生长发育;严重的喉裂可以表现为严重的呛咳误吸,导致阵发性青紫,甚至死亡。随着内镜技术的发展,使得喉裂可以被早期发现和诊断。不同类型的喉裂治疗方案差异较大,早期的诊断与评估对于儿童耳鼻喉科医生至关重要。中国医师协会儿科医师分会儿童耳鼻咽喉专业委员会联合全国多家儿童医疗中心讨论拟定诊治共识,从症状学、客观检查等方面给出喉裂的诊断治疗方案,为临床规范化诊疗提供指导性意见。.
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  • 文章类型: Journal Article
    A laryngotracheoesophageal cleft (LC) is a rare congenital anomaly of the upper aerodigestive tract resulting from the absence of fusion of the posterior cricoid lamina, which affects an abnormal communication between the larynx, trachea and esophagus. The genetic etiology of LC remains elusive. The involvement of genetic factors in the development of LC is suggested by reports of familial occurrence, and the increased prevalence of component features among first-degree relatives of affected individuals and murine knockout models. No consistent pattern of inheritance has been found in nonsyndromic patients, except for cases associated with described syndromes. Once the syndrome related to the laryngeal cleft is considered, an active search for the cleft must be initiated. The genetic evaluation of patients with LCs should be guided by the type and location of the malformation, specific medical history and a detailed physical examination. The application of genetic approaches, such as microarrays and exome sequencing might lead to elucidating the etiology of LCs.
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