Clinical practice guidelines for pediatric open laryngotracheoplasty(2026)
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摘要: 喉气管狭窄是儿童常见且病因复杂的疾病,病情严重威胁生命。开放性喉气管成形术是其关键治疗手段,对于围手术期的管理及术者操作技术的要求较高,临床实践中仍存在诸多需要规范的问题。本指南依据循证医学原则,综合国内外最新文献与多学科专家共识,旨在规范并完善喉气管成形术的围术期评估与管理、手术方法与流程,同时梳理相关技术进展,为我国从事儿童喉气道疾病诊治的相关医务人员提供参考依据。Abstract: Laryngotracheal stenosis is a common pediatric disease with complex etiologies, and its severe condition poses a life-threatening risk. Open laryngotracheoplasty serves as the key therapeutic intervention. Given the high requirements for perioperative management and the operator's surgical proficiency, numerous issues requiring standardization persist in clinical practice. Based on the principles of evidence-based medicine, this guideline integrates the latest domestic and international literature as well as multidisciplinary expert consensus. It aims to standardize and refine the perioperative assessment and management, surgical methods and procedures of open laryngotracheoplasty, while systematically summarizing the development of related technologies, thereby providing a reference for medical professionals engaged in the diagnosis and treatment of pediatric laryngotracheal diseases in China.
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Key words:
- pediatric /
- laryngotracheal stenosis /
- laryngotracheoplasty /
- guidelines
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表 1 喉气管狭窄相关重要概念与术语
序号 术语(英文,缩写) 定义 1 喉气管狭窄(Laryngotracheal Stenosis,LTS) 指喉部或气管的管腔因先天性发育异常或者后天创伤、感染等原因发生狭窄,引发危及生命的气道梗阻性疾病[2] 2 声门下狭窄(Subglottic Stenosis,SGS) 指位于声门以下至环状软骨下缘的气道,部分发生管腔结构变窄的疾病。可由先天性因素(如环状软骨发育畸形、先天性喉蹼等)或后天性因素(如气管插管、气管切开、外伤、感染、肿瘤等)引起 3 先天性声门下狭窄(Congenital Subglottic Stenosis,Congenital SGS) 出生时即存在的声门下结构异常导致的狭窄,通常因环状软骨发育异常或黏膜下组织增生引起 4 继发性声门下狭窄(Acquired Subglottic Stenosis,Acquired SGS) 后天因素(如长期插管、外伤、感染或炎症性疾病)导致的声门下狭窄 5 插管后声门下狭窄(Post-intubation Subglottic Stenosis,Post-intubation SGS) 因长期气管插管(通常超过48 h)引起的声门下损伤,表现为黏膜溃疡、肉芽组织增生或纤维化狭窄 6 喉蹼(Laryngeal Web) 喉部先天性或获得性膜状结构,连接声带前段,可能导致声门下或声门区狭窄 7 声门下血管瘤(Subglottic Hemangioma) 声门下区域的良性血管肿瘤,由增生血管组织构成,常导致进行性气道狭窄(狭窄程度可达10%~99%),多见于婴幼儿 8 肉芽肿性多血管炎相关声门下狭窄(GPA-associated Subglottic Stenosis,GPA-associated SGS) 肉芽肿性多血管炎(一种ANCA相关性血管炎)引起的声门下或气管狭窄,表现为复发性炎症和纤维化病变 9 喉气管成形术(Laryngotracheoplasty,LTP) 外科手术修复声门下或气管狭窄,常使用自体软骨(如肋软骨、甲状软骨或舌骨)作为移植物扩大气道管径 Ⅰ期喉气管成形术(Single-stage LTP):指在修复狭窄的同时不依赖长期气管造口 Ⅱ期喉气管成形术(Double-stage LTP):指手术修复后需保留气管造口作为临时气道,待稳定后再行闭口 10 显微喉气管支气管镜检查(Microlaryngotracheoscopy,MLB) 使用内镜对喉及气管进行精细检查,用于诊断狭窄、异物或肿瘤性病变 11 环状软骨前裂开(Anterior Cricoid Split,ACS) 外科手术通过正中裂开环状软骨前部以解除声门下狭窄 12 环状软骨后裂开(Posterior Cricoid Split,PCS) 通过环状软骨后部裂开并植入移植物(如肋软骨)以扩大声门下腔的手术方式[3] 13 肋软骨移植物(Costal Cartilage Graft,CCG) 使用自体肋软骨为移植物,用于喉气管重建术中支撑和扩大狭窄的气道 14 甲状软骨移植物(Thyroid Cartilage Graft,TCG) 使用自体甲状软骨作为移植物在喉气管成形术中提供结构支持[3] 15 舌骨移植物(Hyoid Graft) 舌骨或其部分作为移植物用于喉部重建手术 16 气管切开术(Tracheotomy) 外科手术在颈段气管前壁切开,建立有效气道通路,用于严重声门下狭窄或气道梗阻的患儿 17 气管造口术(Tracheostomy) 外科手术在颈段气管前壁造口,建立长期气道通路,用于严重呼吸道梗阻的患儿 18 气管造口前壁塌陷(Anterior Tracheal Wall Collapse) 气管造口术后因软骨支撑不足或软组织增生导致的前壁塌陷 19 气道支架(Airway Stent) 用于喉气管成形术后气道支撑,主要用于Ⅱ期喉气管成形术,有利于气道内黏膜上皮化和气道塑型 20 T型管(T-tube) 用于Ⅱ期喉气管成形后的气道支架,T型的三通管道,可通过颈部造口通气和进行气道护理。 21 纤维内镜吞咽功能评估(Fiberoptic Endoscopic Evaluation of Swallowing,FEES) 通过经鼻置入纤维内镜,直接观察咽部及喉部结构与分泌物管理,并让受试者吞咽不同性状(液体、糊状、固体、染色食物等)的试验物,评估吞咽安全性与效率的一种检查,提供吞咽后的残留、渗入或误吸的证据。 22 改良钡剂吞咽功能评估(Modified Barium Swallow Study,MBS) 该检查是在透视下记录吞咽过程的动态X线造影,用于客观评估口咽期吞咽生理及误吸或残留,并指导吞咽障碍的诊断与治疗策略。它通常与FEES并列,被广泛认为是评估口咽期吞咽功能的重要检查手段之一 表 2 多学科评估详情表
科室/团队 主要角色与职责 耳鼻咽喉头颈外科 牵头实施耳鼻咽喉相关专科检查与喉功能评估;定位狭窄部位、明确狭窄性质与程度;统筹协调多学科诊疗意见,主导制定和优化手术方案 麻醉科 系统评估手术麻醉风险及气道狭窄分级;制定个体化困难气道管理与术中通气策略;维持术中生命体征平稳;参与制定术后镇静与镇痛方案 呼吸与介入科 评估基础肺功能,量化上气道梗阻严重程度;优化围手术期慢性肺部疾病管理;参与评估术后拔管时机 重症监护室 负责术后气道管理与呼吸支持;气道支架、T管及吻合口的护理;实施规范化的镇静镇痛;维持循环功能稳定;加强营养支持,预防并发症 消化内科 评估与管理胃食管反流/喉咽反流(术后再狭窄的重要危险因素);指导抗反流药物治疗;评估抗反流手术的必要性与时机 言语治疗科与康复科 建立术前嗓音、吞咽功能基线数据库;开展健康宣教,预先指导术后呼吸、嗓音及吞咽训练方法,促进功能恢复 表 3 儿童喉气管成形术后疗效评分
序号 评分项目 评分依据 评分 0分 1分 2分 3分 1 误吸 术后呛咳频率及严重程度,反映气道保护功能恢复情况[38] 无呛咳(正常饮食) 轻度呛咳(偶发,不影响进食) 中度呛咳(频繁,需调整饮食质地) 重度呛咳(持续,需鼻饲或管饲) 2 吞咽功能 采用视觉模拟量表(VAS)评估吞咽障碍程度(0~10分,分值越高障碍越严重)[39] VAS ≤2分(吞咽正常) VAS 3~4分(轻度障碍,偶有吞咽困难) AS 5~6分(中度障碍,需稠食喂养) VAS ≥7分(重度障碍,依赖管饲) 3 声音嘶哑程度 采用CAPE-V(Consensus Auditory-Perceptual Evaluation of Voice)共识听觉感知嗓音评估量表(总分0~100分)[40] CAPE-V ≤20(声音正常) CAPE-V 21~40(轻度嘶哑,不影响交流) CAPE-V 41~60(中度嘶哑,嗓音粗糙) CAPE-V ≥61(重度嘶哑,声带固定或闭合不全) 4 呼吸困难程度 参考喉梗阻Ⅰ~Ⅳ分度,术后随访采用喉梗阻分级评估活动耐量 [41] 无呼吸困难(活动正常) 轻度呼吸困难(活动后轻微气促) 中度呼吸困难(静息时轻度气促,活动后加重) 重度呼吸困难(静息时明显气促,三凹征阳性) 5 喉内镜瘢痕增生 通过术后喉镜检查,按Myer-Cotton分级评估再狭窄程度 [35] 无狭窄(Myer-Cotton Ⅰ级) 轻度狭窄(Myer-Cotton Ⅱ级,阻塞51%~70%) 中度狭窄(Myer-Cotton Ⅲ级,阻塞71%~99%) 重度狭窄(Myer-Cotton Ⅳ级,完全阻塞) 6 气管插管拔管率 是否成功拔管及拔管时间 [42-43] 术后一次性拔管成功 T型管/气管套管留置6~12个月拔管 带管>12个月后拔管 拔管失败(依赖气管切开) 7 二次手术必要性 是否因再狭窄或并发症需二次手术,再狭窄需二次手术 [39, 44] 无需二次手术 术后肉芽增生需内镜干预 因移植物感染/坏死需局部修复 需挽救性开放手术(如再次重建) -
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