Tracheal Stenosis

气管狭窄
  • 文章类型: Journal Article
    背景:小儿喉气管狭窄通常需要开放气道重建。虽然这些手术建立了充分通气的气道,许多患者随后出现发音困难。许多研究报告了与声音有关的结果。
    目的:本研究旨在评估开放式气道重建后儿科患者的发音障碍,专注于声学参数,感知语音质量,和语音相关的生活质量。
    方法:在6个数据库中使用系统评价和荟萃分析(PRISMA)指南的首选报告项目进行全面搜索,确定了涉及接受开放式气道重建并报告术后声乐声学参数的儿科患者的文章。感知语音质量,与语音相关的生活质量,或声乐力学。文章进行了偏倚风险评估,和共同结局采用meta分析进行定性和定量综合.
    结果:在4089篇文章中,包括21个,涉及497名儿科患者。喉气管成形术是最常见的手术,其次是环气管切除术。语音共识听觉感知评估(CAPE-V)量表经常用于评估语音质量,平均得分为55.6[95%置信区间(CI):47.9-63.3]。使用儿科语音障碍指数(pVHI)和儿科语音相关生活质量调查测量语音相关生活质量,平均得分为35.6分(95%CI:21.4-49.7)和83.7分(95%CI:74.1-93.2),分别。基频为210.5(95%CI:174.6-246.3)。其他常见发现包括声门上发声,前连合钝化,后声门分离,和异常的声带活动。
    结论:在开放气道重建后出现发音困难的儿童患者表现出语音质量中度下降和语音相关生活质量下降。然而,研究方案和使用的结局衡量标准存在不一致.在气道重建过程中保持语音质量对于避免对生活质量的负面影响至关重要。
    BACKGROUND: Pediatric laryngotracheal stenosis often requires open airway reconstruction. While these surgeries establish an airway for adequate ventilation, many patients develop subsequent dysphonia. Numerous studies have reported outcomes related to voice.
    OBJECTIVE: This study aims to evaluate dysphonia in pediatric patients following open airway reconstruction, focusing on acoustic parameters, perceptual voice quality, and voice-related quality of life.
    METHODS: A comprehensive search using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines across 6 databases identified articles involving pediatric patients who underwent open airway reconstruction and reported postoperative vocal acoustic parameters, perceptual voice quality, voice-related quality of life, or vocal mechanics. Articles were assessed for bias risk, and common outcomes were synthesized qualitatively and quantitatively using meta-analyses.
    RESULTS: Among 4089 articles, 21 were included, involving 497 pediatric patients. Laryngotracheoplasty was the most common procedure followed by cricotracheal resection. The Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) scale was frequently used to assess voice quality, with a mean score of 55.6 [95% confidence intervals (CIs): 47.9-63.3]. Voice-related quality of life was measured using the pediatric Voice Handicap Index (pVHI) and Pediatric Voice-Related Quality of Life Survey, with mean scores of 35.6 (95% CI: 21.4-49.7) and 83.7 (95% CI: 74.1-93.2), respectively. The fundamental frequency was 210.5 (95% CI: 174.6-246.3). Other common findings included supraglottic phonation, anterior commissure blunting, posterior glottic diastasis, and abnormal vocal cord mobility.
    CONCLUSIONS: Pediatric patients experiencing dysphonia after open airway reconstruction exhibited moderately decreased voice quality and reduced voice-related quality of life. However, there was inconsistency in study protocols and outcome measures used. Preserving voice quality during airway reconstruction is crucial to avoid negative impacts on quality of life.
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  • 文章类型: Systematic Review
    背景:Slide气管成形术已成为先天性气管狭窄(CTS)的金标准手术。这种情况很少见,手术可能具有挑战性,由三级中心经验丰富的外科医生进行。已经发表了一些涉及相对较小的队列的报告。这篇综述的目的是评估用于CTS的小儿滑动气管成形术的术后死亡率和发病率。
    方法:根据PRISMA指南进行系统文献综述。使用与图书馆员合作定义的搜索策略来筛选Medline和EMBASE数据库。我们纳入了文章,报道了用于治疗儿童CTS的滑动气管成形术的术后死亡率。当包括至少10名患者时。
    结果:共审查了932篇文章,15项研究符合资格,共845例患者.术后总死亡率为9.3%,大多数死亡与气道有关。术后开放翻修手术率为2.8%,内镜翻修率为27.6%。
    结论:这项研究强调了手术前要考虑的关键因素,并有助于预测CTS患儿的术后随访注意事项。几个因素被确定为死亡率的预测因素,包括年龄,手术时的体重以及与肺发育不全或发育不全的关联。
    结论:尽管近年来由于疗效较好,滑动气管成形术越来越受欢迎,它仍然是一个有死亡风险的重大手术,需要多学科管理.
    BACKGROUND: Slide tracheoplasty has become the gold standard surgery for congenital tracheal stenosis (CTS). This condition is rare and the surgery can be challenging and is performed by experienced surgeons in tertiary centers. A few reports involving relatively small cohorts have been published. The aim of this review is to evaluate the post-operative mortality and morbidity of pediatric slide tracheoplasty for CTS.
    METHODS: A systematic literature review was performed according to PRISMA guidelines. The Medline and EMBASE databases were screened using a search strategy defined in collaboration with a librarian. We included articles reporting the post-operative mortality rate of slide tracheoplasties for treatment of CTS in children, when at least 10 patients were included.
    RESULTS: A total of 932 articles were reviewed, and 15 studies were eligible with a total of 845 patients. The overall post-operative mortality rate was 9.3 %, and most deaths were airway related. The open revision surgery rate after surgery was 2.8 % and the endoscopic revision rate was 27.6 %.
    CONCLUSIONS: This study highlights key factors to consider before the surgery and helps anticipate post-operative follow-up considerations for children with CTS. Several factors were identified as predictors of mortality including young age, weight at the time of surgery and association with lung hypoplasia or aplasia.
    CONCLUSIONS: Although slide tracheoplasty has gained popularity in recent years due to better outcomes, it remains a major surgery with mortality risk and the need for multidisciplinary management.
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  • 文章类型: Systematic Review
    背景:支架已成为治疗犬气管塌陷的流行方法,但可能会出现并发症并对治疗结果产生负面影响.
    目的:确定犬气管支架置入并发症的总体患病率。
    方法:对2000年至2020年的出版物进行了书目检索。评估了狗气管支架置入后8种最常见并发症的证据质量和患病率(支架骨折,支架迁移,复发崩溃,肉芽肿形成,气管支气管感染,和早期,迟到,和临床相关的晚期咳嗽)。随机效应荟萃分析用于估计合并并发症的患病率。
    结果:15项研究符合纳入标准。咳嗽(早期:99%;95%置信区间[95%CI]:95%-100%,晚:75%;95%CI:63%-85%,和临床相关:52%;95%CI:42%-61%),气管支气管感染(24%;95%CI:14%-35%),气管支架置入后常见肉芽肿(20%;95%CI:11%-30%)。支架骨折(12%;95%CI:5%-20%),复发性塌陷(10%;95%CI:5%-15%),和支架迁移(5%;95%CI:1%-9%)频率较低。支架骨折的估计患病率在研究中发现了显著的异质性,肉芽肿,感染,迟来的咳嗽。
    结论:犬气管支架置入术与咳嗽的高风险以及气管支气管感染和肉芽肿形成的中等风险相关。因为大多数并发症会影响狗的生活质量,必须告知患者气管支架置入术是一种二线手术,不一定能减轻医疗和频繁随访的需要.需要进一步的研究来确定这些并发症的危险因素。
    BACKGROUND: Stenting has become popular to treat tracheal collapse in dogs, but complications might arise and negatively affect treatment outcome.
    OBJECTIVE: Determine the overall prevalence of complications of tracheal stenting in dogs.
    METHODS: A bibliographic search was performed of publications from 2000 to 2020. Studies were assessed for quality of evidence and measured prevalence of the 8 most commonly reported complications after tracheal stenting in dogs (stent fracture, stent migration, relapsing collapse, granuloma formation, tracheobronchial infections, and early, late, and clinically relevant late cough). Random effects meta-analyses were used to estimate pooled complications prevalence.
    RESULTS: Fifteen studies met inclusion criteria. Cough (early: 99%; 95% confidence interval [95% CI]: 95%-100%, late: 75%; 95% CI: 63%-85%, and clinically relevant: 52%; 95% CI: 42%-61%), tracheobronchial infections (24%; 95% CI: 14%-35%), and granulomas (20%; 95% CI: 11%-30%) were common after tracheal stenting. Stent fractures (12%; 95% CI: 5%-20%), relapsing collapse (10%; 95% CI: 5%-15%), and stent migration (5%; 95% CI: 1%-9%) were less frequent. Significant heterogeneity among studies was identified for the estimated prevalence of stent fracture, granulomas, infections, and late cough.
    CONCLUSIONS: Tracheal stenting in dogs is associated with a high risk of coughing and a moderate risk of tracheobronchial infections and granuloma formation. Because most complications will impact a dog\'s quality of life, owners must be informed that tracheal stenting is a second-line procedure that does not necessarily alleviate the need for medical treatment and frequent follow-up visits. Additional studies are warranted to identify the risk factors of these complications.
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  • 文章类型: Case Reports
    纳入研究的2例患者患有混合型和难治性结核后气管支气管狭窄(PTTS),术前经历过球囊扩张和V形支架置入等不成功的介入治疗。安全放置L形硅胶支架后,术后第1个月和第3个月的纤维支气管镜检查显示,两名患者的支气管粘膜炎症均显着减少。此外,上部和下部分支段的开口直径增加,胸部CT扫描显示左肺病变明显吸收。手术后三个月,纤维支气管镜检查证实支架固定稳定,无任何运动。患者的肺功能有了实质性的改善,呼吸困难指数,和血气分析,没有报告的不良并发症。7个月后,1例纤维支气管镜随访显示支架固定效果良好。同时,胸部CT扫描显示有利的再扩张。L形硅胶支架的放置证明可有效防止移位,缓解气道狭窄或阻塞,并确保PTTS治疗的安全性和有效性-特别是在V形硅胶支架置入失败的情况下。据我们所知,这是第一项描述2例PTTS患者使用L型硅胶支架的研究.
    使用特殊的L形硅胶支架成功治疗因肺结核引起的严重气道狭窄本文讲述了两名患有肺结核后气管支气管狭窄(PTS)的复杂肺部疾病的患者的故事。想象一下你的气道-将空气输送到肺部的管道-由于过去的肺结核发作而严重伤痕累累并变窄。这两名患者尝试了以前的治疗方法,如球囊扩张(在狭窄的气道内膨胀一个小球囊以使其变宽)和使用V形支架(放置在气道中以保持其打开的柔性支撑),但是这些方法并不能提供持久的缓解。在这种创新的方法中,医生使用了专门设计用于患者气道受影响部位的L形硅胶支架。放置这些支架后,定期检查显示出显着的改善。气道内膜肿胀明显减轻,通向肺部上部和下部的开口变得更宽。胸部X光片(CT扫描)甚至显示患者的左肺愈合良好。三个月后,支架牢牢地保持在原位,两个病人都没有遇到任何问题。呼吸变得更容易,肺功能检查有所改善,血液测试显示氧气水平更好。七个月后,一名患者继续做得非常好,支架牢固固定,胸部扫描显示肺部扩张良好。这项开创性的研究表明,当其他方法失败时,使用L形硅胶支架可以有效治疗PTTS。他们不仅呆在原地,防止堵塞,但它们也安全有效地缓解了气道狭窄。这是第一次在PTTS患者中成功使用这种L形支架,为面临类似挑战的人们带来新的希望。
    The two patients included in the study had mixed and refractory post-tuberculosis tracheobronchial stenosis (PTTS), having experienced unsuccessful interventional therapies such as balloon dilation and V-shaped stent placement before the operation. Following the secure placement of L-shaped silicone stents, examinations with a fiberbronchoscope during the first and third months post-operation revealed a significant reduction in bronchial mucosa inflammation for both patients. Additionally, the opening diameter of the upper and lower branch segments increased, and chest CT scans indicated a noticeable absorption of left pulmonary lesions. Three months post-operation, fiberbronchoscopy confirmed the stable fixation of the stent without any movement. The patients exhibited substantial improvements in pulmonary function, dyspnea index, and blood gas analysis, with no reported adverse complications. After 7 months, a follow-up fiberbronchoscope for one case revealed excellent stent fixation. Simultaneously, the chest CT scan indicated favorable re-expansion. The placement of L-shaped silicone stents proves effective in preventing displacement, alleviating airway stenosis or obstruction, and ensuring the safety and efficacy of PTTS treatment - particularly in cases where V-shaped silicone stent placement has failed. To our knowledge, this is the first study describing the L-shaped silicone stent in two patients with PTTS.
    Successful treatment of severe airway narrowing due to tuberculosis using special L-shaped silicone stentsThis article tells the story of two patients who suffered from a complex lung condition called post-tuberculosis tracheobronchial stenosis (PTTS). Imagine your airways - the tubes that carry air to your lungs - getting severely scarred and narrowed due to a past bout with tuberculosis. These two patients had tried previous treatments like balloon dilation (where a small balloon is inflated inside the narrowed airway to widen it) and using V-shaped stents (flexible supports placed in the airway to keep it open), but these methods didn’t provide lasting relief. In this innovative approach, doctors used L-shaped silicone stents specifically designed to fit in the affected parts of the patients’ airways. After placing these stents, regular checks showed remarkable improvements. The swelling in the airway lining reduced significantly, and the openings leading to the upper and lower parts of the lungs got wider. Chest X-rays (CT scans) even showed that the patient’s left lung was healing well. Three months later, the stents stayed firmly in place, and neither patient experienced any problems. Breathing became easier, lung function tests improved, and blood tests showed better oxygen levels. Seven months down the line, one patient continued to do extremely well, with the stent securely fixed and the chest scan showing good lung expansion. This groundbreaking study shows that using L-shaped silicone stents can effectively treat PTTS when other methods fail. Not only do they stay in place, preventing blockages, but they also safely and effectively alleviate narrowing of the airways. It’s the first time such L-shaped stents have been used successfully in PTTS patients, offering new hope for those facing similar challenges.
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  • 文章类型: Case Reports
    SARS-CoV-2病毒导致了前所未有的气管狭窄。刚性支气管镜检查可作为气管切除的治疗措施或桥接疗法。我们还简要讨论了长时间插管和SARS-CoV-2病毒引起的气管狭窄的病理生理学。应将其与其他形式的气道阻塞区分开,例如被认为是假性气管狭窄疾病的气管支气管软化症。这项研究的目的是评估气道正压通气或“PAP”疗法以及所需的支架置入和/或随后的气管切除术无法改善的狭窄。通过进行刚性支气管镜检查和随后的气道支架置入术,我们证明了SARS-CoV-2病毒继发插管患者的长期气道通畅结局.我们在连续病例系列中展示了出色的结果,其中6例患者接受了硬质支气管镜检查,气道支架和气管切除术。所有患者均由本研究中提到的医生从肺部角度进行管理。
    SARS-CoV-2 virus has led to an unprecedented amount of tracheal stenosis. Rigid bronchoscopy can serve as a curative measure or bridge therapy to tracheal resection. We also briefly discuss the pathophysiology of tracheal stenosis from prolonged intubation and SARS-CoV-2 virus. This should be differentiated from other forms of airway obstruction such as tracheobronchomalacia which would be considered a pseudo-tracheal stenotic disease. The aim of this study is to evaluate stenosis that is unable to be improved with positive airway pressure or \"PAP\" therapies and required stenting and/or subsequent tracheal resection. By performing Rigid Bronchoscopy and subsequent stenting of airways, we demonstrated outcomes for long term airway patency regarding patients who were intubated secondary to the SARS-CoV-2 virus. We demonstrate superb outcomes in a consecutive case series of 6 patients managed with rigid bronchoscopy, airway stent and tracheal resection. The patients were all managed from a pulmonary perspective by the physicians mentioned in this study.
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  • 文章类型: English Abstract
    BACKGROUND: Suprastomal collapse (SSC) is considered a major late complication of paediatric tracheostomy and can be responsible for decannulation failure in up to 20% of tracheostomised children. Depending on the severity of SSC, surgery may be required. Various strategies and techniques are available, of which the treating with airway team should be aware.
    OBJECTIVE: This article intends to summarise the aetiology of SSC, its classification, clinical presentation, and the gold standard diagnostic and therapeutic algorithms according to the current literature.
    METHODS: A panel of experts reviewed the available literature on SSC. Published evidence on the different surgical techniques and their advantages and disadvantages was reviewed in detail, and a treatment algorithm created.
    RESULTS: The gold standard diagnostic procedure for SSC is flexible transnasal laryngotracheoscopy in spontaneous breathing followed by microlaryngoscopy (MLS) under general anaesthesia. Two main types of SSC can be differentiated, which differ in terms of surgical treatment. Purely anterior SSC is usually treated by tracheoplasty using an anterior costal cartilage graft (ACCG). Simple closure of the tracheostomy or excision of SSC with a potassium-titanyl-phosphate (KTP) laser are also described as less invasive approaches. For anterolateral SSC, segmental tracheal resection with end-to-end anastomosis or tracheoplasty with ACCG represent promising treatment options. Tracheal reinforcement with absorbable microplates is also discussed in the literature. With both types of SSC and depending on severity and the age of the child, a watch-and-wait strategy should always be considered.
    CONCLUSIONS: Dynamic airway endoscopy in spontaneous breathing followed by MLS in general anaesthesia should always be performed before decannulation. It is particularly important to visualise all segments of the airway during spontaneous breathing. The decision regarding the best surgical option for each child is based on the type and localisation of SSC, as well as on the patient\'s medical and surgical history and age.
    UNASSIGNED: HINTERGRUND: Der suprastomale Kollaps (SSC) gilt als eine späte Komplikation der kindlichen Tracheotomie und kann bei bis zu 20 % der tracheotomierten Kinder zu einem Scheitern des Dekanülierungsversuchs führen. Je nach Ausprägung erfordert ein SSC eine chirurgische Therapie. Es stehen einige unterschiedliche Strategien und Verfahren zur Verfügung, die dem behandelnden Team bekannt sein sollten. ZIEL: Ziel der vorliegenden Arbeit war eine Zusammenfassung der Ätiologie des SSC, seiner Klassifikation, klinischen Symptomatik und des Goldstandards in Bezug auf diagnostische und therapeutische Algorithmen gemäß der aktuellen Literatur.
    METHODS: Die verfügbare Literatur zum Thema des SSC wurde von erfahrenen HNO-Ärzten in Bezug auf die chirurgischen Techniken, deren Vor- und Nachteile begutachtet und ein Behandlungspfad erarbeitet.
    UNASSIGNED: Der Goldstandard zur Verifizierung eines SCC ist die flexible, transnasale Laryngotracheoskopie in Spontanatmung mit anschließender Mikrolaryngoskopie (MLS) in Vollnarkose. Die allgemein gültige Klassifizierung des SSC benennt 2 Hauptformen. Diese unterscheiden sich wesentlich in Bezug auf die chirurgischen Therapieoptionen. Bei einem rein anterioren SSC stehen hauptsächlich die laryngotracheale Rekonstruktion (LTR) anterior mit Rippenknorpel zur Verfügung. Die Pexie der Tracheavorderwand durch Nahttechnik und die Exzision des Kollapses mit dem Kaliumtitanylphosphat(KTP)-Laser werden ebenfalls beschrieben. Beim anterolateralen SSC gibt es die Tracheaquerresektion oder die LTR anterior mit Rippenknorpel als erfolgversprechende chirurgische Verfahren. Die Verstärkung der malazischen Trachea mit resorbierbaren Mikroplatten wird ebenfalls in der Literatur diskutiert. Bei beiden Formen des SSC sollte je nach Alter des Kindes und Ausprägung auch immer ein abwartendes Verhalten erwogen werden.
    UNASSIGNED: Vor einem Dekanülierungsversuch sollte immer eine Laryngotracheoskopie in Spontanatmung gefolgt von einer MLS in Vollnarkose erfolgen. Insbesondere auf die Visualisierung sämtlicher Abschnitte des Atemwegs in Spontanatmung ist zu achten. Für die Entscheidungsfindung der besten Therapieoption ist die genaue Diagnosestellung, aber auch das Einbeziehen vorangegangener rekonstruktiver Eingriffe, Begleiterkrankungen sowie des Alters des Kindes entscheidend.
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  • 文章类型: Journal Article
    背景:不适合常规手术的长段气道狭窄患者可能受益于气管移植。然而,这个过程只有轶事报道,和它的迹象,技术,和结果尚未广泛审查。
    方法:我们进行了系统的文献检索,以确定所有报道人类气管移植尝试的原始文章。
    结果:在初始搜索中找到的699篇文章中,系统审查中包括11个,描述14例气管移植。9例患者因良性狭窄行移植,5例恶性肿瘤。在12例中,通过将移植物包裹在血管化的受体组织中来提供气管的血液供应,2例气管直接移植为血管化复合同种异体移植物。两名患者在原位移植前中止了移植程序。在剩下的12名患者中,有1例手术死亡,4例患者出现并发症。术后对大多数患者使用免疫抑制剂药物,只有一组作者试图撤回,5名患者在后续行动结束时,手术中幸存的11名患者都活着,但2例患者有复发性气管狭窄,需要使用气道矫治器进行呼吸。
    结论:人类气管移植仍处于胚胎期。文献中的研究报告了不同的手术技术,关于长期结果的信息仍然有限。需要进一步的研究才能了解该程序的临床价值。
    Patients with long-segment airway stenosis not amenable to conventional surgery may benefit from tracheal transplantation. However, this procedure has been only anecdotally reported, and its indications, techniques, and outcomes have not been extensively reviewed.
    We conducted a systematic Literature search to identify all original articles reporting attempts at tracheal transplantation in humans.
    Of 699 articles found by the initial search, 11 were included in the systematic review, describing 14 cases of tracheal transplantation. Patients underwent transplantation for benign stenosis in nine cases, and for malignancies in five cases. In 12 cases blood supply to the trachea was provided by wrapping the graft in a vascularized recipient\'s tissue, while in 2 cases the trachea was directly transplanted as a vascularized composite allograft. The transplantation procedure was aborted before orthotopic transplantation in two patients. Among the remaining 12 patients, there was 1 operative mortality, while 4 patients experienced complications. Immunosuppressants drugs were administered to the majority of patients postoperatively, and only one group of authors attempted their withdrawal, in five patients. At the end of follow-up, all 11 patients surviving the operation were alive, but 2 had a recurrent tracheal stenosis requiring an airway appliance for breathing.
    Human tracheal transplantation is still at an embryonic phase. Studies available in the Literature report different surgical techniques, and information on long-term outcomes is still limited. Future research is needed in order to understand the clinical value of this procedure.
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  • 文章类型: Review
    背景:淋巴瘤可以出现在身体的各个部位,并表现出不同的症状。然而,支气管淋巴瘤少见,可误诊为气道恶性肿瘤或肺部疾病。患者:一名患有气管淋巴瘤的老年女性经历了严重的呼吸困难,胸部计算机断层扫描显示气道严重狭窄。她对反复的抗生素治疗没有反应,手术切除肿瘤后,根据病理结果,她最终被诊断为淋巴瘤。
    方法:患者因气管内炎性肉芽肿性病变或肿瘤而被诊断为胸段气管狭窄。
    方法:治疗包括使用高频电刀,冻结,和氩等离子体凝固术.
    结果:患者报告呼吸困难有所改善,咳嗽,手术后的其他症状。病理结果证实为滤泡性淋巴瘤。纤维支气管镜复查显示,介入治疗后气管中段和上段狭窄程度明显减轻。
    结论:内镜介入治疗是治疗气管淋巴瘤的有效方法。
    BACKGROUND: Lymphoma can appear in all parts of the body and present with different symptoms. However, bronchial lymphoma is rare and can be misdiagnosed as airway malignancy or lung disease.Patient: An older adult woman with tracheal lymphoma experienced severe breathing difficulties, and chest computed tomography indicated severe narrowing of the airway. She did not respond to repeated antibiotic treatment, and she was eventually diagnosed with lymphoma based on pathology after surgical removal of the tumor.
    METHODS: The patient received a diagnosis of thoracic tracheal stenosis due to intratracheal inflammatory granulomatous lesions or a tumor.
    METHODS: Treatment involved the use of a high-frequency electrotome, freezing, and argon plasma coagulation.
    RESULTS: The patient reported improvements in dyspnea, cough, and other symptoms after the operation. The pathological results confirmed follicular lymphoma. Reexamination by fiberbronchoscopy indicated that the degree of stenosis in the middle and upper tracheal segments was significantly reduced following interventional therapy.
    CONCLUSIONS: Endoscopic interventional therapy can be an effective treatment for tracheal lymphoma.
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  • 文章类型: Systematic Review
    背景:先天性喉气管狭窄(CLS)是新生儿喘鸣的罕见原因。有证据表明,一些家庭成员可能受到CLS的影响。了解家族性先天性喉气管狭窄(FCLS)的病理生理学将使更有效的治疗策略成为可能。
    目的:确定家族性先天性喉气管狭窄(FCLS)的临床病程和预后。
    方法:在一个月(2023年9月)的时间内,通过搜索几个数据库进行了文献检索,以确定从开始到2023年8月31日发表的研究。
    结果:在确定的256篇论文中,五篇文章符合纳入标准。总共确定了17例女性占主导地位的患者(59%)。据报道,女性双胞胎(100%)患有家族性先天性气管狭窄。列出了各种临床表现。对所有患者进行了内镜气道研究。64.8%的纳入儿童接受手术治疗。对41%的儿童进行的遗传研究无法定位遗传异常。
    结论:血缘关系,双胞胎出生,女性可能是FCLS的诱发因素,尽管由于条件的稀有性,证据的质量很低。
    BACKGROUND: Congenital laryngotracheal stenosis (CLS) is a rare cause of stridor among newborns. Evidence has shown that several family members can be affected by CLS. Knowledge of the pathophysiology of familial congenital laryngotracheal stenosis (FCLS) will enable more effective therapeutic strategies.
    OBJECTIVE: To determine the clinical course and outcome of familial congenital laryngotracheal stenosis (FCLS).
    METHODS: A literature search was conducted over a period of one month (September 2023) by searching several databases to identify studies published from inception to 31st August 2023.
    RESULTS: Of 256 papers identified, five articles met the inclusion criteria. A total of 17 patients with slight female predominance (59 %) were identified. Familial congenital tracheal stenosis was reported in female twins (100 %). A variety of clinical presentations were listed. An endoscopic airway study was performed on all patients. 64.8 % of the included children were managed surgically. Genetic studies performed on 41 % of children could not locate genetic abnormalities.
    CONCLUSIONS: Consanguinity, twin births, and female gender could be predisposing factors for FCLS, although the quality of evidence is low due to the rarity of the condition.
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  • 文章类型: Case Reports
    气管狭窄是指气管变窄,可导致呼吸急促,喘鸣甚至窒息。本研究报告了患有这种疾病的患者的临床过程,旨在帮助临床医生获得有关这种罕见疾病的更多信息并确定潜在的治疗方案。一名2岁的女性儿童出现进行性呼吸急促和喘鸣。她最初是通过气管造口术进行管理的;但是,这未能缓解狭窄.随后的干预措施,包括硬支气管镜检查和扩张术成功缓解了病情。通过活检鉴定出支气管壁的良性肥大。病人接受了类固醇和抗生素治疗,经过1年的随访,她的症状明显改善,且无症状.气管狭窄是一种罕见的,但可能危及生命的严重状况。因此,这种情况的早期诊断和治疗对于改善预后至关重要.
    Tracheal stenosis is a narrowing of the windpipe that can lead to shortness of breath, stridor and even suffocation. The present study reports the clinical course of a patient with this condition in an aim to help clinicians obtain more information about this rare condition and identify potential treatment options. A 2-year-old female child presented with progressive shortness of breath and stridor. She was initially managed with tracheostomy; however, this was unsuccessful in relieving the stenosis. Subsequent interventions, including rigid bronchoscopy and dilatation were successful in relieving the condition. A benign hypertrophy of the bronchial wall was identified through biopsy. The patient was treated with steroids and antibiotics, and she experienced a marked improvement in symptoms and remained asymptomatic after a 1-year follow-up. Tracheal stenosis is a rare, yet serious condition that may be life-threatening. Thus, the early diagnosis and treatment of this condition are essential in order to improve outcomes.
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