Tracheobronchomalacia

气管支气管软化症
  • 文章类型: Case Reports
    气管软化是一种情况,其中气管壁异常柔软,并且在呼吸努力增加时容易塌陷。气道软化症可以表现为像喉软化症这样的节段性疾病,气管软化和支气管软化,或作为弥漫性条件,如气管支气管软化(TBM)。与长段先天性气管狭窄不同,手术可能是首选治疗方法,长段TBM的管理仍然存在争议。
    Tracheomalacia is a condition where the tracheal wall is abnormally soft and prone to collapse during increased respiratory efforts. Airway malacia can manifest as segmental conditions like laryngomalacia, tracheomalacia and bronchomalacia, or as diffuse conditions such as tracheobronchomalacia (TBM). Unlike long-segment congenital tracheal stenosis, where surgery may be the preferred treatment, the management of long-segment TBM remains controversial.
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  • 文章类型: Case Reports
    气管支气管软化症,气道的结构弱化导致通气力学改变。此病例报告描述了一名患有已知气管支气管软化症的患者,该患者在经口内窥镜肌切开术(POEM)治疗有症状的门失弛缓症期间经历了呼气性中央气道塌陷和动态肺过度充气。我们讨论了合并气管支气管软化的POEM手术的生理考虑因素和潜在并发症,并提出了预防和处理这种潜在有害组合的围手术期策略。虽然气管支气管软化症是我们病人的一种已知疾病,这可能是一种未诊断的疾病,可能首先在术中出现。
    In tracheobronchomalacia, the structural weakening of the airway results in altered ventilatory mechanics. This case report describes a patient with known tracheobronchomalacia who experienced expiratory central airway collapse and dynamic pulmonary hyperinflation during peroral endoscopic myotomy (POEM) to treat symptomatic achalasia. We discuss the physiological considerations and potential complications of a POEM procedure with superimposed tracheobronchomalacia and present perioperative strategies for the prevention and management of this potentially deleterious combination. Although tracheobronchomalacia was a known condition in our patient, it is likely an underdiagnosed condition that may first present intraoperatively.
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  • 文章类型: Journal Article
    简介:下气道软化症(LAM)的特征是安静呼吸时横截面管腔面积减少。没有黄金标准的诊断测试;然而,柔性纤维支气管镜(FFB)是最常用的。LAM的确切患病率和发病率未知。这项研究旨在确定诊断为LAM的儿科患者的患病率,详细了解他们的人口统计学和临床特征,并研究两种特定类型的LAM之间的区别,即,气管软化(TM)和支气管软化(BM)。材料和方法:使用FFB诊断为LAM的18岁以下患者纳入本回顾性病例系列。比较了孤立的BM患者和孤立的TM或气管支气管软化症(TM/TBM)患者的人口统计学和临床特征以及合并症。结果:在390例接受FFB的患者中,65人(16.6%)被诊断为LAM,16(24.6%)与TM,和56(86.2%)与BM。诊断时的中位年龄为15个月。其中,59(90.8%)有其他合并症;胃肠道(GI)疾病是最常见的(38.5%)。支气管镜检查最常见的适应症是下呼吸道感染(LRTI)或喘息(43.1%),而最常见的呼吸道体检发现是喘鸣(35.4%)。TM/TBM患者的早产频率明显较高,stridor,撤回,和胃肠道疾病。结论:没有典型喉软化特征或复发或长期LRTI的喘鸣患者应及时进行LAM评估。还应考虑胃肠道疾病如胃食管反流病和吞咽功能障碍的潜在共存。
    Introduction: Lower airway malacia (LAM) is characterized by a reduction in the cross-sectional luminal area during quiet respiration. There is no gold standard diagnostic test; however, flexible fiberoptic bronchoscopy (FFB) is most frequently utilized. The exact prevalence and incidence of LAM are unknown. This study aimed to determine the prevalence rates of pediatric patients diagnosed with LAM, offer a detailed understanding of their demographic and clinical characteristics, and investigate distinctions between two specific types of LAM, namely, tracheomalacia (TM) and bronchomalacia (BM). Materials and Methods: Patients younger than 18 years diagnosed with LAM using FFB were included in this retrospective case series. Demographic and clinical characteristics and comorbid disorders were compared between patients with isolated BM and those with isolated TM or tracheobronchomalacia (TM/TBM). Results: Among 390 patients who underwent FFB, 65 (16.6%) were diagnosed with LAM, 16 (24.6%) with TM, and 56 (86.2%) with BM. The median age at diagnosis was 15 months. Among them, 59 (90.8%) had other comorbidities; gastrointestinal (GI) disorders were the most common (38.5%). The most common indications for bronchoscopy were recurrent/prolonged lower respiratory tract infections (LRTI) or wheezing (43.1%), while the most frequently observed respiratory physical examination finding was stridor (35.4%). Patients with TM/TBM had significantly higher frequencies of premature births, stridor, retraction, and GI disorders. Conclusion: Patients with stridor without typical laryngomalacia features or recurrent or prolonged LRTI should undergo prompt evaluation for LAM. The potential coexistence of GI disorders such as gastroesophageal reflux disease and swallowing dysfunction should also be considered.
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  • 文章类型: Case Reports
    我们报告了一例53岁的女性,该女性在进行无并发症的机器人子宫切除术和双侧附件卵巢切除术以治疗绝经后出血后立即出现气管支气管软化症。中度困难插管时,麻醉诱导明显,通过GlideScope施加环甲压力和小的6.5气管内导管放置来管理。手术过程顺利。患者仍在麻醉后护理单元中插管,但未提供潮气量。尝试用较大的导管替换气管导管失败,患者暂时无法通气。快速故障排除发现喉罩气道(LMA)可以为患者充分通气。一位耳鼻喉科医生能够进行直接支气管镜检查,显示气管和支气管的动态前后塌陷超过50%。病人随后被麻醉苏醒,并在重症监护室接受监测,用LMA通风。几个小时后,确定患者的气道受到保护,LMA被移除.
    We report the case of a 53-year-old female who developed tracheobronchomalacia immediately following an uncomplicated robotic hysterectomy with bilateral salpingo-oophorectomy to treat postmenopausal bleeding. Induction of anesthesia was notable for moderately difficult intubation, managed with applied cricothyroid pressure and a small 6.5 endotracheal tube placement via GlideScope. The surgical course was uneventful. The patient remained intubated in the post-anesthesia care unit but was not providing end-tidal volumes. Attempts to replace the endotracheal tube with a larger tube were unsuccessful and the patient was temporarily unable to ventilate. Rapid troubleshooting discovered that a laryngeal mask airway (LMA) could sufficiently ventilate the patient. An otolaryngologist was able to perform direct bronchoscopy, which revealed more than 50% dynamic anterior-posterior collapse of the trachea and bronchi. The patient was subsequently awakened from anesthesia and monitored in the intensive care unit, ventilating with an LMA. After a couple of hours, it was determined that the patient\'s airway was protected, and the LMA was removed.
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  • 文章类型: Case Reports
    复发性多软骨炎(RP)的气道受累通常会使人衰弱并危及生命。诸如支架置入的介入程序可用于改善气道狭窄。该病例被诊断为RP,左主支气管周围阻塞。我们确定将放置硅胶气道支架。由于肉芽组织,22个月后取出硅胶支架。支架移除后,支气管镜检查结果显示呼气时左主支气管塌陷,但吸入过程中保持气道通畅,无任何呼吸道症状.在这种情况下,去除支架后仍保留支气管软化。然而,因为没有严重的呼吸道症状,我们认为没有必要更换支架.总的来说,在严重的气管支气管软化症中,很难移除气道支架;然而,在单侧主支气管狭窄的情况下,临时支架可能是一种有用的方法。
    Airway involvement in relapsing polychondritis (RP) can often be debilitating and life threatening. Interventional procedures such as stenting can be useful to improve airway stenosis. This case was diagnosed with RP with a circumferential obstruction at the left main bronchus. We determined that a silicone airway stent would be placed. The silicone stent was removed after 22 months due to granulation tissue. After stent removal, bronchoscopic findings revealed a collapsing left main bronchus during exhalation, but airway patency was maintained during inhalation without any respiratory symptoms. In this case, bronchomalacia remained after stent removal. However, since there were no severe respiratory symptoms, we decided that stent replacement was unnecessary. In general, it is difficult to remove airway stents in severe tracheobronchomalacia; however, temporary stenting might be a useful procedure in cases with unilateral main bronchial stenosis.
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  • 文章类型: Letter
    我们报告了一系列四名患有严重支气管肺发育不良(BPD)的患者,他们因严重气管软化(TM)而接受了后气管固定术。虽然后气管固定术是与气管食管瘘相关的TM的既定手术治疗方法,以前在与BPD相关的TM中没有描述过。术中或术后均无明显并发症。四名患者中有三名需要气管切开术和机械通气,这可能反映了这些患者的肺部疾病和其他多系统合并症的程度。需要更多的研究来确定后气管固定术是否是与BPD相关的TM的有效手术选择。
    We report a series of four patients with severe bronchopulmonary dysplasia (BPD) who underwent posterior tracheopexy for severe tracheomalacia (TM). While posterior tracheopexy is an established surgical treatment for TM associated with tracheoesophageal fistula, it has not been previously described in TM associated with BPD. There were no significant intraoperative or postoperative complications from the surgeries. Three of the four patients required tracheostomy and mechanical ventilation, which may reflect the degree of lung disease and other multisystem comorbidities in these patients. More investigation is needed to determine whether posterior tracheopexy is an effective surgical option for TM related to BPD.
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  • 文章类型: Case Reports
    Relapsing polychondritis is an immune disorder of unknown etiology involving multiple systems that is characterized by persistent inflammation and destruction of cartilage, including the ears, nose, costal, joint, and airways. Airway involvement caused by relapsing polychondritis is common, and tracheobronchomalacia is the most serious complication, which is life-threatening. Currently, the exact mechanism of relapsing polychondritis with tracheobronchomalacia is unknown. Although glucocorticoids and immunosuppressive agents are administered, failures often occur. Currently, bronchoscopy-guided intervention therapy used in tracheobronchomalacia caused by chronic obstructive pulmonary disease or other etiology has gradually increased, but bronchoscopy-guided intervention therapy with extracorporeal membrane oxygenation assist used in tracheobronchomalacia caused by relapsing polychondritis has not been reported. Here, we report a case of relapsing polychondritis with severe tracheobronchomalacia. Although drug therapy was provided and airway stent implantation was performed, the tracheal stenosis was further aggravated. Because conventional anesthesia and mechanical ventilation cannot meet the needs of bronchoscopy-guided intervention therapy or guarantee sufficient safety. The intervention treatment was performed with the support of extracorporeal membrane oxygenation, which was successfully completed without obvious complications. The symptoms were significantly improved, and the patient was discharged uneventfully.
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  • 文章类型: Case Reports
    恶性淋巴瘤有时表现为败血症样休克症状。我们报告了一例外周T细胞淋巴瘤,在没有明显淋巴结肿大的情况下,出现无法解释的复发性休克。病人也有不同的症状,包括严重的胸部和背部疼痛,气管支气管软化引起的呼吸窘迫,皮疹,和发烧。
    Malignant lymphoma sometimes manifests with septic-like shock symptoms. We report a case of peripheral T-cell lymphoma presenting with unexplained recurrent shock in absence of apparent lymphadenopathy. The patient also experienced varied symptoms, including severe chest and back pain, respiratory distress due to tracheobronchomalacia, skin rash, and fever.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    三体12是一种罕见的常染色体非整倍体。所有出生后诊断为12三体的个体都已针对这种染色体异常进行了镶嵌。我们在此报告了一名2周龄时患有严重先天性心脏病的女婴,气管支气管软化症,和畸形特征。该患者的所有畸形特征均符合已知的马赛克三体12表型谱,尽管该患者独特地表现为大头畸形。气管支气管软化症以前曾被描述过,但对该患者的临床过程有重大影响。由于心脏和呼吸系统并发症,患者在2个月大时去世。对患者外周血样品的染色体单核苷酸多态性(SNP)微阵列分析显示,大约50%的细胞中有12三体。未培养的血细胞的同时荧光原位杂交分析检测到相当水平的12三体镶嵌。与传统的细胞遗传学相比,SNP微阵列检查所有有核细胞,没有采样偏差,增加了估计镶嵌水平的能力,并可以提供对潜在机制的快速评估。在这里,我们证明了SNP微阵列在那些曾经被认为罕见疾病的临床诊断中的应用,但可能被传统的细胞遗传学技术所遗漏。
    Trisomy 12 is a rare autosomal aneuploidy. All postnatally diagnosed individuals with trisomy 12 have been mosaic for this chromosome abnormality. We herein report an infant girl presented at 2 weeks of age with severe congenital heart defect, tracheobronchomalacia, and dysmorphic features. All of the dysmorphic features of this patient fit into the known phenotype spectrum of mosaic trisomy 12, although this patient uniquely presented with macrocephaly. Tracheo-bronchomalacia has been described once previously but had a significant impact on this patient\'s clinical course. The patient passed away at 2-month-old due to cardiac and respiratory complications. Chromosomal single nucleotide polymorphism (SNP) microarray analysis on a peripheral blood sample from the patient revealed trisomy 12 in approximately 50% of cells. Concurrent fluorescence in situ hybridization analysis of uncultured blood cells detected a comparable level of trisomy 12 mosaicism. Compared to conventional cytogenetics, SNP microarray examines all nucleated cells without sampling bias, has an increased power to estimate mosaicism level, and can provide a quick assessment of the underlying mechanism. Here we demonstrate the utilization of SNP microarray in the clinical diagnosis of those once considered rare disorders but might have been missed by conventional cytogenetic techniques.
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