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Abstract：
BACKGROUND: Tracheal compression (TC) due to vascular anomalies is an uncommon, but potentially serious cause of chronic respiratory disease in childhood. Vascular slings are congenital malformations resulting from abnormal development of the great vessels; in this group of disorders the most prevalent entity is the aberrant innominate artery (AIA). Here we provide a report on diagnosis and treatment of AIA in nine children with unexplained chronic respiratory symptoms. We describe the cases, perform a literature review, and provide a discussion on the diagnostic workup and treatment that can help manage AIA.
METHODS: Clinical history, diagnostic procedures and treatment before and after the AIA diagnosis were retrospectively reviewed in nine children (5 boys and 4 girls), who were referred for recurrent-to-chronic respiratory manifestations over 10 years (2012-2022). We performed a comprehensive report on the ongoing clinical course and treatment as well as an electronic literature search on the topic.
RESULTS: Diagnoses at referral, before AIA was identified, were chronic dry barking cough associated with recurrent pneumonia (n = 8, 89%), lobar/segmental atelectasis (n = 3, 33%), atopic/non atopic asthma (n = 3, 33%); pneumomediastinum with subcutaneous emphysema complicated the clinical course in one case. When referred to our Unit, all patients had been previously treated with repeated antibiotic courses (n = 9, 100%), alone (n = 6, 67%) or combined with prolonged antiasthma medications (n = 3, 33%) and/or daily chest physiotherapy (n = 2, 22%), but reported only partial clinical benefit. Median ages at symptom onset and at AIA diagnosis were 1.5 [0.08-13] and 6 [4-14] years, respectively, with a relevant delay in the definitive diagnosis (4.5 years). Tracheal stenosis at computed tomography (CT) was ≥ 51% in 4/9 cases and ≤ 50% in the remaining 5 subjects. Airway endoscopy was performed in 4 cases with CT evidence of tracheal stenosis ≥ 51% and confirmed CT findings. In these 4 cases, the decision of surgery was made based on endoscopy and CT findings combined with persistence of clinical symptoms despite medical treatment. The remaining 5 children were managed conservatively.
CONCLUSIONS: TC caused by AIA may be responsible for unexplained chronic respiratory disease in childhood. Early diagnosis of AIA can decrease the use of expensive investigations or unsuccessful treatments, reduce disease morbidity, and accelerate the path toward a proper treatment.
METHODS: Clinical history, diagnostic procedures and treatment before and after the AIA diagnosis were retrospectively reviewed in nine children (5 boys and 4 girls), who were referred for recurrent-to-chronic respiratory manifestations over 10 years (2012-2022). We performed a comprehensive report on the ongoing clinical course and treatment as well as an electronic literature search on the topic.
RESULTS: Diagnoses at referral, before AIA was identified, were chronic dry barking cough associated with recurrent pneumonia (n = 8, 89%), lobar/segmental atelectasis (n = 3, 33%), atopic/non atopic asthma (n = 3, 33%); pneumomediastinum with subcutaneous emphysema complicated the clinical course in one case. When referred to our Unit, all patients had been previously treated with repeated antibiotic courses (n = 9, 100%), alone (n = 6, 67%) or combined with prolonged antiasthma medications (n = 3, 33%) and/or daily chest physiotherapy (n = 2, 22%), but reported only partial clinical benefit. Median ages at symptom onset and at AIA diagnosis were 1.5 [0.08-13] and 6 [4-14] years, respectively, with a relevant delay in the definitive diagnosis (4.5 years). Tracheal stenosis at computed tomography (CT) was ≥ 51% in 4/9 cases and ≤ 50% in the remaining 5 subjects. Airway endoscopy was performed in 4 cases with CT evidence of tracheal stenosis ≥ 51% and confirmed CT findings. In these 4 cases, the decision of surgery was made based on endoscopy and CT findings combined with persistence of clinical symptoms despite medical treatment. The remaining 5 children were managed conservatively.
CONCLUSIONS: TC caused by AIA may be responsible for unexplained chronic respiratory disease in childhood. Early diagnosis of AIA can decrease the use of expensive investigations or unsuccessful treatments, reduce disease morbidity, and accelerate the path toward a proper treatment.
摘要：
背景：由于血管异常引起的气管压迫（TC）并不常见，而是儿童慢性呼吸道疾病的潜在严重原因。血管吊索是由大血管异常发育引起的先天性畸形；在这组疾病中，最普遍的实体是异常无名动脉（AIA）。在这里，我们提供了9例无法解释的慢性呼吸道症状儿童AIA的诊断和治疗报告。我们描述了这些案例，进行文献综述，并就可以帮助管理AIA的诊断检查和治疗进行讨论。
方法：临床病史，对9名儿童（5名男孩和4名女孩）进行了AIA诊断前后的诊断程序和治疗，因反复至慢性呼吸道表现转诊超过10年(2012-2022年).我们对正在进行的临床过程和治疗进行了全面的报告，并对该主题进行了电子文献检索。
结果：转诊时的诊断，在AIA被确认之前，与复发性肺炎相关的慢性干性吠叫咳嗽（n=8，89%），肺叶/节段性肺不张（n=3，33%），特应性/非特应性哮喘（n=3，33％）；纵隔气肿伴皮下气肿并发临床病程1例。当提到我们的单位时，所有患者以前都接受过反复抗生素疗程的治疗（n=9，100%），单独（n=6，67%）或联合长期抗哮喘药物（n=3，33%）和/或每日胸部理疗（n=2，22%），但报告仅有部分临床获益。症状发作和AIA诊断的中位年龄分别为1.5[0.08-13]和6[4-14]岁，分别,最终诊断有相关延迟（4.5年）。4/9例计算机断层扫描（CT）时气管狭窄≥51％，其余5例≤50％。对4例CT证据为气管狭窄≥51％的患者进行了气道内镜检查，并证实了CT表现。在这4个案例中,手术的决定是根据内窥镜检查和CT检查结果以及尽管接受了药物治疗但仍存在的临床症状。其余5名儿童得到保守管理。
结论：由AIA引起的TC可能是儿童时期无法解释的慢性呼吸道疾病的原因。AIA的早期诊断可以减少昂贵的检查或不成功的治疗的使用。降低疾病发病率，加速走向适当治疗的道路。
方法：临床病史，对9名儿童（5名男孩和4名女孩）进行了AIA诊断前后的诊断程序和治疗，因反复至慢性呼吸道表现转诊超过10年(2012-2022年).我们对正在进行的临床过程和治疗进行了全面的报告，并对该主题进行了电子文献检索。
结果：转诊时的诊断，在AIA被确认之前，与复发性肺炎相关的慢性干性吠叫咳嗽（n=8，89%），肺叶/节段性肺不张（n=3，33%），特应性/非特应性哮喘（n=3，33％）；纵隔气肿伴皮下气肿并发临床病程1例。当提到我们的单位时，所有患者以前都接受过反复抗生素疗程的治疗（n=9，100%），单独（n=6，67%）或联合长期抗哮喘药物（n=3，33%）和/或每日胸部理疗（n=2，22%），但报告仅有部分临床获益。症状发作和AIA诊断的中位年龄分别为1.5[0.08-13]和6[4-14]岁，分别,最终诊断有相关延迟（4.5年）。4/9例计算机断层扫描（CT）时气管狭窄≥51％，其余5例≤50％。对4例CT证据为气管狭窄≥51％的患者进行了气道内镜检查，并证实了CT表现。在这4个案例中,手术的决定是根据内窥镜检查和CT检查结果以及尽管接受了药物治疗但仍存在的临床症状。其余5名儿童得到保守管理。
结论：由AIA引起的TC可能是儿童时期无法解释的慢性呼吸道疾病的原因。AIA的早期诊断可以减少昂贵的检查或不成功的治疗的使用。降低疾病发病率，加速走向适当治疗的道路。
