支气管闭锁模仿斯威-詹姆斯综合征

IF 0.5 Q4 RESPIRATORY SYSTEM Pneumon Pub Date : 2023-12-05 DOI:10.18332/pne/175441
Sophia Papathanasiou, Garyfallia Perlepe, E. Karetsi, Euaggelia Sdroulia, I. Gerogianni, Dimitrios Papadopoulos, Konstantinos I. Gourgoulianis
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引用次数: 0

摘要

). CT与支气管镜检查结果的相关性确定了先天性支气管闭锁的诊断。支气管闭锁是一种罕见的先天性异常,支气管缺乏中央气道通讯。假设发生于妊娠16周后由于宫内缺血引起的局灶性支气管中断2。胸部x线表现典型为透光度高、肺门肿块或两者兼而有之。CT是其诊断的金标准,主要表现为支气管膨大、远端肺高充血、低血流量3,4。支气管的主要特征是闭锁段的黏液阻塞。恶性充气可以通过Kohn孔、Lambert支气管肺泡通道或通过止回阀机制引起的空气捕获来解释5。尽管这些几乎是典型的表现,但有必要将单侧透光症与Swyer-James综合征区分。它描述的是一种获得性疾病,通常发生在儿童时期,是传染性闭塞性支气管炎的后遗症,导致肺实质扩张和破坏,伴有空气潴留1和周围肺血管发育不全。CT发现正常的远端支气管分支模式,没有远端和中央气道之间的连接,是鉴别支气管闭锁和Swyer-James综合征的关键1。其他应纳入鉴别诊断的先天性情况包括先天性叶性肺气肿、肺隔离和先天性囊性腺瘤样畸形,因为它们可能有共同的致病途径。在成年期,手术治疗适用于反复感染的患者。
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Bronchial atresia mimicking Swyer–James syndrome
). The correlation of CT and bronchoscopic findings established the diagnosis of congenital bronchial atresia. Bronchial atresia is a rare congenital abnormality in which the bronchus lacks central airway communication 1 . It is hypothesized to occur as focal bronchial interruption due to intrauterine ischemia after the 16th week of gestation 2 . Chest x-ray findings typically show hyperlucency, hilar mass, or both. CT is the gold standard for its diagnosis and the main findings are bronchocele, hyperinflation of the distal lung, and hypovascularity 3,4 . The main characteristic of bronchoceles is the mucoid impaction of atretic segment. Hyperinflation can be explained by collateral ventilation through the pores of Kohn, bronchoalveolar channels of Lambert, or via air-trapping due to check valve mechanism 5 . Despite these almost pathognomonic findings, it is necessary to differentiate unilateral hyperlucency from Swyer-James syndrome. It describes an acquired condition that usually develops during childhood as a sequel of post-infectious bronchitis obliterans, leading to the dilation and destruction of lung parenchyma with air trapping 1 and underdevelopment of the peripheral pulmonary vessels. The CT finding of a normal distal bronchial branch pattern without a connection between the distal and central airways is essential to differentiate bronchial atresia from Swyer-James syndrome 1 . Other congenital situations that should be included in differential diagnosis are congenital lobar emphysema, pulmonary sequestration and congenital cystic adenomatoid malformation, as there are possible common pathogenic pathways. During adulthood, the surgical treatment is meant for patients who present repeated infections 6 .
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来源期刊
Pneumon
Pneumon RESPIRATORY SYSTEM-
CiteScore
0.60
自引率
28.60%
发文量
25
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