Cohen S R, Landing B H
Ann Otol Rhinol Laryngol. 1976 Sep-Oct;85(5 Pt.1):582-90. doi: 10.1177/000348947608500504.
Three patients with aberrant left pulmonary artery (sling artery) are reported to illustrate associated tracheobronchial abnormalities. The clinical picture was that of severe episodic or progressive respiratory distress without dysphagia in early infancy. Striking narrowing of the trachea by complete "ring cartilages," unrelated to compression by the abnormal pulmonary artery, was present. Tracheotomy and intubation failed to relieve the obstruction. In one patient the bronchi and bronchial segmentation pattern were normal, but in the other two patients, bronchial abnormalities included wide irregular cartilages in the main bronchi, forming more complete rings than is normal. In both, the right main bronchus was relatively longer than normal, and the bronchus intermedius showed poor cartilage ring formation and was relatively short compared to the main bronchus. This discrepancy did not appear to be due to distal displacement of the right upper lobe bronchus. In these two patients the left main bronchus was relatively short and wide with reduced number of cartilage rings (five-six vs usual normal number of nine), so that the right and left main bronchi were almost of equal length. However, the branch patterns of the lobar bronchi were within normal range. Bronchoscopy seems essential to demonstrate such ring tracheal cartilages (absence of the pars membranacea of the trachea), which when found should alert the examiner to the possible presence of an abnormal left pulmonary artery. Since surgical correction of tracheal stenosis of this type is not possible at present, the ultimate prognosis of patients with sling artery may depend more on the severity of the tracheal anomaly rather than on success of surgical correction of the abnormal left pulmonary arterial course. Although aberrant (sling) left pulmonary artery can occur in patients without respiratory tract symptoms, tracheal stenosis due to ring tracheal cartilages occurs in a significant fraction of patients with this arterial anomaly. Bronchoscopic study of the trachea, and possibly air or contrast bronchography of right and left main bronchi, can be of aid in diagnosis of this complex.
本文报告了3例左肺动脉异常（吊带动脉）患者，以说明相关的气管支气管异常情况。临床表现为婴儿早期出现严重的发作性或进行性呼吸窘迫，无吞咽困难。气管出现由完整“环状软骨”导致的明显狭窄，与异常肺动脉的压迫无关。气管切开术和插管未能缓解梗阻。1例患者的支气管及支气管分段模式正常，但另外2例患者的支气管异常包括主支气管内宽而不规则的软骨，形成的环比正常情况更完整。在这2例患者中，右主支气管相对比正常长，中间支气管软骨环形成不佳，与主支气管相比相对较短。这种差异似乎并非由于右上叶支气管的远端移位所致。在这2例患者中，左主支气管相对短而宽，软骨环数量减少（5 - 6个，而正常通常为9个），因此左右主支气管几乎等长。然而，肺叶支气管的分支模式在正常范围内。支气管镜检查似乎对于显示这种环状气管软骨（气管膜部缺失）至关重要，一旦发现应提醒检查者可能存在异常左肺动脉。由于目前无法对这种类型的气管狭窄进行手术矫正，吊带动脉患者的最终预后可能更多地取决于气管异常的严重程度，而非异常左肺动脉走行的手术矫正是否成功。尽管异常（吊带）左肺动脉可发生于无呼吸道症状的患者，但因环状气管软骨导致的气管狭窄在相当一部分有这种动脉异常的患者中出现。气管的支气管镜检查以及左右主支气管的空气或造影支气管造影可能有助于诊断这一复杂情况。
