Department of Surgery, University of California San Francisco, 400 Parnassus Avenue, San Francisco, CA, USA.
Division of Pediatric Surgery, Department of Surgery, American Family Children's Hospital, University of Wisconsin School of Medicine and Public Health, 600 Highland Avenue, MC 7375, Madison, WI, 53792, USA.
J Cardiothorac Surg. 2021 May 25;16(1):143. doi: 10.1186/s13019-021-01520-z.
Congenital single lung (CSL) is a rare condition, and symptomatic patients often present with respiratory distress or recurrent respiratory infection due to mediastinal shift causing vascular or airway compression. Aberrant right subclavian artery (ARSA) is another rare congenital anomality that can lead to tracheal or esophageal compressions. There is only one other case of concurrent presentation of CSL and ARSA reported, which presented unique challenge in surgical management of our patient. Here we present a step-wise, multidisciplinary approach to manage symptomatic CSL and ARSA.
An infant girl with a prenatal diagnosis of CSL developed worsening stridor and several episodes of respiratory illnesses at 11 months old. Cross-sectional imaging and bronchoscopic evaluation showed moderate to severe distal tracheomalacia with anterior and posterior tracheal compression resulting from severe mediastinal rotation secondary to right-sided CSL. It was determined that her tracheal compression was mainly caused by her aortic arch wrapping around the trachea, with possible additional posterior compression of the esophagus by the ARSA. She first underwent intrathoracic tissue expander placement, which resulted in immediate improvement of tracheal compression. Two days later, she developed symptoms of dysphagia lusoria due to increased posterior compression of her esophagus by the ARSA. She underwent transposition of ARSA to the right common carotid with immediate resolution of dysphagia lusoria. As the patient grew, additional saline was added to the tissue expander due to recurrence in compressive symptoms.
Concurrent presentation of CSL and ARSA is extremely rare. Asymptomatic CSL and ARSA do not require surgical interventions. However, if symptomatic, it is crucial to involve a multidisciplinary team for surgical planning and to take a step-wise approach as we were able to recognize and address both tracheomalacia and dysphagia lusoria in our patient promptly.
先天性单肺（CSL）是一种罕见的情况，由于纵隔移位导致血管或气道受压，有症状的患者常表现为呼吸窘迫或反复呼吸道感染。右位锁骨下动脉异常（ARSA）是另一种罕见的先天性异常，可导致气管或食管受压。仅报告过一例同时存在 CSL 和 ARSA 的病例，这在我们患者的手术管理中带来了独特的挑战。在此，我们介绍了一种逐步的、多学科的方法来管理有症状的 CSL 和 ARSA。
一名患有产前 CSL 诊断的女婴，在 11 个月大时出现进行性喘鸣和数次呼吸道感染发作。横断面成像和支气管镜评估显示，中度至重度远端气管软化，由于右侧 CSL 导致严重纵隔旋转，前后气管受压。确定她的气管压迫主要是由主动脉弓围绕气管引起的，ARSA 可能对食管造成额外的后向压迫。她首先接受了胸腔内组织扩张器放置术，这立即改善了气管压迫。两天后，由于 ARSA 对食管的后向压迫增加，她出现了吞咽困难性呃逆症状。她接受了 ARSA 向右侧颈总动脉的转位，吞咽困难性呃逆立即得到缓解。随着患者的成长，由于压迫症状再次出现，在组织扩张器中添加了额外的生理盐水。
CSL 和 ARSA 同时出现极为罕见。无症状的 CSL 和 ARSA 不需要手术干预。然而，如果出现症状，就需要多学科团队参与手术规划，并采取逐步的方法，正如我们能够及时识别和处理我们患者的气管软化和吞咽困难性呃逆。
