Hashmi Syed Faisal, Hanif Mohammad, Maroo Sanjay, Pollock J C S
Departments of Pediatric Cardiothoracic Surgery, Royal Hospital for Sick Children, Glasgow, UK.
Congenit Heart Dis. 2009 Nov-Dec;4(6):474-7. doi: 10.1111/j.1747-0803.2009.00286.x.
This case report describes a rare example of double aortic arch with a dominant left aortic arch, patent minor right aortic arch, left descending aorta, and right ligamentum arteriosum causing tracheobronchial compression in a twin baby girl with DiGeorge syndrome. She also had large right subclavian artery arising from right-sided diverticulum of Kommerell, aplastic thymus, T cell lymphopenia with normal immunoglobulin, hypocalcemia, and hypomagnesemia. The diverticulum of Kommerell was resected and minor right aortic arch, right ligamentum arteriosum, and right subclavian artery were divided through right posterolateral thoracotomy. Aortopexy was performed under bronchoscopic guidance to relieve the airway compression. We strongly suggest a right-sided approach in this type of vascular arrangement for easy access and better outcome.
本病例报告描述了一个罕见的双主动脉弓病例，其特征为左主动脉弓占优势、右主动脉弓较小且通畅、左位降主动脉以及右动脉韧带，导致一名患有22q11.2微缺失综合征（DiGeorge综合征）的双胎女婴出现气管支气管受压。她还存在起源于Kommerell憩室右侧的粗大右锁骨下动脉、胸腺发育不全、免疫球蛋白正常的T细胞淋巴细胞减少、低钙血症和低镁血症。通过右后外侧开胸手术切除Kommerell憩室，并切断右主动脉弓、右动脉韧带和右锁骨下动脉。在支气管镜引导下进行主动脉固定术以缓解气道压迫。我们强烈建议对于这种血管排列类型采用右侧入路，以便于操作并获得更好的治疗效果。
