KD was firstly reported by Kommerell et al. in 1936 [3]. KD with RAA and ALSCA is a rare congenital aortic anomaly, with an incidence of 0.05–0.1% in the general population [1]. KD often presents with respiratory symptoms due to airway stenosis and/or dysphagia due to airway and/or esophageal compression. Emergency surgery has been reported due to rupture of an enlarged KD [4] even in asymptomatic patients. The mortality rate is 80–100% once KD is ruptured [4, 5]. Therefore, early diagnosis and surgical treatment are potentially life-saving [6].

In the current case, a KD of < 20 mm diameter caused symptoms associated with esophageal compression. In contrast, the anteroposterior diameter of the thorax (the distance from the posterior sternum to the anterior thoracic spine) at the level of the KD was < 50 mm. This relationship between the diameter of the KD and the thorax may have caused dysphasia in this case.

The indication for surgery of KD should be determined by its size and the symptoms caused by compression of the surrounding organs. Yu et al. [7] reported that surgical indications of KD in childhood is a KD diameter that is 1.5 times larger than that of the ALSCA origin, or apparent compression of the trachea caused by KD. However, there are many options depending on the case, or that there is no agreement on a standard procedure. Baker et al. [8] reported satisfactory results after performing diverticulectomy and extra-anatomical bypass from the common carotid artery to the subclavian artery bypass in 20 patients aged 9.1 ± 6.5 years old (1.5–29.1 years) with symptomatic KD. However, they reported that after each procedure, the residual diverticulum was problematic in the long-term. In addition, ligation or division of the ALSCA could cause ischemia and subclavian steal syndrome [9]. Therefore, as a curative treatment, we recommend to perform a complete resection of the diverticulum and graft replacement of the descending aorta with reconstruction of the LSA [10].

Recently, less invasive endovascular treatment of KD aneurysms has been performed in adults with low mortality and morbidity [11]. However, there are several problems with endovascular treatment during the growth period. First, the long-term durability of the endovascular approach is questionable because of the potential growth of a child’s body and vessels. The durability of the stent graft itself is also a matter of discussion because of possible material fatigue and late complications. Considering both symptomatic improvements and physical growth, we selected a more invasive procedure in the present case.

The selection of the graft size is controversial. There are few papers [12] describing the risk of aortic stenosis in adulthood due to size mismatch of the graft used in childhood. Okawa et al. [13] reported a case of acute Stanford type A aortic dissection in a 12-year-old boy who had undergone hemiarch replacement using a 22-mm graft. His descending aorta had quite a small diameter, but they used as large a graft as possible, taking his future growth into consideration.

For the diameter of the thoracic aorta, Hegde et al. [14] reported the normal ranges of effective aortic diameter in children. They reported, the effective diameter of the descending aorta was around 17 mm when the BSA was 1.4 m2. Even if the patient’s BSA were 2.0 m2, the diameter of the descending aorta would still be around 20 mm; a 20-mm graft would not cause stenosis. Therefore, in the present case, it was considered that the size of the graft selected was sufficient and allowed for the patient’s physical growth. In addition, the graft was bent with a slight curve to accommodate the aortic growth.