Kommerell 中文读作“科默雷尔”或“科莫瑞尔”,Kommerell憩室主要表现为胸主动脉瘤形成,而异常锁骨下动脉起始部的扩张,为该病的特征性表现。Kommerell憩室是胚胎后期由左侧第四动脉弓残余组织所形成的一种血管畸形。
Kommerell's diverticulum (KD) is a rare condition characterized by both an aneurysm of the thoracic aorta and an aneurysmal orifice of an aberrant subclavian artery. A possible etiology of the subclavian artery’s anomalous origin would be an abnormality in regression of the fourth primitive aortic arch during embryonic development.
【文献阅读】
右位主动脉伴迷走左锁骨下动脉以及右侧椎动脉双支起源的16岁女患者一例
Kommerell Diverticulum: Right Aortic Arch with Anomalous Origin of Left Subclavian Artery and Duplicity of Right Vertebral Artery in a 16-Year-Old Girl.
患儿,女,16岁,主诉焦虑症后偶尔出现心动过速和轻度非特异性呼吸困难。体格检查显示左上肢的脉搏幅度低于右上肢,血压值存在差异:右上肢血压100×60 mmHg,左上肢血压80×60 mmHg。
A 16-year-old female patient presenting with complaints of occasional tachycardia and mild non-specific dyspnea after anxiety crises. Physical examination revealed lower amplitude of the pulses in the left upper limb compared to the right upper limb, and a difference in blood pressure (BP) values: right upper limb BP 100×60 mmHg and left upper limb BP 80×60 mmHg.
心电图正常,24小时动态心电图无心律失常。
The electrocardiogram was within normal range, and 24-h Holter monitoring showed no arrhythmia.
经胸彩色多普勒超声心动图(ECHO)显示右主动脉弓(图1)出现血管结构,右主动脉下区出现三相脉冲血流,提示锁骨下动脉异常。
The transthoracic color Doppler echocardiography (Echo) revealed a right aortic arch (Figure 1) with emergence of a vascular structure, and a three-phase pulsed pattern flow in the infra-aortic region on the right, suggestive of an aberrant subclavian artery.
图1 彩色多普勒超声心动图显示右主动脉弓
Figure 1 Color Doppler echocardiography showing right aortic arch
在静脉注射碘化非离子造影剂,采用标准成像方案,获得了胸主动脉和主动脉主干的16层CT血管造影(MDCTA)。随后采用多平面重建(MPR)和体积绘制技术(VRT)(图2之2-4)进行多平面和三维重建,右主动脉弓显示清楚,右侧边界出现轻微压迹,气管出现轻微的向左移位。16层CT血管造影显示出左侧锁骨下动脉的异常起源,食管后病变和出现的Kommell憩室扩张以及右侧椎动脉的双支起源。
A 16-multislice detector computed tomography angiography (MDCTA) of the thoracic aorta and supra-aortic trunks was obtained using standard protocols with 1-mm collimation during the venous administration of iodinated nonionic contrast. This was followed by multiplanar and three-dimensional reconstructions using multiplanar reconstruction (MPR) and volume-rendering techniques (VRT) (Figures 22–4), which demonstrated the presence of RAA, causing a mild impression on the right lateral border and a mild leftward displacement of the trachea. MDCTA also showed the aberrant origin of the LSCA, with a retroesophageal course and dilation of its emergence – Kommerell diverticulum – as well as duplicity of RVA.
图2
X线胸片显示肺动脉主干的正常位置(黄色箭头)和右上纵隔的扩大,这是由右侧主动脉弓和上腔静脉(蓝色箭头)协同作用造成的。
Figure 2.
The frontal digital topogram of the MDCT shows the normal position of the pulmonary artery trunk (yellow arrow) and enlargement of the right upper mediastinum, caused by the sum of the RAA and superior vena cava (blue arrows).
图3上纵隔的轴向16层CT血管造影图像
A中,同侧颈总动脉(空心的黑色箭头)、右颈总动脉(空心的蓝色箭头)、右侧锁骨下动脉(空心的绿色箭头)和左侧锁骨下动脉(空心的黄色箭头)中观察到左侧椎动脉(空心的红色箭头)的起源。在B中,图像显示右侧主动脉弓(实心蓝色箭头),在C中,Kommerell憩室(实心红色箭头)位于左侧锁骨下动脉起点的水平。
Figure 3.
Axial MDCT images of the upper mediastinum show in A, the origin of the LVA (open red arrow) is observed in the ipsilateral common carotid (open black arrow), the position of the right common carotid artery (open blue arrow), the RSCA (open green arrow), and the LSCA (open yellow arrow). In B, images show the RAA (solid blue arrows), and in C, Kommerell diverticulum (solid red arrow) at the level of the origin of the LSCA.
图4
使用VRT(体积绘制技术)对胸主动脉进行三维重建的多排CT图像——A和C冠状视图、B左矢状视图和D后视图显示心脏、胸主动脉和主动脉上干的起源。A和B也显示胸骨。图像显示右主动脉弓(A、C和D中的蓝色实心箭头)、主动脉上干起源的解剖结构(白色开放箭头-A-D中的左颈总动脉、蓝色开放箭头-A、C、D中的右颈总动脉、红色开放箭头-右侧锁骨下动脉和黄色开放箭头-A-D中的左侧锁骨下动脉)和在A-D中左侧锁骨下动脉起源的水平的Kommell憩室(红色实心箭头)。同侧颈总动脉中左侧椎动脉(黄色实心箭头)的起源如A-D所示。
Figure 4.
(A–D) MDCT images with three-dimensional reconstruction of the thoracic aorta using VRT (Volume Rendering Technique) – A and C coronal views, B left sagital view and D posterior view show the heart, thoracic aorta and the origin of the supraaortic trunks. A and B also show the thoracic bones. The images show RAA (blue solid arrows in A, C and D), anatomical condition involving the origin of the supra-aortic trunks (white open arrow – left common carotid artery in A–D, blue open arrow - right common carotid artery in A, C, D, red open arrow – RSCA and yellow open arrow – LSCA in A–D) and Kommerell’s diverticulum (red solid arrow) at the level of the origin of the LSCA in A–D. Origin of the LVA (yellow solid arrow) in the ipsilateral common carotid artery is shown in A–D.
患者接受了上消化内镜检查,显示食道中三分之一处有轻微的外部压迫,并对食道进行了放射学对比造影检查(钡剂食道造影),显示右主动脉弓对食道的间断性压迹(图5)。
The patient underwent upper digestive endoscopy, which showed a slight extrinsic compression in the middle third of the esophagus, and a contrast radiographic study of the esophagus (barium esophagram) that showed a discrete esophageal impression by the RAA (Figure 5).

图5.食管钡造影
图像显示右侧主动脉弓(蓝色箭头)和在吞咽不同时刻钡造影剂时在食道造成的压迹(绿色箭头)。A和B正面视图和C-E右侧视图。
Figure 5. Barium esophagram. The images show RAA (blue arrows) and the impression caused in the esophagus (green arrows) during swallowing of the barium contrast at different moments. A and B frontal views, and C–E right lateral views.
有症状的患者出现直径大于5 cm的Kommell憩室是关键的手术指证;在这种情况下,可能会出现气管软化、食管扩张或动脉瘤破裂等并发症。由于该患者的憩室直径为15×13毫米,认为较小,治疗建议是定期进行临床随访。
The key surgical indication occurs when the Kommerell diver-ticulum presents with a diameter greater than 5 cm in symptomatic patients; in such a situation, complications such as tracheomalacia, esophageal dilation, or rupture of the aneurysm may occur. As the diverticulum of our patient was 15×13 mm in diameter, it was considered small, and the medical decision was to maintain periodic clinical follow-up.
经胸超声、胸主动脉多排CT及食管造影检查发现异常。这些检查对于全面了解血管解剖及其与邻近结构的关系以及验证伴随的心脏结构改变很重要。
Abnormal findings were observed by transthoracic Echo, MDCT of the thoracic aorta, and contrasted radiographic esophageal study of the patient. These tests were important for a comprehensive understanding of the vascular anatomy and its relationships with adjacent structures, as well as to verify concomitant cardiac structural alterations.
带有右侧主动脉弓的Kommerell憩室是一种罕见的先天性解剖异常,其临床进程尚未完全确定。可能会发生并发症,这取决于局部结构的解剖分布、动脉瘤的大小和范围。血管改变和更广泛的病变与更严重的不良后果有关,如血栓、动脉夹层和破裂。血管磁共振不仅可以作为诊断的一种成像方法,而且可以作为门诊随访的一种成像方法,因为它不使用电离辐射或碘造影剂来获取图像。
Kommerell diverticulum with a right-sided aortic arch is a rare congenital anatomical condition in which clinical progression is not completely established. Complications may occur, which depend on the anatomical distribution of local structures, the size, and the extent of the aneurysm. Greater fragility this vascular alteration and more extensive lesions are associated with occurrence with more adverse consequences such as thromboses, dissections, and ruptures. Magnetic angio-resonance can be used as an imaging method not only for diagnosis, but also in outpatient follow-up, since it does not use ionizing radiation or iodinated contrast for image acquisition