背景:儿童上主动脉瓣梗阻并不常见,在某些独特的情况下可见。虽然在老年人群中可以看到由于严重钙化引起的管腔内阻塞,在儿科人群中没有描述。珊瑚礁主动脉是一种罕见且独特的钙化疾病,可导致成人肾上主动脉腔阻塞。这种诊断的定义完全依赖于病变的独特方面和一致性,坚硬的岩石,不规则,坚硬的斑块,有白色的内腔表面,类似于珊瑚礁。然而,在儿童中没有描述这种情况。
方法:我们介绍了一个青春期男孩,他出现了严重钙化的升主动脉病变,伴有主动脉病变和高血压,主动脉缩窄修复术后12年。调查包括超声心动图,磁共振和计算机断层成像。打印了3-D模型,以便预先可视化和计划手术步骤,以安全放置夹具并定义切除范围。此外,它提供了一个关于组织质量的想法,厚度,空间关系,和相对于周围结构的方向。在体外循环支持下,成功切除和替换了主动脉的病变段。术后恢复顺利,在6个月的随访中,病人做得很好。在这份报告中,已经讨论了这些病变的各个方面,包括临床表现,并发症,安全体外循环的规划和实施,以及手术期间的预防措施。
结论:儿童并发梗阻性主动脉病变需要仔细评估,适当的先进成像,以及3D打印技术的使用,以便计划和执行安全有效的手术管理。儿童严重钙化主动脉的病因可能与代谢因素有关,以前的手术,使用同质移植物,或者炎症过程。然而,这还没有得到证明。
BACKGROUND: Supra aortic obstruction in children is uncommon and is seen in certain unique conditions. While intraluminal obstruction due to heavy calcification is seen in older populations, it is not described in pediatric populations. The coral reef aorta is a rare and distinct calcifying disease causing luminal obstruction of the suprarenal aorta in adults. The definition of this diagnosis relies entirely on the unique aspects and consistency of the lesions, which are rock-hard, irregular, gritty plaques with a white luminal surface resembling a coral reef. However, no such case has been described in children.
METHODS: We present an adolescent boy who presented with a heavily calcified ascending aortic lesion associated with aortopathy and hypertension, 12 years after an aortic coarctation repair. The investigations included echocardiography, magnetic resonance and computer-tomographic imaging. A 3-D model was printed in order to visualize and plan surgical steps in advance for safe placement of clamps and defining the extent of resection. In addition, it provided an idea about tissue quality, thickness, spatial relationship, and orientation in relation to surrounding structures. Successful resection and replacement of the diseased segment of the aorta were achieved on cardiopulmonary bypass support. Post-operative recovery was uneventful, and at 6-month follow-up, the patient is doing well. In this report, various aspects of such lesions have been discussed, including clinical presentations, complications, planning and conduct of a safe cardiopulmonary bypass, and precautions during surgery for a successful outcome.
CONCLUSIONS: Complicated obstructive aortic lesions in children require careful assessment, appropriate advanced imaging, and the use of 3-D printing technology in order to plan and perform safe and effective surgical management. The etiology of severe calcified aorta in children may be related to metabolic factors, previous surgery, use of a homograft, or an inflammatory process. However, it has yet to be proven.