Iqbal Shams, Chaudhary Mahesh
Department of Interventional Radiology, Massachusetts General Hospital, Boston, MA, USA.
Cardiovasc Diagn Ther. 2021 Oct;11(5):1172-1176. doi: 10.21037/cdt-20-846.
Median arcuate ligament syndrome (MALS) is a rare condition which is due to the compression of celiac trunk by low riding of fibrous attachments of median arcuate ligament and diaphragmatic crura. Technically, MALS is a diagnosis of exclusion, consisting of vague symptoms comprising of postprandial epigastric pain, nausea, vomiting and unexplained weight loss. Different imaging modalities like Doppler ultrasound, computed tomography, magnetic resonance imaging and mesenteric angiogram are helpful to demonstrate celiac axis compression. The goal of treatment is decompression of celiac trunk either by open, laparoscopic or robotic method along with adjuvant interventional procedures like percutaneous transluminal angioplasty (PTA) and stenting. Surgical is the mainstay of treatment. This approach is based on open, laparoscopic or robotic release of compressed ligament along with celiac ganglionectomy and celiac artery revascularization. The role of interventional radiology is limited to angioplasty and stenting to open the stenosis rather than addressing the underlying compression of celiac trunk which has resulted in the symptoms. However, both the diagnosis and therapeutic intervention remains challenging. Extensive evaluation of etiology and pathophysiology of MALS and addressing the same through minimally invasive techniques may yield best prognosis in future. In this review article, we discuss briefly about the MALS in terms of etiology, diagnosis and its management including the role of interventional radiology.
正中弓状韧带综合征(MALS)是一种罕见疾病,病因是正中弓状韧带和膈脚的纤维附着点位置下移,压迫腹腔干。从技术层面讲,MALS是一种排除性诊断,症状模糊,包括餐后上腹部疼痛、恶心、呕吐和不明原因的体重减轻。不同的成像方式,如多普勒超声、计算机断层扫描、磁共振成像和肠系膜血管造影,有助于显示腹腔干受压情况。治疗的目标是通过开放手术、腹腔镜手术或机器人手术方法对腹腔干进行减压,并辅以经皮腔内血管成形术(PTA)和支架置入等介入程序。手术是主要的治疗方法。这种方法基于对受压韧带进行开放、腹腔镜或机器人松解,同时进行腹腔神经节切除术和腹腔动脉血运重建。介入放射学的作用仅限于通过血管成形术和支架置入来打开狭窄,而不是解决导致症状的腹腔干潜在受压问题。然而,诊断和治疗干预仍然具有挑战性。对MALS的病因和病理生理学进行广泛评估,并通过微创技术加以解决,可能在未来产生最佳预后。在这篇综述文章中,我们简要讨论了MALS的病因、诊断及其管理,包括介入放射学的作用。