Medical student, Jefferson Medical College, Philadelphia, Pennsylvania2now with Department of Surgery, University of California, San Francisco (UCSF), Fresno.
Division of Vascular and Endovascular Surgery, Department of Surgery, Thomas Jefferson University, Philadelphia, Pennsylvania.
JAMA Surg. 2016 May 1;151(5):471-7. doi: 10.1001/jamasurg.2016.0002.
Median arcuate ligament (MAL) syndrome is a rare disease resulting from compression of the celiac axis by fibrous attachments of the diaphragmatic crura, the median arcuate ligament. Diagnostic workup and therapeutic intervention can be challenging.
To review the literature to define an algorithm for accurate diagnosis and successful treatment for patients with MAL syndrome.
A search of PubMed (1995-September 28, 2015) was conducted, using the key terms median arcuate ligament syndrome and celiac artery compression syndrome.
Typically a diagnosis of exclusion, MAL syndrome involves a vague constellation of symptoms including epigastric pain, postprandial pain, nausea, vomiting, and weight loss. Extrinsic compression of the vasculature and surrounding neural ganglion has been implicated as the cause of these symptoms. Multiple imaging techniques can be used to demonstrate celiac artery compression by the MAL including mesenteric duplex ultrasonography, computed tomography angiography, magnetic resonance angiography, gastric tonometry, and mesenteric arteriography. Surgical intervention involves open, laparoscopic, or robotic ligament release; celiac ganglionectomy; and celiac artery revascularization. There remains a limited role for angioplasty because this intervention does not address the underlying extrinsic compression resulting in symptoms, although angioplasty with stenting may be used in recalcitrant cases.
Median arcuate ligament syndrome is rare, and as a diagnosis of exclusion, diagnosis and treatment paradigms can be unclear. Based on previously published studies, symptom relief can be achieved with a variety of interventions including celiac ganglionectomy as well as open, laparoscopic, or robotic intervention.
中位弓状韧带(MAL)综合征是一种罕见的疾病,由膈脚的纤维附着物压迫腹腔动脉引起,即中位弓状韧带。诊断和治疗干预可能具有挑战性。
回顾文献,定义一种用于准确诊断和成功治疗 MAL 综合征患者的算法。
在 PubMed 上进行了搜索(1995 年-2015 年 9 月 28 日),使用的关键词是中位弓状韧带综合征和腹腔动脉压迫综合征。
MAL 综合征通常是一种排除性诊断,涉及一系列模糊的症状,包括上腹痛、餐后疼痛、恶心、呕吐和体重减轻。血管和周围神经节的外源性压迫被认为是这些症状的原因。多种成像技术可用于显示 MAL 引起的腹腔动脉压迫,包括肠系膜双功能超声、计算机断层血管造影、磁共振血管造影、胃测压和肠系膜动脉造影。手术干预包括开放、腹腔镜或机器人韧带松解、腹腔神经节切除术和腹腔动脉血运重建。血管成形术的作用有限,因为这种干预并不能解决导致症状的潜在外源性压迫,尽管在顽固病例中可能使用血管成形术加支架。
中位弓状韧带综合征罕见,作为一种排除性诊断,诊断和治疗方案可能不明确。基于先前发表的研究,通过各种干预措施可以缓解症状,包括腹腔神经节切除术以及开放、腹腔镜或机器人干预。