Hu Qiang, Zhao Yan, Yang Yi, Wang Gang
Department of Cardiology, Air Force Hospital of Western Theater Command, Chengdu, China.
Department of Ultrasound, Air Force Hospital of Western Theater Command, Chengdu, China.
J Cardiothorac Surg. 2025 Apr 18;20(1):214. doi: 10.1186/s13019-025-03415-9.
Median arcuate ligament syndrome (MALS), also called celiac artery compression syndrome or Dunbar syndrome, is a rare disorder caused by the compression of the celiac trunk by the median arcuate ligament, which results in patients presenting with bloating, vomiting, nausea, weight loss, and postprandial abdominal pain.
A 77-year-old male was admitted to our center with irregular abdominal pain over the epigastric region for the past 5 months. The pain occurred with no apparent causes, which had intensified in the last 10 days, without nausea, vomiting, and other symptoms. The physical examination, laboratory examination, abdominal ultrasound, and gastroenterological endoscope showed no obvious abnormalities. The angiography showed that the celiac artery was 90% narrowed, so revascularization was performed, leading to a resolution of the symptoms. After 6 months, the patient presented with a recurrence of abdominal pain. Computed tomography angiography showed the stent in the ostial celiac artery was compressed and deformed, which obstructed the vessel. Finally, due to the advanced age, and high surgical risk, the patient was not willing for the decompression of the celiac artery, and the post-dilation was performed, resulting in < 50% residual stenosis in the ostial celiac artery and resolution of pain.
The current diagnosis of MALS is still based on postprandial abdominal pain and imaging modalities. However, due to the atypical symptoms and imaging manifestation, MALS is diagnosed precisely only after extensive evaluation and exclusion. In our case, celiac artery stenosis was initially diagnosed based on the symptoms and the results from angiography, so the revascularization of the celiac artery was conducted, leading to the deformation of the stent and a recurrence of abdominal pain 6 months later. MALS and decompression of the celiac trunk were finally considered. Although he refused to undergo celiac artery decompression because of the high surgery risk, the abdominal pain was relieved by post-dilation during the follow-up of 8 months.
Due to the vague manifestation, MALS should be considered after excluding intestinal disorders using different imaging modalities. Once diagnosed, the goal of treatment was centered around the decompression of the celiac artery.
正中弓状韧带综合征(MALS),也称为腹腔干压迫综合征或邓巴综合征,是一种罕见的疾病,由正中弓状韧带压迫腹腔干引起,导致患者出现腹胀、呕吐、恶心、体重减轻和餐后腹痛。
一名77岁男性因上腹部不规则腹痛5个月入住我院。疼痛无明显诱因发作,在过去10天内加重,无恶心、呕吐等症状。体格检查、实验室检查、腹部超声和胃肠内镜检查均未发现明显异常。血管造影显示腹腔干狭窄90%,因此进行了血运重建,症状得以缓解。6个月后,患者腹痛复发。计算机断层血管造影显示腹腔干开口处的支架受压变形,阻塞了血管。最后,由于患者年龄较大且手术风险高,患者不愿接受腹腔干减压,遂进行了球囊扩张,导致腹腔干开口处残余狭窄<50%,疼痛缓解。
目前MALS的诊断仍基于餐后腹痛和影像学检查。然而,由于症状和影像学表现不典型,MALS只有在经过广泛评估和排除后才能准确诊断。在我们的病例中,最初根据症状和血管造影结果诊断为腹腔干狭窄,因此进行了腹腔干血运重建,导致支架变形,6个月后腹痛复发。最终考虑为MALS并进行腹腔干减压。尽管由于手术风险高他拒绝接受腹腔干减压,但在8个月的随访中,球囊扩张缓解了腹痛。
由于表现模糊,在使用不同影像学检查排除肠道疾病后应考虑MALS。一旦确诊,治疗目标应以腹腔干减压为中心。