Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS

Orphanet Journal of Rare Diseases

Research

Alexander Kania¹  Jörg C. Kalff¹  Alexander Semaan¹  Andreas Block¹  Anna Woestemeier¹  Philipp Lingohr¹  Frauke Verrel¹  Jan Arensmeyer¹  Jonas Dohmen¹  Martin Mücke² 

[1] Department for General, Visceral, Thoracic and Vascular Surgery, University Hospital of Bonn, Bonn, Germany;Institute for Digitalization and General Medicine, University Hospital Aachen, Aachen, Germany;Center for Rare Diseases Aachen (ZSEA), University Hospital Aachen, Aachen, Germany;

关键词: Median arcuate ligament syndrome;    Dunbar syndrome;    Celiac artery compression syndrome;    Mast cell activation syndrome;    CT angiography;    Vascular compression;   

DOI  :  10.1186/s13023-023-02952-7

 received in 2022-07-19, accepted in 2023-10-10,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundThe median arcuate ligament syndrome (MALS) is a rare disease caused by compression of the celiac artery (ORPHA: 293208). Surgical treatment of MALS aims to restore normal celiac blood flow by laparoscopic celiac artery decompression. However, surgical success rates vary widely between patients, therefore adequate selection of patients is essential to improve surgical outcome. Symptoms of MALS might also overlap with other chronic multi-system disorders such as mast cell activation syndrome (MCAS). So far, no clinical or radiological parameter was found to be predictive of the postoperative outcome. We, therefore, aim to study preclinical parameters in one of the largest MALS cohorts with the focus to identify patients that would benefit from surgical MAL release.ResultsBy analyzing 20 MALS patients that underwent surgical celiac artery decompression, we found 60% of patients (12/20) had a postoperative relief of their symptoms and a simultaneous decrease of analgetic use. No demographic, radiologic or operative parameter was able to predict postoperative symptom relief. However, mast cell activation syndrome correlated significantly (p = 0.04) with persistent symptoms after the operation.ConclusionsOverall, laparoscopic MAL release can provide immediate symptomatic relief. Despite the missing predictive value of demographic and imaging data, our data show a correlation between persistent symptoms and a co-existing mast cell activation syndrome. This suggests that MCAS symptoms might be interpreted as MALS symptoms in the presence of celiac artery stenosis and therefore surgical treatment should be evaluated carefully. Overall, the selection of patients who are most likely to respond to surgical MAL release may best be accomplished by an interdisciplinary team of gastroenterologists, radiologists and surgeons.

【 授权许可】

CC BY   
© Institut National de la Santé et de la Recherche Médicale (INSERM) 2023

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