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Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS
Anna Woestemeier1, Alexander Semaan2, Andreas Block2
1Department for General, Visceral, Thoracic and Vascular Surgery, University Hospital of Bonn, Bonn, Germany. anna.woestemeier@ukbonn.de.
Insights
Median arcuate ligament syndrome (MALS) surgery offers symptom relief for many. However, co-existing mast cell activation syndrome (MCAS) may predict persistent symptoms, necessitating careful patient selection for surgical MAL release.
Area of Science:
- Vascular Surgery
- Gastroenterology
- Rare Diseases
Background:
- Median arcuate ligament syndrome (MALS) involves celiac artery compression, treated surgically via laparoscopic decompression.
- Surgical success in MALS varies, highlighting the need for improved patient selection.
- MALS symptoms can overlap with other conditions like mast cell activation syndrome (MCAS).
Purpose of the Study:
- To identify preclinical parameters predicting surgical outcomes in MALS patients.
- To determine factors influencing successful laparoscopic celiac artery decompression.
- To investigate the role of MCAS in MALS treatment outcomes.
Main Methods:
- Analysis of 20 patients undergoing laparoscopic celiac artery decompression for MALS.
- Evaluation of demographic, radiological, and operative parameters.
- Statistical correlation analysis between MCAS and postoperative symptom relief.
Main Results:
- 60% of MALS patients experienced symptom relief and reduced analgesic use post-surgery.
- No demographic, radiological, or operative factors predicted symptom relief.
- Mast cell activation syndrome significantly correlated with persistent symptoms (p=0.04).
Conclusions:
- Laparoscopic MAL release can provide immediate symptomatic improvement in MALS.
- Co-existing MCAS is associated with persistent symptoms post-surgery, suggesting potential symptom overlap.
- Careful patient selection, potentially involving an interdisciplinary team, is crucial for optimizing surgical outcomes in MALS.
Background:
The 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.
Results:
By 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.
Conclusions:
Overall, 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.
