Cargando…

Prognostic factors for the long term outcome after surgical celiac artery decompression 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 p...

Descripción completa

Detalles Bibliográficos
Autores principales: Woestemeier, Anna, Semaan, Alexander, Block, Andreas, Arensmeyer, Jan, Dohmen, Jonas, Kania, Alexander, Verrel, Frauke, Mücke, Martin, Kalff, Jörg C., Lingohr, Philipp
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10594872/
https://www.ncbi.nlm.nih.gov/pubmed/37872625
http://dx.doi.org/10.1186/s13023-023-02952-7
_version_ 1785124744887009280
author Woestemeier, Anna
Semaan, Alexander
Block, Andreas
Arensmeyer, Jan
Dohmen, Jonas
Kania, Alexander
Verrel, Frauke
Mücke, Martin
Kalff, Jörg C.
Lingohr, Philipp
author_facet Woestemeier, Anna
Semaan, Alexander
Block, Andreas
Arensmeyer, Jan
Dohmen, Jonas
Kania, Alexander
Verrel, Frauke
Mücke, Martin
Kalff, Jörg C.
Lingohr, Philipp
author_sort Woestemeier, Anna
collection PubMed
description 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13023-023-02952-7.
format Online
Article
Text
id pubmed-10594872
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher BioMed Central
record_format MEDLINE/PubMed
spelling pubmed-105948722023-10-25 Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS Woestemeier, Anna Semaan, Alexander Block, Andreas Arensmeyer, Jan Dohmen, Jonas Kania, Alexander Verrel, Frauke Mücke, Martin Kalff, Jörg C. Lingohr, Philipp Orphanet J Rare Dis Research 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13023-023-02952-7. BioMed Central 2023-10-23 /pmc/articles/PMC10594872/ /pubmed/37872625 http://dx.doi.org/10.1186/s13023-023-02952-7 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research
Woestemeier, Anna
Semaan, Alexander
Block, Andreas
Arensmeyer, Jan
Dohmen, Jonas
Kania, Alexander
Verrel, Frauke
Mücke, Martin
Kalff, Jörg C.
Lingohr, Philipp
Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS
title Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS
title_full Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS
title_fullStr Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS
title_full_unstemmed Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS
title_short Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS
title_sort prognostic factors for the long term outcome after surgical celiac artery decompression in mals
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10594872/
https://www.ncbi.nlm.nih.gov/pubmed/37872625
http://dx.doi.org/10.1186/s13023-023-02952-7
work_keys_str_mv AT woestemeieranna prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT semaanalexander prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT blockandreas prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT arensmeyerjan prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT dohmenjonas prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT kaniaalexander prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT verrelfrauke prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT muckemartin prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT kalffjorgc prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals
AT lingohrphilipp prognosticfactorsforthelongtermoutcomeaftersurgicalceliacarterydecompressioninmals