First description of a histopathologic grading system and relationship to outcomes after robotic median arcuate ligament release with celiac ganglionectomy and lymphadenectomy Jamie DeCicco, BS a , Fnu Raja, MD b,d , Santhi Ganesan, MD b,d , Kevin El-Hayek, MD c,d,e,* a Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH b Department of Pathology, The MetroHealth System, Cleveland, OH c Division of General Surgery, Department of Surgery, The MetroHealth System, Cleveland, OH d Case Western Reserve University School of Medicine, Cleveland, OH e Northeast Ohio Medical University, Rootstown, OH article info Article history: Accepted 6 September 2023 Available online xxx abstract Background: Two dominating theories regarding median arcuate ligament syndrome include vascular and neurogenic etiologies from celiac artery and ganglion compression, respectively. Celiac ganglionectomy is not routine during surgery, and specimens are rarely excised; therefore, the extent of nerve involvement and his- topathology are unknown. Our study aims to characterize histopathologic findings in median arcuate ligament syndrome, establish a histopathologic grading system, and correlate with clinical outcomes. Methods: Robotic median arcuate ligament release, celiac ganglionectomy, and lymphadenectomy were performed with specimens excised and stained using hematoxylin & eosin, trichrome, and S100. Neu- rofibrosis, adiposity, and reactive changes were described, a grading scale was developed, and results were analyzed with clinical outcomes. Results: Fifty-four patients were evaluated, of whom 36 met inclusion criteria (81% female, 34.9 [25.9e47.5] years, body mass index 23.5 [19.6e28.1] kg/m 2 ). Histopathologic evaluation revealed fibrosis (hematoxylin & eosin and trichrome median score 1.5 [0e2.5]), reactive lymphadenopathy (89%), intraparenchymal nerves (31%), and lipogranulomas (31%). Greater fibrosis was associated with a lack of preoperative celiac plexus block relief (100% vs. 30%, P ¼.044) and lower postoperative celiac artery velocities (198 vs 323 cm/s, P ¼ .02). Intraparenchymal nerves were associated with greater decreases in pre to postoperative velocities (161 vs 84 cm/s, P ¼ .037). Symptoms improved in 28 patients (78%). Conclusion: We developed the first histopathologic grading system and identified unique findings of intraparenchymal nerves and lipogranulomas. Histopathologic abnormalities were associated with objective improvement and symptomatic relief postoperatively. These findings support nerve compression and inflammation as predominant contributors to median arcuate ligament syndrome pain, celiac ganglia resection to treat symptoms, and continued histopathologic analysis to better elucidate median arcuate ligament syndrome etiology. © 2023 Elsevier Inc. All rights reserved. Introduction Median arcuate ligament syndrome (MALS) is a rare disorder characterized by chronic postprandial abdominal pain, nausea, vomiting, and weight loss. 1,2 The median arcuate ligament (MAL) is a fibrous band of the diaphragm arching over the aortic hiatus to join the right and left diaphragmatic crura around the level of T12 to L1. 3,4 In 1917, Lipshutz first reported anatomical celiac artery compression by the MAL via an abnormally cephalad celiac trunk origin and/or an abnormally caudad diaphragmatic insertion. 4,5 Upwards of 10% to 25% of individuals have this anatomic varia- tion, putting some at risk for MALS. 4 Clinically, MALS was first described by Harjola in 1963 as postprandial epigastric pain with celiac axis stenosis. 6 In 1965, Dunbar proposed the mechanism of chronic or intermittent abdominal ischemia and published the first surgical treatments. 7e9 Presented at Central Surgical Association, June 8e10, 2023, in Cleveland, OH. * Reprint requests: Kevin El-Hayek, MD, FACS, Section Head, Endoscopic Surgery, Division of General Surgery, Section Head, Hepato-Pancreato-Biliary Surgery, Di- vision of Surgical Oncology, Department of Surgery, MetroHealth System, 2500 MetroHealth Drive, Mail Code H924, Cleveland, OH 44109. E-mail address: kelhayek@metrohealth.org (K. El-Hayek); Twitter: @jamiepdecicco, @fnuraja, @KevinElHayekMD Contents lists available at ScienceDirect Surgery journal homepage: www.elsevier.com/locate/surg https://doi.org/10.1016/j.surg.2023.09.024 0039-6060/© 2023 Elsevier Inc. All rights reserved. Surgery xxx (2023) 1e11 Downloaded for Anonymous User (n/a) at The MetroHealth System from ClinicalKey.com by Elsevier on December 13, 2023. For personal use only. No other uses without permission. Copyright ©2023. Elsevier Inc. All rights reserved.