Pediatric Outcomes After Robotic Median Arcuate Ligament Release, Celiac Ganglionectomy, and Lymphadenectomy

• Published in: Journal of pediatric surgery p. 161664
• Main Authors: DeCicco, Jamie, Raja, Fnu, Ganesan, Santhi, Kim, Chong, El-Hayek, Kevin
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 06.08.2024
• Subjects: Celiac artery compression syndrome (CACS); Celiac ganglionectomy; Chronic abdominal pain; Histopathology; Median arcuate ligament syndrome (MALS); Robotic surgery; Chronic abdominal pain; TAP; ASA; Histopathology; TPN; POTS; hEDS; BMI; H&E; Median arcuate ligament syndrome (MALS); SMAS; EMR; ICG; MCAS; Robotic surgery; CTA; GERD; Celiac ganglionectomy; ND; MALS; Celiac artery compression syndrome (CACS); NG; NJ; MRA; NCS
• ISSN: 0022-3468; 1531-5037; 1531-5037
• DOI: 10.1016/j.jpedsurg.2024.08.004

Median arcuate ligament syndrome (MALS) is a neurovascular disorder characterized by gastrointestinal symptoms due to celiac artery and ganglion compression. Limited literature primarily focuses on adults. This study aims to characterize clinical and histopathologic findings in pediatric MALS. Patients <18 years undergoing robotic MAL release, celiac ganglionectomy, and lymphadenectomy from 2020 to 2024 were evaluated. Clinical and histopathologic outcomes were analyzed. Twelve patients met inclusion criteria and were reviewed (15.7 ± 1.2 years, 75% female, BMI 20.9 [18.6–24.0] kg/m2). Comorbidities included depression/anxiety (83%), postural orthostatic tachycardia syndrome (POTS) (50%), gastroesophageal reflux disease (GERD) (50%), nutrition support (50%), mast cell activation syndrome (MCAS) (42%), hypermobile Ehlers-Danlos syndrome (hEDS) (42%), other vascular compression syndromes (33%). All patients who received preoperative celiac plexus block had temporary symptom relief (10/10). Mean operative time was 119.7 ± 22.4 min. No intraoperative complications, 30-day readmissions, reoperations, or complications occurred. Histopathologically, 92% had fibroadipose tissue, 100% had reactive lymph nodes, none had intraparenchymal nerves, and one had lipogranulomas. Median fibrosis scores were 1 [0.5–2] on H&E and 2 [1–2] on trichrome. Fibrosis score severity was not significantly associated with symptom improvement (χ2 = 3.67, p = 0.16). Median postoperative celiac artery velocity was 258.5 [192.5–350.5] cm/s with a median change of −80.5 [-106.1–+82.8] cm/s from preoperative 308.0 [229.3–344.0] cm/s (S = −5.0, p = 0.55). Those with lower preoperative velocities were more likely to have symptom improvement postoperatively (S = 19, p = 0.04). MALS symptoms improved in 83%; however, despite reporting “MALS pain” was improved, 64% (all female) had other comorbidities such as POTS, MCAS, hEDS, and colonic dysmotility contributing to other ongoing symptoms. Robotic MALS surgery is safe and effective in pediatrics. Clinical improvement rates and fibrosis scoring are comparable to adults; however, findings reveal challenges with multiple comorbidities contributing to separate symptoms that may continue postoperatively, particularly in females. We recommend a multidisciplinary team approach in addressing comorbidities and optimizing medical and surgical care. IV. •Published research on pediatric MALS is limited. Robotic outcomes and histopathologic findings are unknown.•This article demonstrates safety and efficacy of the robotic approach for pediatric MALS, evidence of neurofibrosis with extent similar to adult MALS, and a greater presence of comorbidities that can impact symptoms compared to adults.