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

BACKGROUND

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.

METHODS

Patients <18 years undergoing robotic MAL release, celiac ganglionectomy, and lymphadenectomy from 2020 to 2024 were evaluated. Clinical and histopathologic outcomes were analyzed.

RESULTS

Twelve patients met inclusion criteria and were reviewed (15.7 ± 1.2 years, 75% female, BMI 20.9 [18.6-24.0] kg/m). 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 (χ = 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.

CONCLUSION

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.

LEVEL OF EVIDENCE

IV.