Pediatric Abdominal Vascular Compression Syndromes: Clinical Presentation, Comorbidities, and Outcomes Following Surgical Correction

The goal of this case series was to characterize the clinical presentation, comorbidities, and postoperative outcomes of pediatric patients who underwent surgical repair for Median Arcuate Ligament Syndrome (MALS) and/or Nutcracker Syndrome (NCS). We conducted a retrospective chart review of 17 pediatric patients who underwent open surgical repair for MALS and/or NCS at a single academic center from 2021-2025. The most common comorbidities included postural orthostatic tachycardia syndrome (POTS), hypermobile Ehlers-Danlos syndrome (hEDS), gallbladder pathology, and additional vascular compressions. All 17 patients presented with abdominal pain, and more than half reported postprandial pain and/or nausea. Open surgical repair provided patient-reported symptomatic improvement in all patients. A subset experienced recurrent pain at longer-term follow-up. Pediatric patients with MALS and/or NCS demonstrate consistent clinical features, including abdominal pain and overlapping comorbidities. In this highly selected surgical cohort, open repair was associated with early and intermediate-term patient-reported improvement in compression-related pain, though symptom recurrence was observed in a subset.