Surgical Management of Pediatric Superior Mesenteric Artery Syndrome: A Single Institution Continued Experience

Introduction: Superior mesenteric artery syndrome (SMAS) is an uncommon cause of abdominal pain, emesis, and weight loss in children. While there are many surgical options, we previously reported a small series of laparoscopic duodenal lowering (DL), which became our standard first approach. Here, we report our expanded experience on the surgical management of patients with SMAS. Methods: We conducted a retrospective chart review of patients who underwent surgical management of SMAS between January 2008 and December 2023 at our institution. Results: 22 patients underwent surgical management for SMAS during this study period. The majority of patients were female ( n = 19, 86%), and median age at time of surgery was 17 years (interquartile ranges: 16.0, 18.4). The median measured aorto-SMA angle was 16° (12, 20), with 3 patients having an aorto-SMA angle as narrow as 10°. Twenty patients underwent laparoscopic DL, and 2 patients underwent laparoscopic duodenojejunostomy, as they had previously undergone surgical procedures for SMAS at another institution (open duodenal de-rotation, open DL, and median arcuate ligament release). Of the 20 patients who underwent DL, 12 patients (60%) had resolution of their symptoms. Two of these patients had recurrence of symptoms within 6 months and underwent subsequent LDJ. Of the 8 patients without improvement after DL, 2 underwent subsequent LDJ with improvement of symptoms. The median follow-up for all patients was 51 days (33, 321). In both univariate and multivariate statistical analyses, having a diagnosis of Ehlers–Danlos syndrome was predictive of failure to achieve symptom resolution after DL ( P = .004). No other clinical or demographic characteristics were found to be predictive of successful resolution of symptoms from surgery. Conclusions: Patients with SMAS are a complex population. The more conservative DL can be an efficacious first step when approaching surgical management of SMAS. Future investigation is warranted to characterize which patients are more likely to fail operative intervention or require repeat operations.