Surgical Outcomes for Patients With Trisomy 21 and Hirschsprung's Disease: An NSQIP-Pediatric Study.

Introduction: Previous studies have demonstrated worse outcomes for Hirschsprung's disease (HD) procedures in Trisomy 21 (T21) patients. Using a large national database, we sought to investigate surgical outcomes in HD patients with T21 compared to non-T21 patients.

Methods: We utilized the deidentified National Surgical Quality Improvement Program Pediatric database from 2012 to 2021. Using International Classification of Diseases, Ninth Revision codes, children <18 y old with HD were included and stratified by T21 diagnosis. Demographics, Current Procedural Terminology codes, case characteristics, length of hospital stay, and postoperative complications were analyzed.

Results: Of 3456 HD patients, 12.0% (n = 413) patients had a concurrent diagnosis of T21. Pull-through (PT) procedures accounted for 54.9% of surgeries (n = 1896), of which 10.0% (n = 189) had T21. T21 patients who underwent PT had a younger gestational age (P < 0.0001), cardiac risk factors (P < 0.0001), hematologic disorders (P < 0.0001), higher American Society of Anesthesiologists class (P < 0.0001), and were older at their index operation (P = 0.03). Though operative times were similar, T21 patients had a longer total length of stay (P = 0.0263), postoperative length of stay (P = 0.0033), and more unplanned reoperations (P = 0.0094). Though only significant in unadjusted analyses, T21 patients had more postoperative complications after PT (P = 0.0034), specifically deep surgical site infections (P = 0.009), organ/space surgical site infections (P = 0.004), wound disruption (P < 0.001), and sepsis (P = 0.025).

Conclusions: We confirm significant differences exist between T21 and non-T21 patients undergoing HD procedures, particularly increased total length of stay, postoperative length of stay, and unplanned reoperations. Understanding these differences will lead to more optimal treatment plans for this unique patient population.