Economic evaluation of Hirschsprung disease testing strategies for children with medically-refractory chronic constipation: A cost-effectiveness analysis.

BACKGROUND

Early and accurate diagnosis of Hirschsprung disease (HSCR) prevents life-threatening complications, improves patient outcomes, and reduces healthcare utilization. Contrast enema (CE), anorectal manometry (AMAN), and rectal biopsy (RB) are commonly used diagnostic tests, with RB being the gold standard. However, the most cost-effective initial diagnostic strategy remains unclear.

METHODS

A decision-analytic model was used to compare CE-first, AMAN-first, and RB-first strategies for the evaluation of HSCR in children between 1 and 18 years of age presenting to specialty care for chronic constipation. The model incorporated diagnostic test performance, disease prevalence, and timing of surgical intervention over a three-year time horizon. Health utilities were estimated by mapping published Pediatric Quality of Life Inventory (PedsQL) scores to Child Health Utility 9D (CHU9D) values. Costs were averaged from institutional data. Willingness-to-pay (WTP) threshold was set at $50,000 per quality-adjusted life year (QALY). Outcomes included costs, QALYs, net monetary benefit (NMB), and incremental cost-effectiveness ratios (ICERs). Deterministic and probabilistic sensitivity analyses were performed.

RESULTS

CE-first emerged as the most cost-effective diagnostic strategy at a WTP of $50,000/QALY. RB-first provided greater effectiveness but at higher cost and was not cost-effective at the base-case threshold. RB-first became preferred when HSCR prevalence exceeded 29.2% and when the sensitivity of the test exceeded 97.8%. AMAN-first was least cost-effective and became preferred only when its sensitivity exceeded 90.5%.

CONCLUSIONS

In the evaluation of children over 1 year of age presenting to specialty care with suspected HSCR, CE was the most cost-effective initial strategy in most scenarios. These findings provide economic insight to guide clinical decision-making for the diagnostic testing of HSCR.