Abstract

This study investigates how the epidemiology, hospital utilization, and surgical management of Hirschsprung disease (HD) have changed over the last decade in the United States, using a statistically valid national sample. HD is a congenital gastrointestinal disorder that requires surgical resection for correction. Some patients experience long-term bowel dysfunction requiring hospital care. Historically, patients had multiple staged operations, whereas more recently, single-stage laparoscopic resection and pull-through operations are more common. Assessment of possible changes over time in HD-associated complications requiring hospitalization and length of hospital stay has not been quantified epidemiologically. The Kids' Inpatient Database was queried for all discharges with an International Classification of Disease, Ninth Revision, Clinical Modification code for HD in the years 1997, 2000, 2003, and 2006. The HD cohorts from these 4 time points were compared, specifically analyzing differences in demographic data, associated diagnoses, in-hospital mortality and length of stay, procedures performed during hospitalization, and frequency of hospitalizations for HD-associated complications. Results reported included estimated frequencies and means with 95% confidence intervals. The estimated numbers of HD discharges, associated demographic data, and numbers of pull-through procedures have remained stable over the decade. The mean age (years) at the time of pull-through has decreased from 1.45 to 1.16 to 1.18 to 0.97 (P = 0.01). The mean length of stay (days) for these procedures has increased from 8.40 to 8.46 to 9.25 to 10.55 (P = 0.002). The estimated numbers of hospital admissions for HD-related constipation increased in recent years from 395 to 340 to 536 to 566 (P = 0.001). The estimated numbers of admissions for enterocolitis suggest an increasing trend from 466 to 402 to 584 to 556 (P = 0.11). HD pull-through procedures are being performed at younger ages over time, and post-pull-through lengths of stay have increased. Admissions for some HD-related complications have increased over the decade. Prospective cohort studies are needed to determine whether causal relationships exist among these trends.

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