Hirschsprung Disease Trends in the United States: Analysis of the National Inpatient Sample

Inpatient Care Utilization and Epidemiology of Hirschsprung Disease in the United States: Analysis of the National Inpatient Sample

Hirschsprung disease (HD) is associated with significant morbidities including long-term bowel dysfunction. The aim of this study was to update national and regional trends in the epidemiology and inpatient care utilization of HD in the United States between 2009 and 2014 using the National Inpatient Sample (NIS).

Hirschsprung disease (HD) is a congenital condition characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the distal intestine, which results in lack of peristalsis and functional intestinal obstruction. The aganglionosis involves the rectum or rectosigmoid in most cases, but it can extend for varying lengths, and in 5-10% of cases can involve the entire colon or even a significant amount of the small intestine. The incidence of this disease is approximately 1 in 5000 live births and is approximately three times more common in males. HD is a multifactorial disease caused by both genetic and environmental factors, and HD may also be part of a syndrome, most commonly Trisomy 21.

The principal purpose of surgical management of HD is to remove aganglionic bowel and reconstruct the intestinal tract by connecting the normally innervated bowel just above the anus so that normal sphincter function is preserved. Over the last decades, surgical management has changed from original three-stage approach to recent introduction of minimally invasive one-stage procedures, which appears to be safe, and facilitates early feeding and discharge. Although surgical treatment definitively removes the histologically defined pathological intestinal segment, many patients with HD continue to experience bowel dysfunction, obstructive symptoms and recurrent enterocolitis requiring frequent hospitalizations.

Huang et al had shown that HD discharges, associated demographics, and numbers of pull-through procedures remained stable from 1997 to 2006. There is a paucity in the literature regarding cost components associated with care of hospitalized patients with the primary diagnosis of HD. An information update regarding inpatient care utilization for HD is needed to better understand current HD disease burden and inpatient care practice. The primary objective of this study was to analyze cost of HD-related admissions in the United States between 2009 and 2014 using the National Inpatient Sample (NIS) database. Secondary objectives were to update national and regional trends in the epidemiology of HD related hospital utilization, and to examine changing demographics, hospital mortality and length of hospital stay (LOS) in these HD patients.

Inclusion Criteria:

• We included all patients who were 18 years or younger with an ICD-9-CM code of 751.3 for HD among the first 10 diagnosis.

Exclusion Criteria:

• Data from 2015 to 2018 were not included since diagnoses and procedures were reported using the ICD-10-CM coding system.