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

February 21, 2025 updated by: Rajmohan Dharmaraj, University of New Mexico

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).

Study Overview

Status

Terminated

Conditions

Intervention / Treatment

Detailed Description

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.

Study Type

Observational

Enrollment (Actual)

2787

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • United States
    • New Mexico
      • Albuquerque, New Mexico, United States, 87106
        • University of New Mexico

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

1 day to 18 years (Child, Adult)

Accepts Healthy Volunteers

N/A

Sampling Method

Probability Sample

Study Population

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. Patients whose discharge disposition was transfer to another hospital were excluded to avoid over-counting. We identified patients with a pull-through procedure using following ICD-9-CM codes: 4592, 4593, 4594, 4595, 4840, 4841, 4842, 4843, 4849, and 4865. We tracked the number of these pull-through procedures a patient received. We used all 15 procedure codes to identify pull-through procedures and other procedures. To identify common discharge diagnoses we used the first five diagnosis codes. To identify congenital anomalies we used all diagnosis codes.

Description

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.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Time Frame
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.
Time Frame: 2009 to 2014.
2009 to 2014.

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

University of New Mexico

Investigators

  • Study Director: Yiliang Zhu, University of New Mexico

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

March 13, 2019

Primary Completion (Actual)

August 15, 2021

Study Completion (Actual)

August 15, 2021

Study Registration Dates

First Submitted

August 31, 2021

First Submitted That Met QC Criteria

August 31, 2021

First Posted (Actual)

September 9, 2021

Study Record Updates

Last Update Posted (Actual)

March 25, 2025

Last Update Submitted That Met QC Criteria

February 21, 2025

Last Verified

February 1, 2025

More Information

Terms related to this study

Additional Relevant MeSH Terms

Other Study ID Numbers

  • 18-697

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

NO

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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