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Epidemiology of Hirschsprung disease in California from 1995 to 2013

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Abstract

Purpose

This study seeks to update current epidemiology of Hirschsprung disease (HD) in California.

Methods

Using data from the California Office of Statewide Health Planning and Development Linked Birth (1995–2012) and Patient Discharge Databases (1995–2013), patients from either dataset with an ICD-9 diagnosis code of HD (751.3) or procedure code of Soave (48.41), Duhamel (48.65), or Swenson/other pull-through (48.49) were included. Patients > age 18 during their first admission were excluded.

Results

Of 9.3 million births, 2,464 patients were identified. Incidence was 2.2 cases/10,000 live births, with rates peaking at 2.9/10,000 births in 2002. Incidence was highest among African American (4.1/10,000) and Asian/Pacific Islander (2.5/10,000) births. Most were male (n = 1652, 67.1%). Sixty patients (2.4%) had Down syndrome. The median gestational age at birth was 38 weeks 6 days (interquartile range [IQR] 37 weeks 1 day–40 weeks 1 day). Mortality during the first year of life was 1.7%. Median age at death was 14.5 days (IQR 0–113 days).

Conclusion

This is one of the largest population-based studies of HD. In California, the incidence of HD is stable, risk is highest among African American children, and the mortality rate is < 2%.

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Funding

This study was not funded.

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Authors and Affiliations

  1. Division of Pediatric General, Thoracic, and Fetal Surgery, University of California, Davis Medical Center, 2215 Stockton Boulevard, OP512, Sacramento, CA, 95817, USA

    Jamie E. Anderson, Melissa A. Vanover, Payam Saadai, Rebecca A. Stark, Jacob T. Stephenson & Shinjiro Hirose

Authors
  1. Jamie E. Anderson
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  2. Melissa A. Vanover
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  3. Payam Saadai
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  4. Rebecca A. Stark
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  5. Jacob T. Stephenson
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  6. Shinjiro Hirose
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Corresponding author

Correspondence to Jamie E. Anderson.

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Conflict of interest

None of the authors have any conflicts of interest to declare.

Ethical approval

All studies involving human participants were in accordance with the ethical standards of the institutional and state research committees (UC Davis Institutional Review Board and the California Committee for the Protection of Human Subjects) and with the 1964 Helsinki declaration and its later amendments.

Informed consent

Informed consent was not obtained as this was a database study and consent was not able to be obtained and data were deidentified; this was found to be acceptable per the institutional research committees listed above.

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Anderson, J.E., Vanover, M.A., Saadai, P. et al. Epidemiology of Hirschsprung disease in California from 1995 to 2013. Pediatr Surg Int 34, 1299–1303 (2018). https://doi.org/10.1007/s00383-018-4363-9

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  • DOI: https://doi.org/10.1007/s00383-018-4363-9

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