Epidemiology of Hirschsprung disease in California from 1995 to 2013

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

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


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%.

Original languageEnglish (US)
Pages (from-to)1299-1303
Number of pages5
JournalPediatric Surgery International
Issue number12
StatePublished - Dec 1 2018


  • California
  • Epidemiology
  • Hirschsprung disease
  • Incidence

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery


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