Epidemiology of Hirschsprung disease in California from 1995 to 2013

PurposeThis study seeks to update current epidemiology of Hirschsprung disease (HD) in California.MethodsUsing 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.ResultsOf 9.3million 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 6days (interquartile range [IQR] 37 weeks 1day-40 weeks 1day). Mortality during the first year of life was 1.7%. Median age at death was 14.5days (IQR 0-113days).ConclusionThis 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%.