title: Hirschsprung disease in the premature newborn: a population based study and 40-year single center experience.
abstract: OBJECTIVE
  Understanding of Hirschsprung disease (HD) in premature newborns (PHD) is anecdotal. We have sought in this study to identify the demographic and clinical features of PHD.
  
  
  METHODS
  All patients with HD 1970-2011 treated at our tertiary care children's hospital were identified. Patients with biopsy confirmed HD and EGA <37weeks were selected for further review. Prenatal and birth data, demographics, clinical signs, radiologic and pathologic data, and operative interventions were examined. The occurrence of PHD was observed using data from the Utah Department of Health database 1997-2011.
  
  
  RESULTS
  404 patients with HD from 1970 to 2011 were treated. Twenty-seven (6.7%) had PHD. Mean birth weight in PHD was 2196grams and mean gestational age 34 (range 29-36)weeks. Seven patients had Down syndrome. Nonchromosomal anomalies occurred in 25%. Median time from birth to biopsy diagnosis was 42days (range 2-316days). The most common presenting signs were abdominal distension and bilious emesis. The HD incidence in Utah for all births was 1/4322 (0.023%) and for premature infants 1/3885 (0.027%).
  
  
  CONCLUSIONS
  PHD are similar to term infants with HD. Diagnosis of HD is often delayed in premature newborns, and associated anomalies are more common.
authors.affiliation: Division of Pediatric Surgery, Primary Children's Medical Center and the University of Utah, Salt Lake City, UT, USA.
authors.forename: Earl C
authors.initials: EC
authors.lastname: Downey
authors.affiliation: Division of Pediatric Surgery, Primary Children's Medical Center and the University of Utah, Salt Lake City, UT, USA.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Hughes
authors.affiliation: Division of Pathology, Primary Children's Medical Center and the University of Utah, Salt Lake City, UT, USA.
authors.forename: Angelica R
authors.initials: AR
authors.lastname: Putnam
authors.affiliation: Division of Radiology, Primary Children's Medical Center and the University of Utah, Salt Lake City, UT, USA.
authors.forename: Henry J
authors.initials: HJ
authors.lastname: Baskin
authors.affiliation: Division of Pediatric Surgery, Primary Children's Medical Center and the University of Utah, Salt Lake City, UT, USA. Electronic address: Michael.Rollins@imail2.org.
authors.forename: Michael D
authors.initials: MD
authors.lastname: Rollins
mesh_terms: D001724:Birth Weight; D002648:Child; D005260:Female; D005865:Gestational Age; D006627:Hirschsprung Disease / Q000175:diagnosis* / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D014589:Utah / Q000453:epidemiology
keywords: Hirschsprung disease; Prematurity