Hirschsprung disease in the premature newborn: a population based study and 40-year single center experience

Earl C Downey; Elizabeth Hughes; Angelica R Putnam; Henry J Baskin; Michael D Rollins

doi:10.1016/j.jpedsurg.2014.10.013

Hirschsprung disease in the premature newborn: a population based study and 40-year single center experience

J Pediatr Surg. 2015 Jan;50(1):123-5. doi: 10.1016/j.jpedsurg.2014.10.013. Epub 2014 Oct 30.

Authors

Earl C Downey¹, Elizabeth Hughes¹, Angelica R Putnam², Henry J Baskin³, Michael D Rollins⁴

Affiliations

¹ Division of Pediatric Surgery, Primary Children's Medical Center and the University of Utah, Salt Lake City, UT, USA.
² Division of Pathology, Primary Children's Medical Center and the University of Utah, Salt Lake City, UT, USA.
³ Division of Radiology, Primary Children's Medical Center and the University of Utah, Salt Lake City, UT, USA.
⁴ 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.

PMID: 25598107
DOI: 10.1016/j.jpedsurg.2014.10.013

Abstract

Background/purpose: 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.

Keywords: Hirschsprung disease; Prematurity.

MeSH terms

Birth Weight
Child
Female
Gestational Age
Hirschsprung Disease / diagnosis*
Hirschsprung Disease / epidemiology*
Humans
Incidence
Infant
Infant, Newborn
Infant, Premature
Infant, Premature, Diseases / diagnosis*
Infant, Premature, Diseases / epidemiology*
Male
Utah / epidemiology