Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21.
OBJECTIVE
The purpose of this study was to study the effect of trisomy 21 (T21) on enterocolitis rates and bowel function among children with Hirschsprung disease (HD).


METHODS
A retrospective cohort study of patients with HD treated at our tertiary children's hospital (2000-2015) and a cohort of patients with HD treated in our pediatric colorectal center (CRC) (2011-2015) were performed.


RESULTS
26/207 (13%) patients with HD had T21. 70 (41%) with HD alone were diagnosed with enterocolitis episodes compared to 9 (38%) with HD+T21 (p=0.71). 55/207 patients were managed in the CRC. 11/55 patients (20%) had HD+T21. 25 (58%) with HD had one or more enterocolitis episodes compared to 4 (36%) with HD+T21 (p=0.20). Number of hospitalizations for enterocolitis was similar between all groups. Toilet training was assessed in 32 CRC patients (25 HD, 7 HD+T21). One child with HD+T21 was toilet trained by age 4years versus 12 with HD (p=0.20). Laxative or enema therapy was required for constipation management in 57% HD versus 64% HD+T21.


CONCLUSIONS
Enterocolitis rates in children with HD+T21 did not differ from rates in children with HD alone. The majority of patients with CRC follow-up had constipation requiring laxative or enema therapy, which demonstrates the need for consistent postoperative follow-up.


METHODS
Retrospective Study - Level II.
University of Utah School of Medicine, 30 N 1900 E, Salt Lake City, UT 84132, USA.
Natasha
N
Kwendakwema
Division of Epidemiology, Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA.
Ragheed
R
Al-Dulaimi
Division of Epidemiology, Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA.
Angela P
AP
Presson
Primary Children's Hospital, 100 N Mario Cappechi Dr., Salt Lake City, UT 84113, USA.
Sarah
S
Zobell
University of Utah School of Medicine, 30 N 1900 E, Salt Lake City, UT 84132, USA.
Austin M
AM
Stevens
University of Utah School of Medicine, 30 N 1900 E, Salt Lake City, UT 84132, USA; Primary Children's Hospital, 100 N Mario Cappechi Dr., Salt Lake City, UT 84113, USA.
Brian T
BT
Bucher
University of Utah School of Medicine, 30 N 1900 E, Salt Lake City, UT 84132, USA; Primary Children's Hospital, 100 N Mario Cappechi Dr., Salt Lake City, UT 84113, USA.
Douglas C
DC
Barnhart
University of Utah School of Medicine, 30 N 1900 E, Salt Lake City, UT 84132, USA; Primary Children's Hospital, 100 N Mario Cappechi Dr., Salt Lake City, UT 84113, USA. Electronic address: Michael.Rollins@imail2.org.
Michael D
MD
Rollins
D016022:Case-Control Studies; D002675:Child, Preschool; D003248:Constipation / Q000209:etiology / Q000628:therapy; D004314:Down Syndrome / Q000150:complications* / Q000503:physiopathology; D004760:Enterocolitis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006627:Hirschsprung Disease / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors
Enterocolitis; HAEC; Hirschsprung disease; Trisomy 21