Hirschsprung Disease in Down Syndrome: An Opportunity for Improvement
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Read the article on jpedsurg.org ↗Article · Feb 2022 · 1 min read
In brief
In brief
Retrospective analysis of 481 newborns with Hirschsprung disease found that the 9% with concurrent Down syndrome experienced delayed diagnosis, longer hospital stays, and higher wound infection rates compared to those without Down syndrome, highlighting opportunities for improved perioperative care in this vulnerable population.
Written by the GCMD Library team from the article.
Background
Down syndrome (DS) is the most common abnormality associated with Hirschsprung disease (HD). It has been suggested patients with HD and DS have worse outcomes, however the literature is controversial.
Methods
The Kids' Inpatient Database (KID) from 2003-2012 was used to identify newborns with HD. Demographics, hospital characteristics, and outcomes were compared amongst patients with and without DS using standard statistical tests.
Results
There were 481 patients identified with HD, of which 45 (9%) had DS. Patients with DS were older at the time of first rectal biopsy (6 [3-11] days vs. 4 [3-6] days, p=0.012). There were no differences in operative versus non-operative management in patients with and without DS (p=0.706). Hospital length of stay was longer in the DS cohort (22 [13-33] days vs. 15 [10-24] days, p=0.019), and patients with DS were more likely to have a concomitant diagnosis of wound infection (