The prevalence and clinical presentation of Hirschsprung's disease in preterm infants: a systematic review and meta-analysis.

PURPOSE: Hirschsprung's disease (HD) is a rare occurrence in premature infants, and the exact prevalence varying across studies. Thus, we conducted this study to determine the prevalence of and clinical presentation of HD in preterm infants.

METHODS

The PubMed, EmBase, and Cochrane library databases were searched for eligible studies throughout May 2021. The pooled incidence with 95% confidence interval (CI) was calculated using the random-effects model.

RESULTS

Thirty studies involved 4,557 children with HD were included. The pooled proportion of HD patients who were preterm infants was 7% (95% CI 5-8%; P < 0.001); the sensitivity analysis indicated that the pooled prevalence of preterm infants with HD ranged from 5.0% to 5.6%. Moreover, the pooled incidence of abdominal distention in HD patients was 83% (P < 0.001), that of vomiting was 61% (P < 0.001), that of bilious vomiting was 54% (P < 0.001), that of delayed meconium passage was 48% (P < 0.001), that of constipation was 44% (P < 0.001), and that of enterocolitis was 13% (P < 0.001).

CONCLUSIONS

This study reported the prevalence of HD patients who were preterm infants, and the most common presentations were abdominal distention, vomiting, bilious vomiting, delayed meconium passage, constipation, and enterocolitis.