Intestinal failure outcomes in children with small-intestinal Hirschsprung disease: A matched study.

INTRODUCTION

Small intestinal-Hirschsprung disease (SI-HD) causing intestinal failure (IF) leads to parenteral nutrition (PN) dependence, liver disease (IFALD), and infections (CLABSI). We aim to identify factors associated with enteral autonomy in patients with SI-HD.

METHODS

Retrospective cohort study of 25 patients with IF from SI-HD matched on expected small bowel length and rehabilitation duration to patients with IF of other etiologies.

RESULTS

SI-HD patients required 53.6 months (31.1, 97.7) of PN versus 22.7 months (9.4, 54.9) for non-SI-HD patients (p = 0.015). Patients with SI-HD and <50 % expected bowel length required longer PN duration (50.1 months) than controls (26.7 months, p = 0.038) (Figure 1). No difference was found in PN duration between groups for patients with >50 % expected bowel length. At follow-up, patients with SI-HD and <50 % expected bowel length received 37.5 % of their calories enterally, while controls received 87.5 % (p = 0.002). There was no difference in percent enteral nutrition in patients with >50 % expected bowel length. SI-HD patients were less likely to have intestinal continuity (96 % with ostomy versus 6 % of controls (p = <0.001)). No SI-HD patients had colon in continuity, compared to 94 % of non-SI-HD patients (p < 0.001). SI-HD patients had a higher incidence of CLABSI (92 % vs 66 %, p = 0.03). There was no difference in incidence of IFALD.

CONCLUSIONS

Patients with SI-HD + IF are more likely to have persistent PN dependence than those with IF from other etiologies, this was most apparent in patients with <50 % bowel length. Worse prognosis is likely related to long-term ostomies and absence of colon.