Journal of pediatric surgery · 2025
Intestinal failure outcomes in children with small-intestinal Hirschsprung disease: A matched study.
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Etskovitz Haley, Chugh Priyanka V, Kim Rosa S, Nes Emily K, Fénnich Hajar, Haroon Mian Arsam, Froehle Leah, Staffa Steven J, Dickie Belinda Hsi, Modi Biren P, Nandivada Prathima
Аннотация
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.
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.