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Intestinal failure outcomes in children with small-intestinal Hirschsprung disease: A matched study
Haley Etskovitz1, Priyanka V Chugh1, Rosa S Kim1
1Department of General Surgery, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.
Insights
Patients with small intestinal-Hirschsprung disease (SI-HD) experience prolonged parenteral nutrition (PN) dependence and are less likely to achieve enteral autonomy, especially those with less than 50% of expected bowel length.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Intestinal Rehabilitation
Background:
- Small intestinal-Hirschsprung disease (SI-HD) is a severe condition causing intestinal failure (IF).
- IF necessitates parenteral nutrition (PN) and is associated with complications like liver disease and central line-associated bloodstream infections (CLABSI).
- Identifying factors for enteral autonomy in SI-HD is crucial for improving patient outcomes.
Purpose of the Study:
- To determine factors associated with achieving enteral autonomy in patients with SI-HD.
- To compare PN dependence and outcomes in SI-HD patients versus those with IF from other causes.
Main Methods:
- Retrospective cohort study comparing 25 SI-HD patients with IF to matched non-SI-HD patients.
- Matching criteria included expected small bowel length and duration of rehabilitation.
- Outcomes assessed included duration of PN, percentage of enteral nutrition, intestinal continuity, and incidence of complications.
Main Results:
- SI-HD patients required significantly longer PN duration (53.6 months) compared to non-SI-HD patients (22.7 months).
- Patients with SI-HD and <50% expected bowel length showed longer PN duration and received less enteral nutrition (37.5%) versus controls (87.5%).
- SI-HD patients had higher rates of ostomy (96%), absence of colon in continuity (100%), and CLABSI (92%) compared to controls.
Conclusions:
- SI-HD patients, particularly those with <50% expected bowel length, face a higher likelihood of persistent PN dependence.
- Long-term ostomies and lack of colonic continuity likely contribute to poorer prognosis in SI-HD.
- These findings highlight the challenges in achieving enteral autonomy for SI-HD patients.
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.
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