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

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.