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Contemporary challenges and outcomes in children with ultrashort bowel syndrome: A descriptive cohort study
Hadar Moran-Lev1,2, Samuel A Kocoshis1,3, Mariah Mukasa1
1Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati School of Medicine, Cincinnati, Ohio, USA.
Insights
Children with ultra-short bowel syndrome (USBS) need more parenteral nutrition but face similar complications as short bowel syndrome (SBS) patients. Specialized care can lead to favorable outcomes for USBS children.
Area of Science:
- Pediatric Gastroenterology
- Intestinal Failure Management
- Surgical Outcomes
Background:
- Ultra-short bowel syndrome (USBS) is the most severe form of short bowel syndrome (SBS).
- Contemporary outcome data for pediatric USBS are limited.
- Understanding USBS management is crucial for improving patient care.
Purpose of the Study:
- To describe the management experience of children with USBS.
- To assess long-term outcomes in pediatric USBS patients.
- To compare USBS outcomes with those of standard SBS.
Main Methods:
- Retrospective analysis of pediatric intestinal failure (IF) patients (2018-2022).
- Matching USBS patients with SBS controls by age and sex.
- Evaluation of outcomes including cholestasis, CLABSI, mortality, oral aversion, and vitamin deficiencies.
Main Results:
- USBS patients had significantly shorter small and large bowel lengths compared to SBS.
- USBS patients showed higher parenteral nutrition (PN) dependency (93% vs. 30%).
- Similar rates of cholestasis and CLABSI were observed; oral aversion was more prevalent in USBS.
Conclusions:
- Children with USBS require greater PN support but have comparable major IF-related complication rates to SBS.
- Specialized care is associated with favorable long-term outcomes in pediatric USBS.
- High rates of vitamin deficiencies necessitate ongoing monitoring in both groups.
Objectives:
Ultra-short bowel syndrome (USBS) constitutes the most severe form of short bowel syndrome (SBS). Contemporary outcome data are scarce. The aim was to describe the experience of managing children with USBS and assess outcomes.
Methods:
This retrospective study analyzed children with intestinal failure (IF) managed at a single center between 2018 and 2022. Patients with USBS were matched to SBS controls by age and sex. Demographics and medical history were retrieved. Primary outcomes focused on long-term complications, including cholestasis, central line-associated bloodstream infections (CLABSI), mortality, oral aversion, and vitamin deficiencies.
Results:
The cohort included 28 children (median age: 44.4 months), 14 with USBS. Compared to SBS, children with USBS had significantly shorter small bowel lengths (percentage of remaining bowel length 6 (4, 8) vs. 25 (18, 29), p < 0.001) and reduced large bowel percentages (58% vs. 100%, p < 0.01). At data collection, 93% of USBS patients required PN, compared to 30% with SBS. Despite longer PN dependency, rates of cholestasis and CLABSI were similar. Oral aversion was more prevalent in USBS (71% vs. 21%, p < 0.001). High rates of vitamin B12 and iron deficiencies were observed in both groups. No mortality was recorded, and one USBS patient achieved enteral autonomy.
Conclusions:
When compared with SBS, children with USBS exhibited greater PN dependency but similar rates of major IF-related complications. These findings highlight that even among children with USBS and intestinal failure, long-term outcomes can be favorable under specialized care.
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