Contemporary challenges and outcomes in children with ultrashort bowel syndrome: A descriptive cohort study

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.