Introduction: Patients with short bowel syndrome (SBS) are dependent on parenteral nutrition (PN) while their bowel attempts to compensate for loss of function. Our objective was to create a SBS disease severity score that would predict the probability of achieving enteral autonomy (EA) using clinical variables available in the early postoperative period.

Methods: A retrospective cohort study of SBS children managed by our Intestinal Rehabilitation Program (IRP) was completed. Data abstracted included demographic, anatomic and outcome variables including serum conjugated bilirubin, proportion of enteral nutrition (EN) and episodes of sepsis specifically at 6 months post gut loss. A univariate analysis and Cox proportional hazards (CPH) model was performed. A score predicting EA was created based on weighting of Cox model coefficients. For all analyses, an alpha-value of <0.05 was considered significant.

Results: 139 patients were analyzed (61% males). Ninety-five (68%) achieved EA. Those who achieved EA had a longer residual small bowel (75% vs 24%; p<0.0001) and colon (100% vs 75%; p<0.0001) and were less likely to have the ileocecal valve removed (26% vs 57%; p=0.0005). At 6 months, children who achieved EA had higher enteral tolerance (100% vs 30%; p<0.0001), a lower conjugated bilirubin (0 vs 71.5umol/L; p<0.0001) and less septic episodes (1.0 vs 2.0; p=0.0112). Cox proportional hazards modeling found >50% residual small bowel (HR 2.68 [95%CI 1.60-4.49], p<0.001), ICV intact (HR 0.61 [95%CI 0.37-1.02], p<0.06) and >50% enteral tolerance at 6 months (HR 5.70 [95% CI 2.77-11.74] p<0.001) were positively associated with EA. Conjugated bilirubin >34umol/L at 6 months was negatively associated with EA (HR 0.42 [95%CI 0.27-0.66], p<0.001). A severity score was created by weighting CPH parameter estimates [small bowel length >50%, ICV intact, CB<34umol/L and EN>50% for a maximum score of 8. Disease severity strata were developed (severe [0-2; 25.7% EA], moderate [3-5; 52.9% EA] and mild [6-8; 97.1% EA]. Disease severity strata were developed (severe [0-2; 9/35 (25.7%) EA], moderate [3-5; 18/34 (52.9%) EA and mild [6-8; 68/70 (97.1%) EA].

Conclusion: We propose a paediatric intestinal failure disease severity score that predicts probability of EA, stratified into mild, moderate and severe. The score allows prognostication of individual patients, and could assist research by adjusting outcome reporting or stratifying recruitment.