AIM. To compare treatment and outcomes between intestinal motility disorders (IMD) and short bowel syndrome (SBS).

METHODS. All consecutive children treated between 1984 and 2012 with < 30% of age-adjusted small intestine remaining or > 3 months duration of PN were enrolled. Patient characteristics, treatment, PN dependence, survival and risk factors of PN-dependency were assessed.

RESULTS. In total, 56 patients with median follow-up of 7.5 (IQR, 3.0-15) years were identified. Duration of PN was 21 (IQR, 7.2-62) months. SBS was underlying etiology of intestinal failure in 38 and IMD in 18. SBS patients had 43 (IQR, 25-60) cm (or 28% of age-adjusted length) small intestine remaining and 21 had ileocecal valve (ICV) present. Autologous surgery to improve intestinal function was performed more often in SBS (13, 34%) than in IMD (2, 11%). Three patients with IMD and none with SBS underwent intestinal transplantation (ITx) (P<0.05). Overall survival (89%) was comparable between the groups. Actuarial PN-dependency was markedly prolonged  in IMD, being 75% at 5 and 10 years versus 20% and 16% in SBS, respectively (P<0.001). IMD patients were less likely to wean off PN (OR 0.14, CI95% 0.05-0.39). Among SBS patients, median duration of PN was longer in those without ICV (23 vs 7.9 months, P<0.05), and length of the remaining small bowel inversely correlated with duration of PN (r=-0.417, P<0.001). In a multivariate Cox regression analysis, only length of the remaining small intestine predicted weaning off PN: those with < 35 cm of small intestine remaining were less likely to achieve intestinal autonomy (OR 0.13, CI95% 0.03-0.49).

CONCLUSIONS. IMD are major predictor of prolonged PN. Children with SBS were more often amenable to autologous intestinal reconstruction and required ITx less often than in IMD. In SBS, length of the remaining small intestine was the major predictor of PN duration.