Evaluation of the prognostic factors for pediatric intestinal motility disorders: A single institution experience

Purpose: To improve the life prognosis of patients with intestinal motility disorder (IMD), we explored predictors of survival. Methods We reviewed the medical records of IMD patients who required total parenteral nutrition (TPN) for four weeks or more at our institution from April 1984 to November 2021. We retrospectively evaluated the characteristics of patients with IMD as predictive factors. Results Fourteen patients were enrolled. They had high mortality rate (50%). The types of IMD were as follows: hypoganglionosis, extensive aganglionosis, and chronic idiopathic intestinal pseudo-obstruction. The mortality did not differ to a statistically significant extent among patients with three types of IMD. A significant difference was observed in the length of enterostomy between survivors and the non-survivors ( p  = 0.028). Cholestasis was a significant prognostic factor ( p  = 0.005). Intestinal failure associated liver disease (IFALD) was the cause of death in the short term. Catheter-related blood stream infection (CRBSI) and acute rejection after small bowel transplantation were causes of death in the long term. Conclusion IMD patients still have a high mortality rate and cholestasis predicts survival in IMD patients. Preventing or improving IFALD and CRBSI due to long-term TPN is important for reducing the mortality rate.