Long-term outcomes after enterostomy for very early-onset inflammatory bowel disease with interleukin-10 signaling deficiency

Abstract

Background: Very early-onset inflammatory bowel disease (VEOIBD) with interleukin-10 (IL10R) signaling deficiency usually requires enterostomy in patients who are refractory to traditional treatment. This study aimed to evaluate long-term outcomes after enterostomy for VEOIBD patients with IL10R signaling deficiency. Methods: The medical records of all patients undergoing enterostomy for signaling deficiency were retrospectively assessed during 2012.1–2022.7 in a tertiary teaching hospital, Children’s Hospital of Fudan University, Shanghai, China. Data on disease history, diagnosis and detailsof enterostomy and stoma closure and follow-up were collected. Univariate and multivariate logistic regression analyses were used to evaluate the risk factors associated with the long-term outcome of delayed stoma closure. Results: A total of 46 patients underwent an enterostomy, and these patients included 19 patients who required emergency enterostomy and 27 patients who required a selective enterostomy. After ten years of follow-up, 35 patients underwent hematopoietic stem cell transplantation(HSCT), and 25 patients were alive after HSCT. The median timeframe between HSCT and stoma closure was 19.6 [15.9,26.2] months. Nineteen patients underwent stoma closure and had an average age of3.9±1.5 years, and 6 patients were waiting for stoma closure. The multivariate logistic regression analysis showed that age atHSCT was an independent predictor of a shorter time to stoma (OR 1.09; 95% CI 0.99-1.19; p = 0.06). We also found a tendency difference in multiple intestinal lesions for the time of stoma closure (OR 3.22; 95% CI 0.37-27.77; p = 0.29). Conclusions: This study determined the long-term outcomes after enterostomy for VEOIBD with interleukin-10 signaling deficiency. Early HSCT may facilitate shorter stoma closure times.