Evaluation of pediatric patients for intestinal transplantation in the modern era

ObjectivesTo review recent evaluations of pediatric patients with intestinal failure (IF) for intestinal transplantation (ITx), waiting list decisions, and outcomes of patients listed and not listed for ITx at our center.MethodsRetrospective chart review of 97 patients evaluated for ITx from January 2014 to December 2021 including data from referring institutions and protocol laboratory testing, body imaging, endoscopy, and liver biopsy in selected cases. Survival analysis used Kaplan-Meier estimates and Cox proportional hazards regression.ResultsPatients were referred almost entirely from outside institutions, one-third because of intestinal failure-associated liver disease (IFALD), two-thirds because of repeated infective and non-IFALD complications under minimally successful intestinal rehabilitation, and a single patient because of lost central vein access. The majority had short bowel syndrome (SBS). Waiting list placement was offered to 67 (69%) patients, 40 of whom for IFALD. The IFALD group was generally younger and more likely to have SBS, have received more parenteral nutrition, have demonstrated more evidence of chronic inflammation and have inferior kidney function compared to those offered ITx for non-IFALD complications and those not listed. ITx was performed in 53 patients. Superior postevaluation survival was independently associated with higher serum creatinine (hazard ratio [HR] 15.410, p = 014), whereas inferior postevaluation survival was associated with ITx (HR 0.515, p = 0.035) and higher serum fibrinogen (HR 0.994, p = 0.005).ConclusionsDespite recent improvements in IF management, IFALD remains a prominent reason for ITx referral. Complications of IF inherent to ITx candidacy influence postevaluation and post-ITx survival. What Is Known Pediatric intestinal transplants (ITx) have declined over the last 15 years especially because of the reduction in frequency of severe intestinal failure-associated liver disease (IFALD). Published indications for ITx have been widely disseminated, but the extent to which pediatric ITx centers conform with these guidelines is unclear.What Is New Progressive IFALD remains an important reason for pediatric ITx referral, particularly with short bowel syndrome, whereas threatened loss of central vein access remains unusual. Complications of intestinal failure including features of chronic inflammation and sarcopenia in patients considered for ITx may contribute to reduced survival after evaluation and ITx when performed.