Objective To characterize the natural history of intestinal failure (IF) among 14 pediatric centers during the intestinal transplantation (ITx) era. Study design The Pediatric Intestinal Failure Consortium performed a retrospective analysis of clinical and outcome data for a multi-center cohort of infants with IF. Entry criteria included infants <12 mo receiving parenteral nutrition (PN) for >60 continuous days. Enteral autonomy was defined as discontinuation of PN for >3 consecutive months. Values are presented as median (25th, 75th percentiles) or as (n, %). Results 272 infants with a gestational age of 34 wks (30, 36) and birth weight of 2.1 kg (1.2, 2.7) were followed for 25.7 mo (11.2, 40.9). Residual small bowel length in 144 patients was 41 cm (25.0, 65.5). Diagnoses were necrotizing enterocolitis (71, 26%), gastroschisis (44, 16%), atresia (27, 10%), volvulus (24, 9%), combinations of these diagnoses (46, 17%), aganglionosis (11, 4%), and other single or multiple diagnoses (48, 18%). Prescribed medications included oral antibiotics (207, 76%), H2 blockers (187, 69%), and PPIs (156, 57%). Enteral feeding approaches varied among centers; 19% of the cohort received human milk. The cohort experienced 8.9 new catheter-related blood stream infections per 1,000 catheter days. The cumulative incidences for enteral autonomy, death, and ITx were 47%, 27%, and 26%, respectively. Enteral autonomy continued into the 5th year after study entry. Conclusions Children with IF endure significant mortality and morbidity. Enteral autonomy may require years to achieve. Improved medical, nutritional, and surgical management may reduce time on PN, mortality and need for transplantation.

Objectives In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy. Study design A multicenter retrospective cohort analysis from the Pediatric Intestinal Failure Consortium (PIFCon) was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with PN dependence >60 days. Enteral autonomy was defined as PN discontinuation >3 months. Results 272 infants were followed for a median (IQR) of 33.5(16.2, 51.5) months. Enteral autonomy was achieved in 118(43%); 36(13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified NEC [OR 95% CI: 2.42 (1.33, 4.47)], care at an IF site without an associated intestinal transplant (ITx) program [OR 2.73 (1.56, 4.78)] and an intact ileocecal valve (ICV) [OR 2.80 (1.63, 4.83)] as independent risk factors for enteral autonomy. A second model (n=144) including only patients with intra-operatively measured residual small bowel length (RSB) found NEC [OR 3.44 (1.36, 8.71)], care at a non-ITx center [OR 6.56 (2.53, 16.98)] and RSB (cm) [OR 1.04 (1.02, 1.06)] to be independently associated with enteral autonomy. Conclusions A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ICV and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF.

Intestinal failure requires the placement and maintenance of a long-term central venous catheter for the provision of fluids and/or nutrients. Complications associated with this access contribute to significant morbidity and mortality, while the loss of access is an increasingly common reason for intestinal transplant referral. As more emphasis has been placed on the prevention of central line-associated bloodstream infections and new technologies have developed, care for central lines has improved; however, because care has evolved independently in local centers, care of central venous access varies significantly in this vulnerable population. The present position paper from the Intestinal Failure Special Interest Group of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) reviews current evidence and provides recommendations for central line management in children with intestinal failure.

Background and Aims Unsedated transnasal endoscopy (TNE) is safer and less costly than sedated EGD. The aim of this study was to evaluate the performance of TNE with biopsies in monitoring the esophageal mucosa of pediatric patients with eosinophilic esophagitis. Methods Patients between 8 and 17 years of age with eosinophilic esophagitis and their parents were enrolled. Unsedated TNE was performed. A 2.8-mm (1.2-mm channel) or a 4-mm flexible bronchoscope (2-mm channel) was used, and esophageal biopsy specimens were obtained. Biopsy specimen analysis, duration, adverse events, and billing charges of TNE were assessed. Immediately after TNE and a minimum of 2 weeks later, a modified Group Health Association of America 9 survey and a preference questionnaire were completed, respectively. Results Twenty-one of 22 enrolled patients underwent TNE. TNE was performed with no serious adverse events. Histopathological analysis revealed 0 eosinophils per high-power field (n = 12), fewer than 15 eosinophils per high-power field (n = 4), and more than 15 eosinophils per high-power field (n = 5). The total epithelial surface area of mucosal biopsy samples from either TNE Forceps (1.2 mm or 2 mm biopsy channel forceps) compared with those obtained during the subject’s previous EGD by using standard endoscopic forceps was not statistically different (P = .308 [1.2 mm]/P = .492 [2 mm]). All parents and 76.2% of subjects would undergo the TNE again. TNE was preferred over EGD by 85.7% of parents and 52.4% of subjects. The modified Group Health Association of America 9 survey revealed a high degree of satisfaction (average, 43.19 ± 2.6; maximum score, 45). Charges associated with TNE were 60.1% lower than for previous EGDs. Conclusions Unsedated TNE is an effective, lower-cost procedure for monitoring the esophageal mucosa of children with eosinophilic esophagitis.

Although all children had persistent aerodigestive symptoms, over 50% had not been seen by an appropriate subspecialist in the year prior to the clinic visit. The multidisciplinary evaluation resulted in new diagnoses of bronchiectasis and active reflux, which can both lead to long-term morbidity and mortality. Children with TEF require evaluation by multiple subspecialists to manage not only current symptoms but also long term risks. Ongoing care should be guided by protocols based on known risks. Pediatr Pulmonol. 2016;51:576-581. © 2015 Wiley Periodicals, Inc.