Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy. Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family’s place of residence and underlying diagnosis determining IF. Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20–18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53–2.20). Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries.

Pediatric Short Bowel Syndrome (SBS) can require prolonged parenteral nutrition (PN). Over the years, SBS management has been implemented by autologous gastrointestinal reconstructive surgery (AGIR). The primary objective of the present review was to assess the effect of AGIR on weaning off PN. We also evaluated how AGIR impacts survival, the need for transplantation (Tx) and the development of liver disease (LD). We conducted a systematic literature search to identify studies published from January 1999 to the present and 947 patients were identified. PN alone was weakly associated with higher probability of weaning from PN (OR = 1.1, p = 0.03) and of surviving (OR = 1.05, p = 0.01). Adjusting for age, the probability of weaning off PN but of not surviving remained significantly associated with PN alone (OR = 1.08, p = 0.03). Finally, adjusting for age and primary diagnosis (gastroschisis), any association was lost. The prevalence of TX and LD did not differ by groups. In conclusion, in view of the low benefit in terms of intestinal adaptation and of the not negligible rate of complications (20%), a careful selection of candidates for AGIR should be required. Bowel dilation associated with failure of advancing EN and poor growth, should be criteria to refer for AGIR.

The superior mesenteric artery syndrome (SMAS) is an uncommon condition in children. We describe a case of a 7-year-old boy with SMAS that occurred 3 years after a Deloyers' procedure for subtotal colonic Hirschsprung who was admitted for bilious vomit, abdominal pain and diarrhea due to unrecognized celiac disease. This case emphasize that SMAS in children needs a close medical and surgical follow-up to avoid an underestimation of early clinical signs unrelated to surgery.

Background/Objectives: Infants with high-output enterostomies often require prolonged parenteral nutrition (PN), increasing risks of infections, liver dysfunction, and impaired growth. Mucous fistula refeeding (MFR) is proposed to enhance intestinal adaptation, weight gain, and distal bowel maturation. This systematic review and meta-analysis assessed its effectiveness, safety, and technical aspects. Methods: Following PRISMA guidelines, studies reporting MFR-related outcomes were included without data or language restrictions. Data sources included PubMed, EMBASE, CINAHL, Scopus, Web of Science, Cochrane Library, and UpToDate. Bias risk was assessed using the Joanna Briggs Institute Critical Appraisal Checklist. Meta-analysis employed random- and fixed-effects models, with outcomes reported as odds ratios (ORs) and 95% confidence interval (CI). Primary outcomes assessed were weight gain, PN duration, and complications and statistical comparisons were made between MFR and non-MFR groups. Results: Seventeen studies involving 631 infants were included; 482 received MFR and 149 did not. MFR started at 31 postoperative days and lasted for 50 days on average, using varied reinfusion methods, catheter types, and fixation strategies. MFR significantly improved weight gain (4.7 vs. 24.2 g/day, p < 0.05) and reduced PN duration (60.3 vs. 95 days, p < 0.05). Hospital and NICU stays were also shorter (160 vs. 263 days, p < 0.05; 122 vs. 200 days, p < 0.05). Cholestasis risk was lower (OR 0.151, 95% CI 0.071–0.319, p < 0.0001), while effects on bilirubin levels were inconsistent. Complications included sepsis (3.5%), intestinal perforation (0.83%), hemorrhage (0.62%), with one MFR-related death (0.22%). Conclusions: Despite MFR benefits neonatal care, its practices remain heterogeneous. Standardized protocols are required to ensure MFR safety and efficacy.