Short Bowel Syndrome and intestinal failure are chronic and severe conditions that may require life-long parenteral nutrition in children. Survival of these children rely on the correct functioning of central venous catheters; therefore, careful management, prevention, and treatment of complications is of paramount importance. Despite a growing awareness of preserving the vascular real estate, a certain number of patients still experience a progressive and life-threatening exhaustion of vascular access. We searched the literature to highlight the current management of children with vascular exhaustion, specifically focusing on vascular access salvage strategies and last-resource alternative routes to central veins. Given the paucity of data, results are reported in the form of a narrative review.
22 February 2023 in La Pediatria Medica e Chirurgica
Current knowledge on congenital microgastria is limited due to its extreme rarity, and the paucity of nutritional and quality of life follow-ups. Patients affected by congenital microgastria cases followed at out center were screened, and general and nutritional status were evaluated at follow-up visits through validated questionnaires. Three cases were included: one patient died because of a complex syndromic picture where microgastria was imperatively approached conservatively. The remaining cases underwent Hunt-Lawrence at 2 and 17 months. After 2 years and 27 years postoperatively, both patients are on full oral intake. The 28-yearold patient did not reach a BMI higher than 18. She rated her quality of life as unimpacted, with a Gastrointestinal Quality of Life Index of 111. In the other case, parents reported about their 2-yearold child an Infant Gastrointestinal Symptom Questionnaire of 13, corresponding to “no distress”. Our findings confirm the literature trend supporting the role of early surgery in microgastria to improve outcomes. We presented the nutritional status and quality of life in two cases of congenital microgastria operated according to Hunt-Lawrence at a 2-year and 27-year distance, which is the longest follow-up reported to date.