Introduction: The majority of children with intestinal failure due to short bowel syndrome (SBS) [dependence on parenteral nutrition (PN) for at least 90 days] are nowadays capable to be weaned from PN. Scarce evidences are available regarding the follow-up setting after intestinal adaptation. The aim of the study was to investigate the occurrence of morbidities after intestinal adaptation in children who underwent neonatal gastrointestinal (GI) surgery.
Methods: In our Home PN Centre we develop a multidisciplinary and long term follow-up to obtain a primary and secondary prevention of the unsuspected and life threatening complications after intestinal resection. Specifically, patients after intestinal adaptation enter in a follow-up program that includes dedicated staff in gastroenterology, nutrition, paediatrics, surgery, nephrology and radiology. Each patient underwent to a serial exams/clinical evaluations including stools assessment for steatorrhea and haematochezia, endoscopy, nutritional intake, urinary analysis, ultrasound and X-ray follow through of GI tract and Breath H2 test.
Results: We included in the analysis 47 children (27 males) with intestinal failure after neonatal GI surgery. The mean gestational age at birth was 32.8±5.1 weeks and the age at the last follow-up visit was: 7.8±5.8 years (0.6-24 years). Among all children 33 were weaned from PN dependence. Causes leading to SBS were: necrotizing enterocolitis (9/33), multiple bowel perforations (6/33), volvulus (5/33), intestinal aganglionosis (4/33), gastroschisis (2/33), intestinal atresia (7/33). The duration of PN of these children was 1.1±1.8 years (3 months-9 years). The length of small bowel residual was 51±39 cm (14-160 cm), 13/33 children had totally preserved the colon, whereas 16/33 had a partial residual colon. Among 33 infants weaned from PN, 42.4% developed morbidities during the follow-up period. Specifically, 3 children were identified as having anastomotic ulcers with clinical anemia, 3 children developed gallstones, 3 children developed kidney stones, 2 children had symptomatic D-lactic acidosis and 1 child developed acute pancreatitis and cholecystitis and 2 child developed venous trombosis. The occurrence of morbidities was found after 4.5±3.3 years (range 0.1-10.7 years) the weaning from PN.
Conclusions: This study suggest that the occurrence of morbidities after PN weaning is not a rare event. Therefore a multidisciplinary and long term follow-up is mandatory.