Preventive Treatment of Various Complications in the Management of Short Bowel Syndrome and Gastrointestinal Neuromuscular Diseases

Aim: To demonstrate the approach for prediction and management of different complications of chronic intestinal failure (IF) caused by short bowel syndrome (SBS) and gastrointestinal neuromuscular diseases (GINMD).

Methods: 58 patients with SBS and 44 with GINMDs aged 1 month to 17 years have been treated in the Department of Pediatric Surgery at Russian Children`s Clinical Hospital. We provide a full range of non-transplant options for IF management: home parenteral nutrition (HPN) program, autologous intestinal reconstructions (AIR), absorption-enhancing drug therapy etc. The most common complications included: catheter-associated bloodstream infections (CABSI) (33,3 %), invasive Candidemia (20,4 %), Intestinal Failure Associated Liver Disease (IFALD) (4,9 %), metabolic bone disease (24,5 %), small intestinal bacterial overgrowth (SIBO) and translocation (11,8 %), recurrent D-lactic acidosis (5,9 %).

Multiple measures are being made to predict and prevent these complications. Thorough training of parents on central line care and PN administration, routine use of antibacterial locks have helped to minimize CABSI. The use of new-generation lipid emulsions and choleretic agents allowed to reduce the number of IFALD. For metabolic bone disease the total serum 25(OH)D level and the level of trace elements are being monitored; parenteral Vitamin D and its active metabolites, biphosphonates and calcium/phosphorus oral supplements are prescribed in accordance with clinical presentation. A combination of pre- and probiotics with rifaximin is used to manage SIBO and recurrent D-lactic acidosis.

Results: SBS patients who underwent AIR revealed a statistically significant dynamics of a decrease in the number of catheter-associated complications, IFALD, invasive Candidemia. In patients with severe GINMDs with recurrent intestinal obstructions we put G-tube and create a T-shaped enteroenteroanastomosis which allows to improve gas evacuation and reduce SIBO.

Conclusions: patients with SBS and GINMDs require a multidisciplinary approach. Prediction and prevention of complications improve the quality of life and shows a great economic value.