Aim: To present the results of multidisciplinary care for the children with short bowel syndrome (SBS).

Methods: A wide range of non-transplant options has been established in the Department of Pediatric Surgery at Russian Children's Clinical Hospital in Moscow. It includes home parenteral nutrition (HPN), autologous intestinal reconstructions (AIR), absorption-enhancing drug therapy etc.

57 patients with SBS aged 1 months to 17 years have been treated for the last 13 years. 40 patients underwent serial transverse enteroplasty (STEP) with our own modification, five children went through repeated STEP procedure. Spiral intestinal lengthening and tailoring (SILT) has been introduced and is considered over STEP in certain cases. Techniques of mechanical intestinal lengthening that can be applicable for SBS care are being studied. Four patients with complicated forms of SBS continue to receive GLP-2 analog - teduglutide (TED) with promising effect.

Results: Fifteenn children remain off PN 32 months after surgery. Twenty children who underwent intestinal reconstruction continue to receive reduced PN 2-4 times a week - all with reassuring growth and nutritional status. For the past year at least 20 new primary patients with SBS have been hospitalized and were introduced to HPN program. In four patients receiving TED-therapy for the past 12 months PN haven reduced by 50 to 75%. Preliminary trials with the device for distraction enterogenesis have been conducted on animal models showing promising effect that poses new solutions for extreme forms of SBS once implemented in clinical practice. Applying routine preventive therapy for all patients with SBS, we were able to minimize the number of complications such as central line-related occlusion/thrombosis, intestinal failure-associated liver disease, gallbladder sludge and stone formation, renal failure and metabolic bone disease. All fatal outcomes (8,7%, n=5) were caused by central line-related infections.

Conclusions: Pediatric SBS is a diverse clinical issue requiring coordinated multidisciplinary care.
Modern strategies for SBS management help to reduce PN, regain enteral autonomy and avoid bowel transplatation. New non-transplant options need to be investigated to find solutions for most complicated forms of SBS.