Short Bowel Syndrome as the Leading Cause of Intestinal Failure in Early Life: Some Insights into the Management

Abstract

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.

Keywords: Autologous bowel reconstruction; GLP-2 analogs; Home parenteral nutrition; Intestinal failure; Intestinal failure associated liver disease; Intestinal rehabilitation centres; Intestinal transplantation; Parenteral nutrition; Serum citrulline; Short bowel syndrome.

Fig. 1. Anatomy of short bowel causing intestinal failure in childhood. Classification of short bowel syndrome according to anatomy of the remnant intestine and different causes of intestinal resection.

TIA: total intestinal aganglionosis, NEC: necrotizing enterocolitis.

Fig. 2. Consequences of SIBO. Consequences of overfeeding a dilated and poorly motile intestine leading to intestinal stasis, SIBO, mucosal injury, bacterial translocation, portal inflammation, cholestasis and fibrosis.

SIBO: small intestinal bacterial overgrowth, ETF: enteral tube feeding.

Fig. 3. Autologous bowel surgery for short bowel syndrome. The LILT procedure and the STEP procedure.

LILT: longitudinal tapering and lengthening, STEP: serial transverse enteroplasty.