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.

Prediction, identification and progression of histopathological liver disease activity in children with intestinal failure

“…Intestinal failure (IF) is an increasingly encountered morbid condition defined as inability of the intestine to absorb adequate amounts of fluid and nutrients, necessitating long-term parenteral nutrition (PN) for survival (1)(2)(3)(4). Patients with IF, especially young children with extensive small bowel resection, long-term PN dependency, recurrent septic infections and limited enteral intake, are at risk of developing IF associated liver disease (IFALD) (2)(3)(4)(5)(6)(7)(8).…”

“…Despite IFALD being the most prevalent significant complication of IF with the greatest risk of death, the diagnostic criteria are not well-defined and lack correlation with liver histopathology (3,4,7,9,10). Typically, IFALD is diagnosed based on a combination of elevated routine laboratory tests reflecting cholestasis and PN-dependency, while excluding other causes of liver disease (9,11).…”

European Pediatric Surgeon' Association Survey on the Management of Short-Bowel Syndrome

“…Intestinal failure (IF) occurs when there is a massive reduction of intestinal function resulting in the inability to digest and absorb adequate nutrients and fluids required for growth and development with long-term dependence on parenteral nutrition. [1][2][3] Short-bowel syndrome (SBS) is the most common cause of IF in children, and is defined by a massive loss of small bowel from either a congenital condition (e.g., intestinal atresia, gastroschisis, and long-segment Hirschsprung's disease) or an acquired lesion (e.g., necrotizing enterocolitis, midgut volvulus, trauma, and vascular thrombosis), usually during the neonatal period. 2 From the primary surgical procedure, preserving small bowel length and function should be the main concerns of the pediatric surgeon because this first surgical management will impact the patient long-term outcomes and survival.…”

“…[1][2][3] Short-bowel syndrome (SBS) is the most common cause of IF in children, and is defined by a massive loss of small bowel from either a congenital condition (e.g., intestinal atresia, gastroschisis, and long-segment Hirschsprung's disease) or an acquired lesion (e.g., necrotizing enterocolitis, midgut volvulus, trauma, and vascular thrombosis), usually during the neonatal period. 2 From the primary surgical procedure, preserving small bowel length and function should be the main concerns of the pediatric surgeon because this first surgical management will impact the patient long-term outcomes and survival. Nevertheless, because SBS is a very rare condition, surgical and nonsurgical management to facilitate enteral autonomy varies widely according to institutional experience and the existence of multidisciplinary intestinal rehabilitation program (IRP).…”

Lipid Emulsion Use in Pediatric Patients Requiring Long‐Term Parenteral Nutrition

“…Sepsis, small intestinal bacterial overgrowth, and conditions such as necrotizing enterocolitis that induce a systemic inflammatory response are closely associated with IFALD, particularly in patients with short bowel syndrome. 10,25 Endotoxin and inflammatory cytokines released by activated hepatic macrophages such as Kupffer cells induce signaling pathways that inhibit the expression and function of hepatobiliary transport mediators, resulting in cholestasis. [26][27][28] Intrahepatic accumulation of bile acids and other toxins and a persistent inflammatory state perpetuate ongoing hepatocyte injury and promote fibrosis, leading to end-stage cirrhosis.…”