<p>Multidisciplinary Management in Pediatric Ultrashort Bowel Syndrome</p>

Abstract

Pediatric intestinal failure (IF) remains a complex and devastating condition resulting in the inability of the gastrointestinal tract to absorb adequate fluids and nutrients to sustain life. The goal in the management of IF is to achieve enteral autonomy and when not possible to avoid and minimize the development of long-term complications. Survival rates for children with IF have continued to improve resulting in an increased population of children with more altered anatomy. While IF remains a rare disease, children with IF secondary to ultrashort bowel syndrome comprise an even smaller patient population. The goal of this article is to review the recent literature related to the impact of multidisciplinary intestinal rehabilitation programs (IRPs) on the management and outcomes of intestinal failure in children with ultrashort bowel syndrome and potential avenues to further improve the long-term outcomes of this patient population.

Necrotizing Enterocolitis in a Dutch Cohort of Very Preterm Infants: Prevalence, Mortality, and Long-Term Outcomes

“…23 This discrepancy in findings of persisting IF may be attributed to our multidisciplinary management of pediatric short bowel syndrome, which is an important factor in improving outcomes for children with IF. 24 In a large recent systematic review, Jones and Hall reported severe neurodevelopmental delay in 25 to 59% of surviving NEC patients, with the highest risks among surgically treated ELBW infants. 3 In an American cohort of 28 infants with a history of severe surgical NEC (defined as residual bowel length < 30 cm), severe neurodevelopmental disability was found in 8 infants (29%).…”

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

“…9,10 Considering this, IRPs are associated not only with decreased mortality and morbidities as previously described, but also with improvement of intestinal adaptation and outcomes of autologous bowel reconstruction procedures. 8,[10][11][12][13] Thus, understanding mechanisms of intestinal adaptation is essential and is required to propose the most accurate medical and surgical strategies to each patient with SBS. 2,[14][15][16] The available approaches to potentiate intestinal adaptation include nutritional strategies, pharmacologic agents and surgery.…”

A beautiful struggle: Parent-perceived impact of short bowel syndrome on child and family wellbeing

“…Most respondents agreed it took substantial time, effort, and organization to manage their child's medical needs. Some research investigating health-related QoL for pediatric patients receiving parenteral nutrition and their parents has found child QoL to be high while parent QoL was impaired [39,40], suggesting that parents may be so committed to ensuring their child's QoL is not compromised that their own wellbeing may be negatively affected [4]. Future research should examine the mechanisms by which SBS shapes child and parent QoL.…”

“…Improved clinical outcomes associated with IRP management, including reductions in mortality and morbidity [41], higher success rates in weaning from PN [42], and reductions in the occurrence of complications including central line infections and liver disease have been described [4,8]. To the best of our knowledge, no research has been published specifically comparing child and family QoL outcomes for children under the care of IRPs with those not receiving IRP management.…”

“…Over the past several decades, considerable advances in the treatment of pediatric short bowel syndrome (SBS) have transformed the condition from terminal to a chronic disease that must be carefully managed, often with the help of intravenous or enteral feeds, specialized diets, hormonal therapies, and clinical or surgical interventions [1][2][3]. Partially responsible for this shift is the introduction of intestinal rehabilitation programs (IRPs) which specialize in providing integrated multidisciplinary care to patients with intestinal failure (IF) and are associated with improved patient outcomes, even for children with ultra-short bowel syndrome [4][5][6][7][8]. Despite this progress, a large share of neonatologists and pediatric surgeons, who are frontline clinicians in managing the care of newborns facing bowel resections, remain pessimistic about long-term quality of life (QoL) for children with SBS [9].…”