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Key Points

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  1. Due to the repair and growth capacity of the bowel, it is crucial to minimize the length of bowel resected at initial and subsequent operations.

  2. Early and consistent administration of enteral nutrition, utilizing enteral feeding access if necessary, helps maximize intestinal adaptation.

  3. Careful consideration of the location of stomas and mucous fistulae can minimize the length of the operation to reestablish intestinal continuity.

  4. Distal refeeding through a mucous fistula is a useful technique for maintaining enteral nutrition despite the presence of a very proximal stoma.

  5. Intestinal failure associated liver disease and catheter-related septic complications may be reduced with application of liver-protective feeding strategies and ethanol lock devices.

  6. Autologous intestinal reconstruction surgery should only be considered after sufficient time has passed to allow for maximal adaptation of the remnant bowel.

  7. Formal multidisciplinary intestinal failure teams can improve patient outcomes.

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Introduction

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Short bowel syndrome (SBS) is the most common cause of intestinal failure and can be the result of congenital disorders, extensive surgical resection, or both. In addition, primary defects in absorption or in peristalsis can contribute to this disorder. The end result is a remaining bowel of insufficient length and/or functional capacity to meet the fluid and/or nutritional needs of the patient without parenteral nutrition (PN) support. The causes of SBS in the pediatric population have changed over the years and are myriad. The most common is necrotizing enterocolitis (NEC), comprising some 35% of cases. Other causes include intestinal atresias, abdominal wall defects (mainly gastroschisis), intestinal volvulus, complicated meconium ileus, and the long-segment Hirschprung disease, as shown in Fig. 50-1. Apart from primary intestinal pathology, mesenteric vascular occlusion necessitating resection may occur from invasive monitoring devices and reduced perfusion states secondary to shock, as well as the use of vasoconstrictive inotropic agents that can result in an ischemic or necrotic bowel.

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Figure 50-1

The causes of short bowel syndrome.

Graphic Jump Location
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Neonatal cases comprise 80% of all pediatric SBS. Surprisingly, data on the incidence and mortality related to SBS are incomplete. The published incidences of SBS in very low and extremely low birth weight neonates at 16 tertiary centers in the United States are 0.7% and 1.1%, respectively, although this excluded cases in term infants. There is a bimodal mortality distribution in this population. The first peak corresponds mainly to infants who undergo massive initial bowel resections and the later peak represents deaths due to complications of SBS, namely central venous catheter (CVC) sepsis and intestinal failure associated liver disease. The survival rate for children with SBS has been quoted as 73% to 89% with lower rates in patients requiring chronic PN.

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General Principles

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SBS is a complex clinical problem and as such, many institutions have developed formal multidisciplinary teams specifically designed to care for these difficult patients. This approach ...

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