Intestinal Failure Causes

• •
  

  Anatomic loss

  
  
  
  
  • ○
    

    Intestinal resection (volvulus, NEC, gastroschisis, intestinal atresia, Crohn disease)

    
    
  • ○
    

    Intestinal bypass

  
  
  
  
• •
  

  Congenital mucosal disorders

  
  
  
  
  • ○
    

    Microvillous inclusion disease

    
    
  • ○
    

    Tufting enteropathy

  
  
  
  
• •
  

  Intestinal motility disorders

  
  
  
  
  • ○
    

    Hirschsprung disease

    
    
  • ○
    

    Intestinal pseudo-obstruction

    
    
  • ○
    

    Megacystis microcolon hypoperistaltic syndrome

  
  

Every effort should be made for intestinal rehabilitation in patients who have some preserved ileal length. In addition to nutritional and fluid rehabilitation, surgical procedures are commonly used to increase the intestinal absorptive surface and control bacterial overgrowth resulting from intestinal stasis. Such procedures include but are not limited to bowel lengthening, such as serial transverse enteroplasty and longitudinal intestinal lengthening and tailoring. Trials are ongoing with enteral growth hormones and glutamine to improve the mucosal health. In addition, control of bacterial overgrowth, avoiding infections, and minimizing lipids exposure have greatly decreased early liver failure, allowing more time for rehabilitation efforts. For some patients, however, these efforts remain temporary bridges that only help to reduce morbidity and mortality while affected patients await transplantation.

Complications of Intestinal Failure

Intestinal failure is a debilitating condition that can result in life-threatening complications. Intestinal transplantation has been a life-saving treatment of such patients. The most serious encountered complications are parenteral nutrition (PN)–associated liver disease, sepsis and loss of intravenous access.

PN liver disease or intestinal failure–associated liver disease has been reported in as many as 50% of patients with intestinal failure who received PN for more than 5 years. It is more frequently seen in infants and toddlers versus adolescents and adults. Progressive liver disease can be identified by persistent elevations in serum bilirubin for more than 3 months to 4 months. These patients are also at risk of developing other complications, such as portal hypertension, variceal bleeding, and liver failure. All efforts should be made to perform intestinal transplant in these children while the liver disease is still reversible. Once liver failure is established, survival on the transplantation wait list is greatly affected and remains at less than 50%. Recently, intravenous lipids minimization and the use of polyunsaturated lipids have helped improve the incidence of (and sometimes reverse) jaundice and halt the rapidity of liver disease progression. But once the liver fibrosis is established, patients also require liver transplantation.

Sepsis is the most common complication of PN in children less than 2 years of age. Excellent central line care, controlling bacterial overgrowth, ethanol, and antibiotics central line locks have all been helpful in decreasing its incidence. Good sepsis control has also been shown to be liver protective. Infectious complications unfortunately remain a reality for all. They are more common in patients with motility disorders and ones with primary or secondary immune deficiencies. Sepsis indications for intestinal transplant include systemic and serious infections, such as endocarditis, brain abscess, and fungal or severe bacterial infections, resulting in multiorgan failure.

In patients who are fully dependent on an intravenous source of nutrition, loss of intravenous access is clearly a life-threatening complication. It is unfortunately frequently encountered in children with intestinal failure and can be the only manifestation of a thrombophilic state.