University of California San Francisco

Short Bowel Syndrome

Intestinal failure occurs when a child's intestines can't digest food and absorb the fluids, electrolytes and nutrients essential to live and to grow and develop normally. The most common cause is short bowel syndrome (SBS), a problem affecting people who have had half or more of their small intestine removed to treat conditions such as trauma, necrotizing enterocolitis, Crohn’s disease, midgut volvulus, or intestinal atresia. 

Children with intestinal failure may receive all or most of their nutrients and calories intravenously through total parenteral nutrition, or TPN. TPN is given through a central venous catheter placed in the arm, groin, neck or chest. Patients on TPN may live for many years, but long-term use of TPN can result in serious complications, such as bone disorders, catheter infections, and liver disease. At the UCSF Intestinal Rehabilitation and Transplantation Program, our goal is to restore your child's intestinal function to minimize and ultimately eliminate the need for TPN. Unfortunately, not every child can be weaned from TPN. In these cases, we work to optimize the use of TPN and decrease the risk of complications.

What causes short bowel syndrome?

Conditions that cause short bowel syndrome in children include:

How is short bowel syndrome diagnosed?

Short bowel syndrome is a clinical diagnosis and not diagnosed by any one test. When a child had gone through one or multiple operations that require removal of certain length of intestine, they may not be able to absorb adequate amount of nutrients or fluids to grow and develop normally.  The length of intestine that is needed for normal absorption depends on the portion of intestine that is removed.  This may be different for each child and depend on the condition that led to the intestinal resection.  Your child will be evaluated by pediatric gastroenterologist and surgeon.  Additional evaluation may involve:

How is short bowel syndrome treated?

As part of the intestinal rehabilitation program, comprehensive, individual treatment plan is designed to best meet your child’s needs.  The type and length of treatment differs for each case, depending on the goals for your child. Some of the treatments include:

Medical Therapy

  • Counseling and education about intestinal rehabilitation
  • Drug and diet modification to train the small intestine to absorb more nutrients
  • Consultations to help assess and correct nutrient deficiencies and prevent damage to kidneys, bones and liver
  • UCSF Benioff Children’s Hospital was one of the sites involved a clinical research study which in demonstrated safety and efficacy of Gattex® (glucagon-like peptide-2), an intestinal hormone that promotes intestinal growth and adaptation. The medication is FDA approved for treatment of SBS in children greater one year of age.

Surgery

  • Placement of catheters in veins called central venous lines allows for the administration of TPN 
  • Gastrostomy tube placement is recommended to provide some degree of continuous enteral feedings to stimulate intestinal growth. 
  • STEP (serial transverse enteroplasty) is bowel lengthening and tapering procedure that can help increase bowel length and nutrient absorption. As part of the intestinal adaptive process, the bowel can be become dilated over time.  This may lead to small bowel intestinal overgrowth syndrome and obstructive symptoms that limit the ability to advance enteral feedings.  STEP is an operation to treat this bowel dilation and has been shown to improve enteral feeding tolerance.  The decision to offer STEP procedure for your child will be made by your child’s pediatric gastroenterologist and surgeon.