Short Bowel Syndrome

What is short bowel syndrome?

Short bowel syndrome refers to a condition that can occur after some of a person's intestines are surgically removed because of a medical necessity. This condition causes digestive issues for children because the remaining intestines are not long enough to function correctly.

In children, the majority of patients with short bowel syndrome undergo surgery very early in life because they are born with abnormal intestines or acquire a serious intestinal disorder. Older children and adults may develop short bowel syndrome after surgery or trauma.

The intestines absorb fluid, electrolytes and other nutrients from the food that we eat and need for normal growth and development. Children with short bowel syndrome typically suffer from faulty absorption. The most common symptom in children is diarrhea, which is usually severe. Without proper treatment, children with short bowel syndrome may develop serious problems related to dehydration and malnutrition.

Children who are just diagnosed with short bowel syndrome or who have a severe case often require all of their nutrition to be delivered intravenously, through an IV. This is called total parenteral nutrition (TPN). TPN is a liquid delivered directly into the blood stream via a catheter that enters a major blood vessel (central venous catheter) rather than a hand IV. It contains the essential nutrients, minerals, vitamins and electrolytes for a growing child.

In some cases, children recover their digestive ability with time and support, through a process called adaptation, where the bowel regains the ability to absorb nutrients. If a child has insufficient intestinal length and function such that they require TPN for greater than 3 months, they have intestinal failure. Children with intestinal failure require TPN constantly in order to maintain adequate hydration or growth.

Infants and children with intestinal failure are at risk for complications related to their underlying intestinal disorders, as well as problems that can arise from the required TPN therapy. The most common complications of TPN therapy in children with intestinal failure include liver disease, blood stream infections and complications related to the central venous catheter.

Aside from TPN management, children with short bowel syndrome and intestinal failure need specialized nutrition. They may require nutritional formulas that are provided via feeding tubes, called gastrostomy tubes or g-tubes, to help promote adaptation and absorption.

Ultimately, the treatment goal for all children with short bowel syndrome and intestinal failure is to maintain growth and development while promoting the healing and progression of their intestinal absorption.

What causes short bowel syndrome and intestinal failure?

The most common conditions that lead to short bowel syndrome in children because they require intestinal removal are:

• Congenital defects of intestinal anatomy, such as gastroschisis, intestinal atresia, or intestinal malrotation with volvulus
• Necrotizing enterocolitis, a condition requiring intestinal surgery that most commonly affects premature infants

Short bowel syndrome is the most common reason that children develop intestinal failure. Other conditions that lead to intestinal failure in children include:

• Severe motility disorders, including chronic intestinal pseudoobstruction
• Rare congenital defects of the intestinal absorptive cells

Who gets short bowel syndrome?

In children, short bowel syndrome most commonly affects infants who are born with congenital anatomic disorders of their intestines. These disorders can only be treated with surgery that removes the affected segment of their intestines, and after the surgery the children may develop short bowel syndrome.

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Gastroenterology - Pediatric, Pediatric Transplant Hepatology

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