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Short Bowel Syndrome in Children

Short bowel syndrome is a condition in which the body cannot absorb enough fluids and nutrients because part of the small intestine is missing (usually due to prior surgery or illness), or is not working properly.

What does the small intestine do?

The small intestine is a part of the digestive system. The small intestine has three sections:

  • The duodenum, which is located next to the stomach (shortest section)
  • The jejunum, which lies between the duodenum and the ileum
  • The ileum, which is the longest section and connects to the large intestine (colon)

(The ileocecal valve forms a barrier between the ileum and the large intestine to prevent the contents of the large intestine from flowing back into the small intestine.)

The small intestine is where the absorption of fluids, proteins, carbohydrates (starches and sugars), iron, fats, vitamins, and minerals (such as calcium, sodium, and potassium) takes place. If the duodenum and a portion of the jejunum have been removed by surgery, the ileum can take on their role in absorbing nutrients. But if a substantial part of the jejunum or the ileum is removed, it is more difficult to obtain adequate nutrition. In these cases, nutrients usually have to be provided in a form other than food.

Children need more calories than adults because they are still growing. If a child is born with portions of the small intestine missing, it can lead to serious problems.

What causes short bowel syndrome?

Short bowel syndrome can occur as a congenital (present at birth) condition. For example, the small intestine might be abnormally short at birth, a section of the bowel might be missing, or the bowel does not form completely before birth (intestinal atresia).

In other cases, patients develop conditions in which a large section of the small intestine has to be removed by surgery. In newborns, especially premature infants, necrotizing enterocolitis (the inflammation and loss of blood flow to the intestine, leading to severe damage) is the most common cause of short bowel syndrome.

Other causes include:

  • Crohn's disease (the intestine becomes inflamed and scarred); in this condition SBS occurs primarily in patients who have undergone extensive surgery to the small bowel
  • Intussusception (part of the intestine is folded into another part and compromises the blood flow to the involved portion of the intestine)
  • Injury to the intestine due to:
    • volvulus (twisting of the intestine)
    • trauma (injury)
    • gastroschisis (when the intestines develop outside the body prior to birth)
    • narrowing or obstruction of the intestines
    • tumors
    • blood clots or abnormal blood flow (ischemia) affecting the circulation to the intestine

What are the symptoms of short bowel syndrome?

Symptoms of short bowel syndrome include:

  • Diarrhea. Watery diarrhea is the most common symptom of short bowel syndrome in infants and children.
  • Bloating
  • Excessive gas and/or foul-smelling stool
  • Poor appetite
  • Weight loss or inability to gain weight
  • Fatigue
  • Vomiting

Other complications can occur as a result of short bowel syndrome, including:

  • Dehydration
  • Vitamin, mineral, and/or electrolyte shortage or imbalance
  • Malnutrition
  • Severe diaper rash caused by frequent diarrhea
  • Abnormal eating habits
  • Kidney stones or gallstones caused by abnormal calcium or bile absorption
  • Bacterial overgrowth (high levels of bacteria in the intestine)

How is short bowel syndrome treated?

A variety of treatments may be required in order to treat short bowel syndrome. The patient has to change his or her diet in order to be able to absorb nutrients correctly. If a patient has had surgery to remove part of the small intestine, it is important to maintain a normal balance of electrolytes, fluids, and other nutrients to prevent dehydration, malnutrition, and other problems.

The patient may need total parenteral nutrition (TPN) after bowel surgery. TPN is a method of providing nourishment while bypassing the digestive system. TPN solutions contain a mixture of fluids and nutrients, such as protein, fats, sugars, and essential vitamins and minerals. The solutions are given intravenously (through a large vein into which a catheter, a flexible plastic tube, has been inserted.). TPN is given over 10 to 12 hours or sometimes longer; infants and children usually receive this type of solution while sleeping.

Some children must remain on TPN indefinitely. Serious complications can occur when this form of nutrition is used over the long term, such as infection at the site where the catheter is inserted, formation of blood clots, and liver damage.

Despite the risk of complications, TPN can be lifesaving in children and adults unable to take in appropriate nutrition through their gastrointestinal tract. In addition, recent changes in TPN regimens, when combined with starting feeds early on, may decrease the chance of developing long-term liver injury.

Over time, enteral nutrition can replace TPN in some patients. Enteral feeding is given through a gastric tube (g-tube) inserted in the stomach via a surgical incision, or placed using an endoscope. In some cases a nasogastric (NG) tube that passes from the nose into the stomach might be used instead of the g-tube. In other patients, use of a similar tube placed in the small intestine (jejunostomy tube or j-tube) is an alternative.

Some children may be able to receive small amounts of solid food and liquids in addition to enteral or parenteral (intravenous) feeding. This helps to allow babies and children to maintain the ability to chew and suck and helps them develop normal eating patterns in the future.

In some cases, patients who have had a part of their intestine removed by surgery undergo a process called intestinal adaptation. During intestinal adaptation, the intestine may grow in size after surgery. The surface area inside the intestine increases as the mucosa (lining of the intestine) becomes thicker. The villi (the lining of the intestine responsible for intestinal absorption) become longer and denser, helping to promote absorption of nutrients. The diameter of the intestine may also increase.

What medications are prescribed for short bowel syndrome?

Medications may be used to help slow the passage of food through the intestine. This allows more time for the nutrients to remain in contact with the cells lining the intestine, which improves absorption.

Anti-diarrhea drugs such as loperamide hydrochloride can be given to children, if recommended by their physician, with limited side effects. Since the stomach is likely to secrete greater amounts of acid during the recovery period, patients can take antacids or an anti-ulcer medication to treat or reduce the risk of stomach ulcers. Antibiotics may be prescribed on occasion to prevent or treat bacterial overgrowth.

Newer medications like Teduglutide, a medication given via injection have been approved in adults with SBS but not in children by FDA. These medications can help reduce TPN requirement in some SBS patients, but will require study in children and have potential significant side effects.

When is surgery needed for short bowel syndrome?

Surgical options can be explored in certain situations in SBS.

In some cases surgery can be done to improve the functional length of intestine by various procedures such as Bianchi procedure and serial transverse enteroplasty procedure (STEP). These procedures may also help decrease the chance of bacterial overgrowth by narrowing the diameter of the intestine which may be increased in cases of SBS. The decision if surgery will help improve an individual patient’s functional status is made on a case by case basis and in many cases is dependent on the length of small intestine that is now present as well as the underlying cause of the SBS.

Intestinal transplantation involves placing a donor small intestine into the patient. This may be considered for patients who cannot use their intestine to absorb food and fluids, are entirely dependent on TPN and are at risk of losing access sites for intravenous nutrition. Liver transplantation may be required at the same time, if patients develop irreversible liver disease from long term TPN use. Since very few centers perform intestinal transplant, an early referral to a transplant center helps in planning the transplant prior to the development of major complications.

What is the prognosis (outlook) for patients who have short bowel syndrome?

The prognosis for infants and children who have short bowel syndrome can be good, depending on the residual length of the intestine. However, they will need lifelong follow-up care. Children need to be closely monitored for any nutritional deficiencies or other conditions that may result from continued use of enteral or parenteral nutrition. Overall, quality of life and chance of coming off TPN have improved with better understanding of the problem and preventing complications.

The main causes of death among infants and children who receive parenteral nutrition are infections and disorders of the liver and biliary tract (the pathway by which bile flows from the liver to the small intestine). Infectious complications may become less frequent with recent advances in protocols for the care of central lines in children. Cholestasis (a condition where bile cannot flow from the liver to the duodenum) is a common complication following long-term use of total parenteral nutrition. Newer approaches to managing TPN have helped to decrease the chance of cholestasis and associated liver disease.

A team approach involving pediatric gastroenterologists, pediatric dietitians, pharmacists, pediatric surgeons and transplant surgeons, and social workers is vital in managing and improving outcomes in patients with short bowel syndrome.

References

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 1/20/2015…#14725