Short Bowel Syndrome in Children Causes, Symptoms, Treatment

Short Bowel Syndrome in Children Causes, Symptoms, Treatment

What is short bowel syndrome?

Short bowel syndrome is a group of problems affecting people who have had half or more of their small intestine removed. The most common reason for removing part of the small intestine is to treat Crohn's disease.

Diarrhea is the main symptom of short bowel syndrome. Other symptoms include cramping, bloating, and heartburn. Many people with short bowel syndrome are malnourished because their remaining small intestine is unable to absorb enough water, vitamins, and other nutrients from food. They may also become dehydrated, which can be life threatening. Problems associated with dehydration and malnutrition include weakness, fatigue, depression, weight loss, bacterial infections, and food sensitivities.

Short bowel syndrome is treated through changes in diet, intravenous feeding, vitamin and mineral supplements, and medicine to relieve symptoms.

Normal Physiology

The small intestine is completely formed by 20 weeks' gestation. Most of its growth prior to birth occurs in the third trimester. Before 27 weeks' gestation, the average length of the small intestine is 115 cm. This length increases to approximately 250 cm with a diameter of 1.5 cm after 35 weeks' gestation. In contrast, the adult intestine is 600 to 800 cm in length and 4 cm in diameter. The mucosal surface area increases with age. Infants have 950 square cm; adults have 7500 square cm.

The intestine has an enormous capacity to absorb secretions and ingested fluids. There is extra intestine normally which is why a major loss of the intestine may not result in SBS. Absorption occurs through the lining (mucosa) of the small intestine. Nutrients, vitamin B12, calcium, iron, and bile acids are absorbed through the cells of this lining. Mucus covers the surface of the mucosa cells and acts as a trap to hold nutrients in contact with the cell surface. Mucus also acts as a bacterial barrier.

Causes of Short Bowel Syndrome in Children

Children develop SBS because they have had a very large segment of their bowel removed. Usually this happens because they were born with a condition that damaged their small bowel, such as gastroschisis.
Short Bowel Syndrome can also occur in children because their bowel didn’t form completely before birth (intestinal atresia). They may have a segment of bowel missing, or doctors may have removed a segment that was closed off from the rest of the bowel.

Children can also develop Short Bowel Syndrome because of conditions that begin after birth. These include necrotizing enterocolitis, volvulus (twisting of the intestines) or trauma (injury) to their bowel.

Rarely, children may have abnormal nerves throughout their intestine. Their small intestine may be a normal length but not work normally. These children may have intestinal pseudo-obstruction or long-segment Hirschsprung’s disease.

Short Bowel Syndrome is the most common cause of intestinal failure.

What are the symptoms of Short Bowel Syndrome in Children?

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 in Children 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.

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.

When is surgery needed for short bowel syndrome?

In cases where short bowel syndrome cannot be treated with medications and diet, surgery may be necessary.

Because the small intestine is usually much longer than is required to digest and absorb food, removing part of the intestine might not cause serious problems. The remaining portion of the intestine can adapt and assume some of the functions of the part that is missing, especially if the ileum is still intact.

Other conditions that may require surgery include:

  • 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
Intestinal transplantation, in which a new small intestine is transplanted into the patient, may be considered for patients who have frequent, severe bouts of diarrhea or other more serious complications of short bowel syndrome.