Short Bowel Syndrome in Children

What is short bowel syndrome?

Short bowel syndrome (SBS) occurs when your child’s body can’t absorb enough nutrients and fluids because part of their small intestine is missing or isn’t working as it should. SBS can be present from birth (congenital) or your child may develop it after surgery to remove a large section of their small intestine.

Another name for short bowel syndrome is short gut syndrome.

What is the small intestine?

Your small intestine is a part of your digestive system. It absorbs fluids, proteins, carbohydrates (starches and sugars), iron, fats, vitamins and minerals (such as calcium, sodium and potassium) from the foods you eat. Food breaks down into a liquid in your small intestine, and then waste from the breakdown of food passes through your large intestine.

Your small intestine has three sections:

• Duodenum: The shortest section, located next to your stomach.
• Jejunum: The middle section, located between the shortest section (duodenum) and the longest section (ileum).
• Ileum: The longest section that connects to your large intestine (colon).

How does short bowel syndrome affect the small intestine?

Changes to your small intestine can affect how much nutrients your body absorbs from the food you eat.

Some medical conditions require surgery to remove parts of a child’s small intestine. Generally, your child is born with an adequate length of small intestine as a reserve. If surgery removes your duodenum and a portion of your jejunum, your ileum can take on its role in absorbing nutrients. But if you have surgery to remove a substantial part of your jejunum or ileum, it’s hard to get adequate nutrition, as the path that food travels to break down and absorb into your body is shorter.

If your child is born with portions of their small intestine missing, it can cause complications like your child not getting enough nutrients from their meals. This can be life-threatening if left untreated.

How common is short bowel syndrome?

Short bowel syndrome is rare and affects an estimated 10,000 to 20,000 people in the United States. This estimate includes both children and adults.

What are the signs and symptoms of short bowel syndrome in children?

Signs and symptoms of short bowel syndrome include:

• Diarrhea.
• Bloating.
• Excessive gas.
• Foul-smelling stool.
• Fatigue.
• Poor growth.

Loose, watery stool or diarrhea are the most common symptoms of short bowel syndrome in infants and children.

What causes short bowel syndrome?

Just like its name, the cause of short bowel syndrome is a short bowel. The length of your small intestine affects your body’s ability to break down and absorb the foods you eat. This can affect how well your body absorbs nutrients from food and how well your small intestine functions.

Causes of short bowel syndrome in children include:

• Small intestine growth abnormalities at birth, including your child’s small intestine being too short, a section of their bowel is missing or their bowel didn’t form completely (intestinal atresia) during fetal development.
• A side effect of surgery, which removed a section of your child’s small intestine to treat an underlying condition.

Some of the most common health conditions that may require surgery to remove a portion of your child’s small intestine include:

• Necrotizing enterocolitis: Inflammation and loss of blood flow to their intestine.
• Gastroschisis: Your child’s intestines develop outside of their body before birth.
• Atresia of the small intestine: Present at birth due to multiple parts of their small bowel being extremely narrow and nonfunctional.
• Volvulus: Your child’s intestines are twisted.
• Crohn's disease: Inflammation and irritation of their digestive tract.
• Intussusception: Part of their intestine folds into another part and affects blood flow.

What are the risk factors for short bowel syndrome?

The most common risk factor for acquired short bowel syndrome is an underlying medical condition or injury that affects your child’s digestive system. For example, your child’s risk is higher if they need surgery to remove a portion of their small intestine because of atresia of the bowel.

What are the complications of short bowel syndrome?

Complications of short bowel syndrome in children can include:

• Dehydration, due to inability to maintain fluid balance.
• Malnutrition, weight loss and inability to gain weight appropriately.
• Vitamin, mineral and/or electrolyte deficiency or imbalance.
• Severe diaper rash, secondary to frequent bowel movements and acidic stools.
• Abnormal eating habits and aversion to eating.
• Liver disease, due to many reasons, though often from long-term use of much-needed IV nutrition.
• Kidney stones, from imbalances in calcium and oxalate absorption.
• Gallstones, secondary to a buildup of sludge in their gallbladder and secondary to long-term use of IV nutrition.
• Bacterial overgrowth or high levels of bacteria in their intestine.
• Food intolerances, like lactose intolerance.

Short bowel syndrome can be life-threatening if left untreated, especially if your child’s body isn’t able to absorb nutrients from the food they eat. If you notice any signs or symptoms of short bowel syndrome in your child, visit a healthcare provider.

How is short bowel syndrome diagnosed?

A healthcare provider will diagnose short bowel syndrome in your child after a complete medical and family history, an exam and testing to learn more about their symptoms and what’s causing them.

What tests diagnose short bowel syndrome?

Tests that diagnose short bowel syndrome include:

• Blood tests (complete blood count, blood chemistry, vitamin and mineral levels).
• A fecal fat test.
• An X-ray, including barium tests.
• A CT (computed tomography) scan.

How is short bowel syndrome in children treated?

Treatment for short bowel syndrome in children focuses on helping your child get the nutrients they need and relieving symptoms they experience. Treatment could include:

• Nutrition changes.
• Medications.
• Surgery.

Nutrition changes

Changes to your child’s food intake and nutrition can help treat short bowel syndrome. Children with short bowel require close monitoring of their nutritional needs. Recommendations may vary based on the severity of the short bowel and include:

• Changing what your child eats: A healthcare provider may recommend small and more frequent meals, a high-calorie meal plan that provides enough carbohydrates and protein, along with vitamins and minerals that your child needs as they grow. They may recommend taking vitamin and mineral supplements and avoiding foods high in fat, simple sugars and fiber. This prevents complications like malnutrition and dehydration and can help your child gain weight.
• Total parenteral nutrition (TPN):Your child may need total parenteral nutrition (TPN) or intravenous feeding after bowel surgery. TPN is a solution given through a vein, using special intravenous catheters called central lines (Broviac or Hickman line). It contains a mixture of fluids and nutrients, such as carbohydrates, protein, fats, sugars, and essential vitamins and minerals. It bypasses your child’s digestive system so they get the nutrients they need directly into their body. Children on TPN require very close monitoring to ensure optimal delivery of calories and nutritional components.
• Enteral nutrition:Enteral nutrition or tube feeding allows your child to get nutrients using a flexible gastric tube placed directly into their stomach, small intestine or inserted through their nose. Tube feeding is a therapy to supplement extra nutrition without eating food through their mouth or in addition to mouth feeding.

Medications

Medications can treat the symptoms of short bowel syndrome, including:

• Antibiotics to prevent small intestinal bacterial overgrowth. This may be given in cycles.
• Proton pump inhibitors and H2 blockers to reduce stomach acid.
• Bile-salt binders to lessen diarrhea.
• Growth hormones to increase nutrient absorption.
• Hypomotility agents such as Imodium® to slow the speed of food moving through their intestines.

Surgery

Your child may need surgery to treat short bowel syndrome. Your child’s healthcare provider will let you know if surgery is right for your child to help improve their intestinal function. This has to be done after careful consideration of many factors.

Surgery can:

• Increase the length of your child’s small intestine.
• Decrease the chance of bacterial overgrowth by narrowing the diameter of their intestine.
• Prevent blockages within their intestine.
• Slow down the speed of food breakdown in your child’s intestine.
• Insert a feeding tube.

In severe cases, your child may require intestinal transplantation and/or liver transplantation to replace their intestine or liver with a healthy one from a donor. Transplants may prevent major or life-threatening complications.

What is intestinal adaptation?

In some cases, children who had a part of their intestine removed by surgery undergo a process called intestinal adaptation. During this process, their intestine may grow after surgery. This is particularly beneficial in younger children, as their bowel will naturally grow until they get close to teenage years.

The surface area inside their intestine increases as the mucosa or the lining of their intestine becomes thicker and by widening of the intestinal lumen. The villi, or the lining of the intestine that’s responsible for intestinal absorption, becomes longer and denser. This helps their body absorb nutrients from food. The diameter of their intestine will also increase, and peristalsis slows down, giving more contact time for food to be absorbed.

Newer treatments like teduglutide, an injection given on a regular basis, can improve adaptation and help children come off IV nutrition and improve absorption.

Are there side effects of the treatment?

Each type of treatment comes with possible side effects, like infections after surgery. TPN is safe but needs extreme care and close monitoring to prevent complications, including liver disease. Talk to your child’s healthcare provider to learn more about the side effects of their treatment before it begins and what to look out for during treatment.

Can short bowel syndrome be prevented?

There’s no known way to prevent short bowel syndrome.

What can I expect if my child has short bowel syndrome?

Short bowel syndrome can be a lifelong condition or a short-term condition for your child. Your child’s body may improve its ability to absorb nutrients over time. If their condition does improve, they’ll still need lifelong follow-ups to monitor growth and nutritional deficiencies that may arise.

With improvements to care and treatment options and monitoring for complications, children can have a positive outlook. Your child’s prognosis and life expectancy depend on the severity of the condition. Many infants and children have a good prognosis and normal life expectancy. This varies based on the length of their intestine. Complications from the condition can be life-threatening, especially if it causes an infection, dehydration or malnutrition.

Who is on my child’s care team?

Your child’s care team for short bowel syndrome may include:

• A pediatrician.
• Pediatric gastroenterologists.
• A pediatric dietitian.
• Pediatric surgeons and/or transplant surgeons.
• Care coordinator (usually an RN).
• Pediatric social worker.

When should my child see a healthcare provider?

Visit your child’s healthcare provider if they experience symptoms of short bowel syndrome. It’s important to see their provider right away if there are complications or signs of short bowel syndrome after they had bowel surgery.

What questions should I ask my doctor?

• What caused short bowel syndrome?
• Does my child need surgery?
• What are the side effects of the treatment?
• What should my child eat and how often?