#1 Ranked Children’s Hospital by U.S. News & World Report
MyPatients provides referring primary care providers with secure access to their patients’ information.
Boston Children's has launched the world's 1st program dedicated to offering hand transplants to children who qualify.
Innovation insider is a semi-monthly e-newsletter analyzes innovations at Boston Children’s, other academic medical centers and from industry.
Read the latest blog by a Boston Children's doctor, clinician or staff member.
There are many ways you can help children and their families get the care they need.
Below is a list of frequently asked questions regarding short bowel syndrome. Click on any one to learn more.
A person with short bowel syndrome is likely to be deficient in a range of important nutrients because their intestine cannot absorb enough nutrients from food. In this condition, known as malabsorption, the intestine tries to compensate by dilating, creating more surface area to absorb whatever nutrients it can.
Many patients with short bowel syndrome must get their nutrients delivered directly into the stomach or small intestine via a tube (a process known as enteral nutrition). In some cases this is not possible and patients must get nutrition intravenously, which is called parenteral nutrition (PN).
The extent of problems associated with short bowel syndrome usually depends on which sections of the small bowel are affected. The normal small bowel varies in length between 10 and 28 feet and is divided into three sections.
Usually, about one half of the small bowel can be removed without causing significant problems, especially if the ileum is not involved. The entire jejunum can usually be removed without problems resulting.
Removal of the ileum accounts for most of the problems seen in short bowel syndrome because it is responsible for the absorption of fats, vitamins, bile salts, and B12.
The most common causes of short bowel syndrome are:
Other causes include:
To determine whether your child has short bowel syndrome, your child's doctor will take a detailed medical history and perform a physical examination. Diagnostic tests may include a combination of the following:
Blood tests: to check the concentration of nutrients in the blood stream
Abdominal x-ray: a painless diagnostic test which uses radiation beams to produce images of internal tissues, bones, and organs onto film.
Abdominal ultrasound: a painless diagnostic imaging technique which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
Stool sample: to assess for the presence of infection or blood or also for the presence of non-absorbed sugar and protein.
Endoscopy: a test that uses a small, flexible tube that is inserted into the mouth and down the throat. Attached to the end of the tube are a light and a camera lens that are used to examine the inside of part of the digestive tract. Tissue samples from inside the digestive tract may also be taken for examination and testing. The test does not hurt but your child may feel slight discomfort. The doctor may spray a local anaesthetic into the back of the throat to numb the throat area. The test usually takes about 10-15 minutes.
Colonoscopy: a test that uses a long, flexible tube with a light and camera lens at the end (colonoscope) to examine inside the large intestine. Before the test, your child may be given a sedative to help him relax during the exam. He also may feel brief discomfort when the colonoscope is first inserted into the colon. The test usually takes about 30-45 minutes.
Indirect calorimetry: a test to analyze the energy (caloric) needs of your child.
Breath hydrogen analysis: a test to measure the degree of malabsorption of carbohydrates or sugars as well as to determine whether excess quantities of bacteria are growing inside of the bowel.
Treatment for short-bowel syndrome consists primarily of nutritional management aimed at helping the remaining intestines adapt and take on the absorption functions that were lost.
Some children may be candidates for the STEP procedure, a surgical intervention.
Regardless of the treatment approach, which is always individualized for the child, multidisciplinary care, which integrates medical, surgical and nutritional management from various specialists in each, has been shown to improve outcomes.
Medicines used in the management of SBS include:
Surgery may be necessary in cases where there is an obstruction in the intestines or in cases of extreme shortening where absorption can be enhanced by operative techniques. Such techniques enhance the surface area of the intestines or prolong the transit time which enhances the exposure of the intestine to the nutrients.
In 2002, Boston Children's doctors performed the world's first serial transverse enteroplasty (STEP) procedure, a surgical technique developed by Tom Jaksic, MD and Heung Bae Kim, MD, which lengthens the bowels of children with short bowel syndrome. Since then, they have performed seven of these procedures with increasing success, establishing Boston Children's Hospital as one of the preeminent hospitals in the world for the treatment of short bowel syndrome.
Read more about this innovative procedure.
It may take months to weeks to possibly years for a patient to progress from feeding enterally to oral feeding as the intestine adapts.
The prognosis for children with short bowel syndrome varies. A lot depends on the length and function of the child's bowel as well as the health of other organs that assist with digestion and absorption.
Certain patients may require lifelong nutritional support depending on the length and health of the residual small bowel. However, many infants with intestinal surgery are able to be weaned from parenteral nutrition within the first days to weeks of their operation.
One common definition of SBS is a dependence on PN for more than 90 days after surgery. Using this definition, we recently determined that the overall mortality of infants with SBS is approximately 30%.
Prolonged use of PN has been associated with a wide range of liver abnormalities, including elevated blood liver enzymes, jaundice, or rarely, progression to liver scarring and liver failure.
The majority of children with blood abnormalities and/or jaundice have improvement of their liver function if PN can be safely discontinued and enteral nutrition advanced. Once liver damage is advanced, however, this may be irreversible.
Treatment of this liver disease may include liver transplantation, or in some cases, transplantation of liver and small intestine as well as other organs.
The signs and symptoms of short bowel syndrome are:
The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”