Is Short Bowel Syndrome Fatal?

Short bowel syndrome is a rare disease that develops when a major section of the small intestine is missing, damaged, or surgically removed, which causes the body to be unable to absorb nutrients to support life.

The severity of short bowel syndrome varies depending on the extent of the intestinal damage and the remaining functional part of the bowel. Short bowel syndrome is a combination of disorders associated with inadequate nutrition absorption and is classified as a malabsorption disorder.

Acquired short bowel syndrome

• Surgical resection of the bowel: The most common cause of short bowel syndrome is surgical removal (resection) of a part of the small intestine due to malformation or diseases such as a tumor, cancer, volvulus, or mesenteric infarction caused by decreased blood flow to the bowel.
• Malfunction of the small intestine: Short bowel syndrome can be caused by any disease, injury, or condition that impairs or inhibits the functions of the small intestine, even if the total length of the colon is unaltered. In intestinal pseudo-obstruction, the gut fails to flow food and liquids normally down its length, compromising food absorption and causing short bowel syndrome.

Congenital conditions (seen in a newborn)

• Malformation of the intestine: Inability of the gut to develop normally
• Crohn's disease: Causes inflammation of the gut wall and hampers the absorption of dietary nutrients if the small intestine is damaged
• Necrotizing enterocolitis: Occurs for unknown reasons and affects infants, particularly preterm neonates (the intestinal lining dies as the blood flow to the intestinal walls is reduced, and the dead tissue must be surgically removed; about one-third of newborns with this disease will die)

Symptoms of short bowel syndrome vary from person to person, as the severity of the disease depends on the length of the bowel affected and the reduced functionality of the bowel.

Short bowel syndrome symptoms may include:

• Vomiting
• Diarrhea
• Abdominal pain
• Bacterial infection
• Bowel obstruction
• Bloating
• Cramping
• Flatulence (intestinal gas)
• Fatigue
• Weakness
• Dehydration
• Weight loss
• Food sensitivities
• Heartburn
• Anemia (low blood counts)
• Poor blood clotting
• Steatorrhea (oily or foul-smelling stool)
• Osteoporosis (thin or fragile bones) and bone pain
• Malnutrition
• Hyperkeratosis
• Bruising
• Muscle spasms
• Kidney stones
• Electrolyte imbalances

To diagnose short bowel syndrome, a detailed medical history will be taken and a physical examination conducted. The following tests may be ordered to determine structural and functional abnormalities:

Radiological tests

• Abdominal X-ray: Done with contrast dye to check for structural abnormalities of the bowel
• Abdominal ultrasound: Evaluates abdominal structures, movements, and blood flow
• Endoscopic examination: Involves inserting an endoscope (a tube with a camera) through the mouth to assess the insides of the intestine; a small sample of the tissue is collected for lab analysis
• Colonoscopy: Involves inserting a colonoscope (a tube with a camera) into the rectum to examine the large intestines; tissue samples are collected for further analysis

Nutrition tests

• Indirect calorimetry: Analyzes a child's dietary needs in the form of calories, carbs, protein, and fat body composition
• Breath hydrogen analysis: Assesses the degree of carb or sugar malabsorption and evaluates whether excessive amounts of bacteria are developing within the gut
• FibroScan: Specialized ultrasound technology that assesses liver stiffness (hardness) and fatty changes
• Other tests: DEXA scans, bone density motility testing, gastrointestinal function tests

Dietary modifications

Following intestinal resection, the patient will initially require the administration of fluids, electrolytes, and liquid nutrients into the circulation intravenously; this procedure is known as total parenteral nutrition (TPN) or parenteral nutrition. 

Two processes that aid in gut adaptation include enteral and oral feeding:

• Enteral nutrition involves delivering a liquid meal combination into the stomach or small intestine through a feeding tube. This process promotes intestinal villi adaptability. For this to work, the patient must have a partially functional gastrointestinal tract. To compensate for the loss of the gut, the remaining intestinal villi expand in length and thickness, increasing the remaining surface area for nutritional absorption.
• Some patients can start an oral diet after a few weeks of surgery, depending on the degree of the resection. Others may require up to a year of adaptation. Food and eating habits may be able to help a person avoid malnutrition and dehydration once they are able to eat orally. Dietitians will modify diets based on which parts of the remaining gut are functional.

Although oral nutrition is favored, some people may not obtain enough nourishment from food alone. In severe situations, even enteral feeding may not be effective, and TPN may be required on a long-term basis. However, this is a difficult type of therapy with the possibility of major consequences such as central line infections, bone disease, and liver disease.

Medications

In addition to dietary support, medications may be prescribed to help with short bowel syndrome management, such as medications to help:

• Regulate stomach acid
• Reduce diarrhea
• Improve intestinal absorption following surgery
• Manage pain

Surgery

If all other treatment options have been exhausted for children and adults with short bowel syndrome, doctors may consider the following:

• Surgery to improve the flow of nutrients through the gut
• Autologous gastrointestinal reconstruction to expand the intestine
• Small bowel transplantation

The method and level of invasiveness of the surgery will determine post-surgery prognosis and rehabilitation.

In some cases, an intestine transplant is required, which involves replacing damaged or missing tissue with healthy small intestinal tissue. The healthy tissue may come from a recently deceased person or a living donor. If a person has an intestinal failure, this can save their life.