What is Short Gut Syndrome?
Short Gut Syndrome, or Short Bowel Syndrome (SBS), is a condition in which the bowel is shorter than normal, typically 50 percent or less. As a result, the bowel does not have the necessary surface area to absorb the water, vitamins, and other nutrients necessary to sustain life.
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Why does the small intestine matter?
Located between the stomach and large intestine, the small intestine does the majority of the work of digesting food and absorbing nutrients. It is made up of three sections, each of which performs a specific function and absorbs specific nutrients. When large portions of the small intestine are missing or not functioning properly, the body can no longer adequately absorb water, vitamins or other nutrients.
What causes Short Gut Syndrome?
Short Gut Syndrome usually follows surgical intervention to remove damaged or dead intestinal tissue. In rare cases, it can also be congenital. Although the body adapts to bowel loss, the body cannot regenerate intestinal length. Segments that are lost do not regenerate. Below is a list of common conditions that can lead to Short Gut.
- Necrotizing enterocolitis, or NEC, a bacterial infection, especially common in premature infants, resulting in damage to and death of intestinal tissue. This is the leading cause of SBS in children.
- Gastroschisis, A birth defect in which the small intestine develops outside of the body because of a hole in the abdominal wall.
Vanishing gastroschisis is a rare complication of gastroschisis in which there is a volvulus of the intestine in the womb, resulting in the intestine losing blood supply, dying and being reabsorbed. In some cases, the abdominal wall will close, leaving no outward evidence of the defect.
- Omphalocele, a birth defect in which various internal organs develop outside of the body, usually enclosed in a semi-transparent sac.
- Intestinal atresia, a birth defect characterized by one or several narrow spots in the small intestine.
- Midgut volvulus, a twisting or tangling of the small intestine that results in a loss of blood supply and death of intestinal tissue.
- Cancer, or complications of cancer treatments
- Crohn's disease, an auto-immune disease affecting the digestive tract
- Intussusception, a condition in which a portion of the intestine folds into itself, like a telescope, causing obstruction.
- Hirschprung's disease, a disease affecting the nerves in the colon. In rare cases, this can extend to the small intestine.
- Meconium ileus, when the meconium, a baby's first stool, is too think and causes an obstruction.
- Hernia, when the small intestine is displaced through a weak point in the abdominal wall.
- Surgical complications.
- Trauma, such as a car accident.
- Vascular injury or disease, causing impaired blood flow to the intestine.
- Adhesions, a form of scar tissue that can cause blockages that require surgical intervention.
- Radiation damage.
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What are the symptoms of Short Gut Syndrome?
Symptoms of Short Gut Syndrome generally are related to nutrition, hydration, and digestive function. The manifestation of these symptoms will vary, however, depending on what sections of intestine are missing, how much is missing, and how the remaining intestine functions.
- Chronic diarrhea is the most common complication. Short Gut results in a faster digestive transit time and less absorptive surface.Patients may experience skin breakdown or diaper rash as a result of the high acidity and frequency of bowel movements. In some cases, this can be severe.
- Other digestive upset including abdominal pain, nausea, vomiting, heartburn, gassiness, etc.
- Malnutrition can result from poor absorption of the ability to absorb nutrients because the anatomy required for absorption is missing. Malnutrition can lead to weight loss and/or poor growth. Patients may struggle with vitamin and mineral deficiencies which can cause anemia,and easy bruising, affect skin and hair, and lead to bone loss.
- Dehydration caused by chronic diarrhea is very common. Signs of severe dehydration include, thirst; dark-colored urine; decreased urination; lethargy, dizziness or faintness; dry skin; mild fever. Short Gut patients may need to be hospitalized to receive IV support for hydration, especially during illness. Proper diet management, such a limiting intake of sugars, fruits and dairy can help to reduce fluid losses that can cause dehydration. Also, drinking an oral rehydration solution that is formulated with a proper balance of sugars and electrolytes increases absorption of fluid and is preferential to drinking water for many SBS patients. Electrolyte imbalances are common due to chronic diarrhea and poor absorption, as well, especially in patients who are missing some of all of their colon.
- Motility problems can result from Short Bowel Syndrome itself or from the disease or injury that caused bowel loss. Usually, patients with Short Bowel Syndrome will experience rapid digestive transit time. However, there may be structural problems such as adhesions, stricture, or scar tissue. There also may be functional problems with the nerves or muscles that affect peristalsis (the wavelike motion that moves food through the digestive tract.) Ileus, known sometimes as pseudo-obstruction, is a paralysis of these movements. Ileus is usually temporary, but may recur or require intervention to resolve. Illness and infection often cause temporary ileus for Short Gut patients. Other motiliy problems may be chronic. Symptoms associated with motility issues include constipation, abdominal distention, abdominal pain, and bacterial overgrowth.
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What are possible complications of Short Gut Syndrome?
- Bacterial overgrowth, sometimes abbreviated SBBO or SIBO, is common in patients with short gut for a few reasons. As the intestine adapts, it may dilate, or stretch. Dilation can lead to poor motility, and poor motility can increase dilation. IIn any case, these problems can allow intestinal contents to pool and ferment. If the ileocecal valve (ICV), which is the valve between the small and large intestine, is missing, bacteria from the colon may backwash into the small intestine, complicating the problem. Antibiotics may be used to treat infections related to IV therapy or even attempts to treat bacterial overgrowth, which can kill beneficial bacteria in the gut. As patients are exposed to more resistant strains of bacteria in the hospital setting, bad bacteria can be introduced. In the end, the bad bacteria can overpower the good bacteria in the gut. This can cause symptoms like gassiness, foul-smelling stools or breath, abdominal pain, loss of appetite, and increased stool output.
- Sepsis is a life-threatening infection caused by a bacterial or fungal infection in the bloodstream. Many short gut patients rely on a form of intravenous nutrition called total parenteral nutrition (TPN) that is given via a central line, an IV catheter that runs directly into the heart. This catheter can become infected either because of external contamination, such as from poor sterile technique, from a tunnel infectionin tunneled catheters, or when bacteria from the gut leaks into the bloodstream, a problem known astranslocation. This is known as central line-associated bloodstream infection (CLABSI). If the infection spreads outside of the line, sepsis develops. The symptoms of sepsis can include fever; chills; cold or clammy skin; rigors (shivering or tremors); fast heart beat; change in mental state; slurred speech; severe muscle pain; feeling dizzy or faint; nausea, vomiting, and diarrhea; or increased stool output. Because sepsis can kill if not treated quickly, patients with a central line are advised to go to the emergency room with every fever or any other concerning symptoms. They will generally be admitted to the hospital and given broad-spectrum IV antibiotics with every fever or with these other symptoms. Importantly, translocation is possible even without a central line. The resulting bacteremiacan cause similar symptoms and even lead to sepsis.
- Loss of central venous access can make it impossible for patients with SBS to be fed. There are only eight central veins that are wide enough and direct enough to fit a central line through them to reach the heart. As these veins become clotted, scarred, or otherwise unusable, it becomes more and more difficult to place new central lines. Eventually, it may become impossible to place a traditional central line in one of these eight veins. In this case, doctors will need to use a special procedure to reopen a vein or use a non-central vein to reach the heart. If doctors cannot restore access, the patient may have to go off TPN, which can lead to starvation if the patient cannot be fed another way. Lost access may also prevent other treatments and surgeries, including transplant.
Some causes of lost central access include venous thrombosis, or inflammation within the veins; scarring of the blood vessels; stenosis, or narrowing of the blood vessels; blood clots, including fibrin sheaths, which are long, threadlike clots at the end of a central venous catheter; catheter infections, bloodstream infections, and sepsis. Central venous access loss can be hard to spot, but some warning signs are difficulty or pain flushing or withdrawing from a central line; reports of difficulty placing a central line; and significant collateral blood vessels, which are blood vessels where the body has rerouted blood flow around an occluded vein. Though not always visible, sometimes collateral vessels may look like thin, blue lines on the skin that resemble a road map.
The superior vena cava at the entry to the heart, through which all upper central lines pass, can also become blocked, resulting in superior vena cava syndrome, which is characterized by facial swelling; and swelling of the upper body. SVC syndrome can be life-threatening and requires immediate treatment.
Evidence-Based Strategies and Recommendations for Preservation of Central Venous Access in Children https://aspenjournals.onlinelibrary.wiley.com/doi/abs/10.1002/jpen.1591
- Liver Disease has long been known to be a side effect of Total Parental Nutrition (TPN). Intestinal failure-associated liver disease (IFALD) describes the liver damage caused by a combination of factors associated with intestinal failure, such as SBS. Although the causes are not entirely understood, liver disease has been associated with long-term TPN use, repeated sepsis, malnutrition, lack of oral or enteral nutrition, and malabsorption of digestive bile acid. Symptoms of liver disease may include yellow skin and eyes (jaundice); enlarged liver and/or spleen; fluid retention; and bruising or bleeding. IFALD associated disorders include fatty liver disease (steatosis), liver inflammation (cholestasis), and gallstones or sludge. The disease can progress to fibrosis, cirrhosis, and end stage liver disease. Changes in the management of TPN, particularly lipids and dextrose, have been shown to help in the prevention and management of liver disease.
Read more: Liver Disease Secondary to Intestinal Failure. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3914483/
- Oral aversion and other feeding difficulties are common in patients with SBS. Oral aversion especially affects children or those who have had short gut since childhood. Because of digestive upset and limited chances to eat and develop oral motor skills, patients may become fearful or defensive about putting foods in their mouths. This may look like picky eating, but it is a much more complex psychological, developmental, and motor issue. Feeding therapy and counseling may be needed. Oral aversion often goes hand in hand with oral sensory-seeking behaviors like chewing on non-food objects. Some patients will seek out strong smells or flavors. Some patients may even develop pica, a mental health condition where the person compulsively swallows non-food objects.
Read more: Oral Feeding Difficulties in Children With Short Bowel Syndrome: A Narrative Review https://pubmed.ncbi.nlm.nih.gov/28521656/
- Food sensitivity and food allergy can be caused by SBS. When the body incorrectly absorbs food, a problem known as malabsorption, the body may see the proteins in the food as threats, causing an immune response. Symptoms of food sensitivity involve stomach upset, diarrhea, and vomiting. Symptoms of food allergy include itching; hives, eczema; swelling of the face, lips, or tongue; nasal congestion; difficulty breathing; abdominal pain, nausea, diarrhea, vomiting; dizziness or lightheadedness, fainting; and anaphylaxis. Short gut patients also have a higher than average risk of eosinophilic disease, which can cause trouble swallowing, heartburn, and chest pain in addition to the above food sensitivity symptoms.
Read more: High Prevalence of Eosinophilic Gastrointestinal Disease in Children With Intestinal Failure https://pubmed.ncbi.nlm.nih.gov/27548247/
- Kidney stones are more common in SBS when the body does not absorb fat correctly. The fat that is not absorbed binds to calcium in the small intestine, leaving too little calcium to bind to a compound known as oxalate. If the oxalate that is left behind is then absorbed in the large intestine, it can travel to the kidney, where it finds and binds to calcium and causes stones. Eating foods high in oxalates can increase the risk of kidney stones for SBS patients who have some remaining colon. Symptoms can include severe, sharp pain in the side of the back, below the ribs, that comes in waves; pain urinating; decreased urination.
Read more: Low Oxalate Diet in Short Bowel Syndrome
Kidney stones are common in patients with short-bowel syndrome receiving long-term parenteral nutrition: A predictive model for urolithiasis https://pubmed.ncbi.nlm.nih.gov/33938015/
- Ulcers are open sores in the digestive tract that can cause abdominal pain and gastrointestinal bleeding. These can be peptic ulcers, caused by too much stomach acid; anastomotic ulcers, or ulcers that form where the intestine has been connected surgically; or staple-line ulcers along the internal scars from surgeries step procedure. Because these ulcers can cause a sudden, rapid loss of blood, they can be life-threatening. It is believed that certain ischemic bowel injuries, such as necrotizing enterocolitis or gastroschisis may put some patients at greater risk of ulcers.
- D-Lactic Acidosis (D-La) is a rare but life-threatening neurologic syndrome that happens when there is too much acid in the blood and tissue. This problem starts when poor digestion leaves foods high in carbs in the intestine where they ferment. Bacteria in the gut are supposed to break down food, but SBS patients can have unusually high numbers of some uncommon bacteria in their intestines. Some of these bacteria release a product called D-Lactic acid as they break down food. Given plenty of carbohydrates to feed on, these bacteria can produce high levels of this acid that change the PH balance of the blood, affecting the brain and nervous system. Symptoms include slurred speech, confusion, delirium, loss of coordination (ataxia), and sleepiness. Patients are often described as appearing drunk. Unless treated, it can lead to encephalopathy, coma, respiratory failure, and death. This condition is especially common in patients who are missing large portions of small intestine but still have a large intestine.
Read more: Oley Foundation Newsletter: D-Lactic Acidosis https://oley.org/general/custom.asp?page=DLacticAcidosis
D-Lactic Acidosis: An Underrecognized Complication of Short Bowel Syndrome https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4421027/
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