Short Gut Syndrome

Short Gut SyndromeSometimes it seems like the intestines are connected to every organ system as children present with abdominal complaints related to so many conditions. These can be intra-abdominal issues (ex, CRAPAppendicitis, Intussusception, Hirschsprung’s) as well as extra-abdominal issues (ex, Strep PharyngitisPneumoniaTesticular TorsionHSP).  Unquestionably, the intestine are integrally related to our health, so what happens when the intestines fail? To consider this, let us look at the unfortunate condition of Short Gut Syndrome:

 

Short Gut Syndrome: Basics

  • Short Gut Syndrome (AKA Short Bowel Syndrome) is a malabsorptive state.
    • High mortality rates
    • One of the most lethal conditions in infancy and childhood. [Duro, 2008]
    • Can be a temporary condition that improves with appropriate therapy.  [Vennarecci, 2000]
  • Can be due to congenital disease or from surgical resection. Examples:
    • Neuromuscular intestinal disorders (ex, total agangliosis)
    • Intestinal atresia
    • Midgut volvulus
    • Necrotizing enterocolitis
    • Gastroschisis
  • Intestinal Failure = reduction of functional gut mass to point of dependence on parenteral nutrition to maintain energy and fluid requirements for growth. [Duro, 2008; Diamanti, 2007]
  • The intestines can adapt and even grow in length and diameter.
  • Successful adaptation is predicated upon: [Duro, 2008]
    • Age
    • Underlying diagnosis
    • Length of small and large bowel resected
    • Specific portions of bowel that remain
    • Presence / absence of ileocecal valve and colon
    • Health of other digestive organs
    • Presence of bacterial overgrowth of small bowel.
    • Presence of enteral nutrients (why early enteral feeds is important)
  • Can lead to alterations in nutritional, metabolic, and infectious consequences. [Bhatia, 2010]

 

Short Gut Syndrome: Location Matters

  • Specific portions of the bowel manage different aspects of digestion and fluid management. [Bhatia, 2010]
    • Duodenum and Jejunum
      • Main sites for absorption of carbohydrates, proteins, and fats.
      • Fat-soluble vitamins, iron, calcium, copper, and water are also absorbed.
      • Water and electrolytes are managed.
    • Distal small bowel
      • Absorbs intrinsic-factor bound Vitamin B12.
      • Conjugated bile salts are transported.
      • Additional fluid and electrolyte management.
    • Colon
      • Further mitigates fluid and electrolyte losses.
  • The specific area of bowel that is lost helps direct medical management.[Bhatia, 2010]

 

Short Gut Syndrome: Complications

  • Complications are related to the length of bowel that has been removed. [Bhatia, 2010Duro, 2008]
    • Malabsorptive diarrhea
    • Fluid and electrolyte abnormalities
    • Micronutrient deficiences
    • Gastric hypersection
    • Bacterial overgrowth
  • Complications are related to the therapies. [Duro, 2008]
    • Parenteral nutrition complications
      • Parenteral Nutrition Associated Liver Disease (PNALD)
        • Steatosis, cholestasis, and cirrhosis
        • Occurs in 40-60% of infants receiving prolonged PN.
        • Development of PNALD negatively impacts survival significantly!
      • Metabolic bone disease
    • Central line complications
    • Post-surgical Complications
      • Gastrostomy / gastrojejunal / jejunostomy tube complications
      • Repeat episodes of necrotizing enterocolitis
      • Intestinal strictures and obstruction
      • Anastomotic leak
      • Anastomotic ulcers
      • Fistula formation

 

Short Gut Syndrome: Management

  • Nutritional support
    • Close monitoring of caloric needs
    • Micronutrients, fluids, and electrolytes
    • Parenteral nutrition is the primary supportive therapy.
      • It is life-saving, but leads to many acute and chronic complications.
      • Some may develop chronic dependence upon total parenteral nutrition. [Vennarecci, 2000]
    • Enteral nutrition
      • Early introduction of enteral nutrition is important to success!
      • Small, continuous enteral feeds are typically tolerated better than bolus feeds.
      • The goal is to transition off of parenteral nutrition.
      • Some can eventually transition to full enteral nutrition. [Duro, 2008]
  • Acid blockade
    • To reduce gastric hypersection, which worsens diarrhea and decreases absorption of nutrients.
  • Control of voluminous and watery stool (AKA diarrhea)
    • Fiber, loperamide, octreotide, and cholestyramine.
  • Counteract cholestasis
    • Prolonged parenteral nutrition can lead to cholestasis.
    • Ursodeoxycholic acid may help.
  • Counteract bacterial overgrowth
    • Rotating courses of antibiotics have been used.
  • Vitamin supplementation
    • Supplementation of fat soluble vitamins (A, D, E, K) is very important to nutritional well-being.
  • Surgical options
    • Feeding tubes
    • Intestinal lengthening procedures
      • Longitudinal intestinal lengthening and tailoring
      • Serial transverse enteroplasty procedure (STEP)
    • Intestinal transplantation
      • Considered when parenteral nutrition is resulting in life-threatening complications.
      • Can replace both the diseased intestine as well as the liver. [Vennarecci, 2000]

 

Moral of the Morsel

  • Short Gut Syndrome can be caused by several conditions.
  • Knowledge of the complex nature and management is important for EM providers to comprehend.
  • These children are complicated and deserve extra vigilance!
  • There underlying condition can lead to complications, but many of the therapies do also.

 

References

Davidovics ZH1,2, Carter BA1,2, Luna RA3,4,5, Hollister EB3,4,5, Shulman RJ1,2,6, Versalovic J7,4,5. The Fecal Microbiome in Pediatric Patients With Short Bowel Syndrome. JPEN J Parenter Enteral Nutr. 2016 Nov;40(8):1106-1113. PMID: 26059898. [PubMed] [Read by QxMD]

Bhatia J1, Gates A, Parish A. Medical management of short gut syndrome. J Perinatol. 2010 Oct;30 Suppl:S2-5. PMID: 20877403. [PubMed] [Read by QxMD]

Duro D1, Kamin D, Duggan C. Overview of pediatric short bowel syndrome. J Pediatr Gastroenterol Nutr. 2008 Aug;47 Suppl 1:S33-6. PMID: 18667916. [PubMed] [Read by QxMD]

Diamanti A1, Basso MS, Castro M, Calce A, Pietrobattista A, Gambarara M. Prevalence of life-threatening complications in pediatric patients affected by intestinal failure. Transplant Proc. 2007 Jun;39(5):1632-3. PMID: 17580205. [PubMed] [Read by QxMD]

Vanderhoof JA1, Young RJ, Thompson JS. New and emerging therapies for short bowel syndrome in children. Paediatr Drugs. 2003;5(8):525-31. PMID: 12895135. [PubMed] [Read by QxMD]

Sigalet DL1. Short bowel syndrome in infants and children: an overview. Semin Pediatr Surg. 2001 May;10(2):49-55. PMID: 11329605. [PubMed] [Read by QxMD]

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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2 Responses

  1. Colucciello, Stephen says:

    In adults with short gut, kidney stones are a big problem.
    -Dr. Colucciello

    • Sean Fox says:

      Dr. Colucciello,
      This is true… and another example of how the intestines affect many other organs! Also a great example of how pediatric illness can become a problem for adults as well (like Adult Congenital Heart Disease).
      Thank you,
      sean

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