Short Bowel Syndrome

Short Bowel Syndrome: Excerpt from The 5-Minute Pediatric Consult

Maria R. Mascarenhas, MBBSJudith Kelsen, MD

Short Bowel Syndrome - BASICS

Short Bowel Syndrome - description

Malnutrition, malabsorption, and/or fluid and electrolyte loss after extensive small-bowel resection

Short Bowel Syndrome - pathophysiology

• Markedly decreased mucosal surface area due to resection
• Loss of trophic hormones
• Loss of peptide hormones that regulate motility
• Abnormal transit
• Malabsorption of protein, fat, carbohydrate, vitamins, electrolytes, and trace elements, depending on site of resected intestine (see “Follow-Up”). The patient can lose as much as half of the intestine if the duodenum, distal ileum, and ileocecal valve (ICV) are present. If the ICV is gone, patients may not be able to tolerate even a 25% loss of intestine without the help of total parenteral nutrition (TPN).
• Normal bowel length: 150–200 cm (26 weeks’ gestation); 200–300 cm (at birth in full-term infant); 600–800 cm (adult)
• Infants have no intestinal reserve and do not tolerate small-bowel resection as well as do adults. However, long-term prognosis may be better because of hypertrophy and hyperplasia of the intestine.
• Gastric acid hypersecretion occurs soon after intestinal resection, but is transient.
• Bowel adaptation can occur over time. Increased surface area due to bowel dilatation, villus hypertrophy, and bowel lengthening can occur. Stimulation of luminal contents is needed for bowel growth and factors such as glutamine, short-chain fatty acids, tropic hormones, and growth factors may be important for bowel growth.

Short Bowel Syndrome - etiology

• Infants: Intestinal resection for necrotizing enterocolitis
• Congenital anomalies include intestinal atresias, gastroschisis, omphalocele, apple peel syndrome, and meconium ileus.
• Malrotation may result in volvulus with bowel resection secondary to ischemic injury.
• Older children: Neoplasms and radiation enteritis
• Intestinal resection secondary to Crohn disease, trauma, pseudo-obstruction syndrome

Short Bowel Syndrome - DIAGNOSIS

Short Bowel Syndrome - signs & symptoms

Short Bowel Syndrome - history

• Stooling pattern: Number, size, nature (watery, bulky, foul smelling), presence of blood and mucus
• Weight loss or gain: Gaining length/height
• Abdominal distention and flatulence
• Intense perianal rashes related to stool acidity and malabsorption of carbohydrates
• Abdominal pain and characteristics
• Vomiting and characteristics
• Diet history: Appetite, oral intake, tube feeds, parenteral nutrition (PN)
• Medication history
• Surgical history

Short Bowel Syndrome - physical exam

• Weight, length, and head circumference measurements (if applicable); try to get previous growth chart if available
• Look for signs of vitamin deficiencies in examination of mouth, lips, skin, hair loss, and healing difficulties.
• Abdominal examination: Surgical scars, ostomies, distention, hepatosplenomegaly, bowel sounds
• Rectal examination: Consistency of stool, heme positivity, perianal rash

Short Bowel Syndrome - tests

Short Bowel Syndrome - lab

• Blood tests:
  • CBC: Check for anemia and mean corpuscular volume.
  • Electrolytes: Check for losses and adequacy of replacement.
  • Minerals: Calcium, phosphorus magnesium, iron; check for losses and adequacy of replacement therapy.
  • Albumin and prealbumin: Check for protein stores and nutritional status.
  • Prothrombin time/partial thromboplastin time and protein induced by vitamin K absence (PIVKA): Assess vitamin K status.
    • A new test known as the PIVKA-II assay is a more sensitive measure of vitamin K status; however, it is more useful in adolescents.
  • Liver function tests: Alanine transaminase (ALT), γ-glutamyl transpeptidase (GGT), bilirubin if on PN to check for TPN-associated liver disease
  • Vitamin levels: Vitamin A, 25-hydroxy vitamin D, vitamin E, folic acid, BZinc level: Check status and adequacy of supplementation.
  • Carnitine: Check status if on long-term PN and presence of liver disease.
  • Breath tests: Lactose and lactulose breath test to check for lactase deficiency and bacterial overgrowth, respectively
• Stool tests:
  • Stool for pH and reducing substances: Check for carbohydrate malabsorption.
  • Stool smear for fat (Sudan stain—qualitative): Check for excessive fat loss.
  • Stool for blood: Check for mucosal damage.
  • Stool elastase: Measure of pancreatic insufficiency
• Tests of absorption:
  • Xylose absorption test and lactose breath test to check for carbohydrate malabsorption
  • 72-hour quantitative fecal fat collection along with concomitant diet record
  • Carotene levels to check for fat absorption
  • 24-hour stool collection for α1-antitrypsin clearance to check for protein absorption

Short Bowel Syndrome - imaging

Upper gastrointestinal series with small-bowel follow-through and barium enema to evaluate length, caliber, and location of remaining bowel

Short Bowel Syndrome - diag-proced-surgery

Endoscopy:

• Upper endoscopy: Look for presence of inflammation that may be contributing to malabsorption; get cultures for bacterial overgrowth.
• Lower endoscopy: Look for presence of colitis, especially eosinophilic colitis, as well as caliber of anastomotic site if in colon.

Short Bowel Syndrome - differencial diagnosis

• Infants: Necrotizing enterocolitis, volvulus, atresia (jejunal and ileal), gastroschisis, meconium peritonitis, congenital short-bowel syndrome
• Older children: Mid-gut volvulus (due to malrotation), Crohn disease, adhesions causing intestinal obstruction, strictures, trauma, aganglionosis of the intestine

Short Bowel Syndrome - TREATMENT

Short Bowel Syndrome - general measures

Short Bowel Syndrome - diet

• Fluid and electrolyte therapy: Extremely important in the acute phase immediately after bowel resection. In the chronic phase, it is important to keep up with ongoing losses, especially when enteral feeds are started.
• Oral diet: In those patients who are able to avoid PN or tube feeds, a low-lactose diet may be well tolerated. Low-oxalate diets are helpful in preventing oxalate stones. In general, a high-calorie diet regardless of carbohydrate and fat composition should be the mainstay of treatment.
• Enteral feeds: More successful in the patient with less extensive resection, intact ICV, and colon in continuity. In extensive loss, feeds initiated after electrolytes are stabilized.
  • Feeds started very slowly, often started with elemental diet to facilitate absorption, and for concern for allergic injury.
  • Enteral feeds stimulate intestinal adaptation. Before 1 year of age formula should have low osmolality: Higher fat content than carbohydrate.
  • After >1 year of age there is no advantage of elemental formulas over intact formulas with respect to tolerance, unless small-bowel damage is present.
• Enteral feeds are important for development of developmental milestones, with suck and swallow.
• PN: Important in the acute phase postoperatively, when nutrition must be maintained in the face of paralytic ileus; indispensable in the chronic phase when full enteral feeds cannot be instituted and nutrition needs to be maintained.
  • Balanced solutions of protein, glucose, and fat should be administered.
  • Prophylactic measures to prevent PN-induced liver damage should be instituted (e.g., prevention of overfeeding, early introduction of enteral feeds, cycling of PN when patient is stable). If cholestasis is present, it is necessary to modify amount of trace elements in PN.
  • Need permanent central access to deliver concentrated PN solutions.

Short Bowel Syndrome - medication

• Supplementation of vitamin (E, D, K, BHAntidiarrheal drugs: Codeine, diphenoxylate, and anticholinergic drugs (e.g., loperamide) to decrease motility (caution in patients with slow transit or bacterial overgrowth).
• Ion-exchange resins: Cholestyramine binds intraluminal dihydroxy bile acids to prevent bile acid–induced diarrhea.
• Octreotide/Somatostatin: Decreases gastric, pancreatic, and intestinal secretions; slows gastrointestinal motility and splanchnic blood flow
• Bacterial overgrowth: Commonly used oral antibiotics are metronidazole, trimethoprim–sulfamethoxazole, vancomycin, and gentamicin.
• Prokinetic agents: Reglan to treat delayed gastric emptying
• Miscellaneous: Sucralfate to treat bile reflux, probiotics to treat bacterial overgrowth, Ursodiol for cholestasis, Polycitra for electrolyte losses, dietary fiber to enhance absorption-caution in infancy.
• Duration of treatment: Depends on amount and site of bowel resected and degree of intestinal adaptation that occurs. The more the resection, the longer is the therapy. Successful enteral feeds decrease the duration of PN. Macronutrient losses decrease with intestinal adaptation. Micronutrient supplementation may be lifelong (e.g., vitamin B

  Short Bowel Syndrome - surgery

  • Surgery is useful in patients who develop strictures and partial obstruction or in those who have very short intestine length.
  • Intestinal interpositions (isoperistaltic or antiperistaltic) can be used to delay gastric emptying, slow intestinal transit, and increase absorption, intestinal valves, and reversed intestinal segments.
  • Intestinal lengthening and tapering procedures, including the Bianchi and step enteroplasty procedures, increase absorptive surface area.
  • In patients with extremely short intestines and PN dependency, small-bowel transplantation or small-bowel/liver transplantation is considered.

  Short Bowel Syndrome - FOLLOW UP

  Short Bowel Syndrome - prognosis

  • Depends on site and amount of bowel resected
  • The greater the amount of bowel resected, the worse is the prognosis.
  • Loss of ICV portends a worse prognosis.
  • Loss of jejunum and ileum creates a poorer clinical condition than loss of colon.
  • The longer it takes to tolerate full enteral feeds in a patient, the worse is the prognosis. Most progress is made in the first year after bowel resection.
  • Development of severe TPN liver disease: Poor prognosis

  Short Bowel Syndrome - complications

  • Fluid and electrolyte loss, resulting in diarrhea, dehydration, and metabolic acidosis
  • Calcium and magnesium deficiency, resulting in bone disease and osteoporosis
  • Carbohydrate malabsorption
  • Fat malabsorption
  • Vitamin A deficiency: Increased susceptibility to infections
  • Vitamin D deficiency: Bone disease (e.g., rickets)
  • Vitamin E deficiency: Peripheral neuropathy, hemolysis
  • Vitamin K deficiency: Prolonged clotting time, bruising
  • Vitamin BFolic acid: Macrocytic anemia
  • Gallstones: Due to disturbed enterohepatic circulation of bile salts and lithogenic bile formation
  • Renal stones: Due to fat malabsorption and increased oxalate absorption
  • Failure to thrive
  • TPN-dependent liver disease: Cholestasis, end stage is cirrhosis and portal hypertension
  • Zinc deficiency: Poor growth, infections
  • Carnitine deficiency: Contributes to development of steatosis
  • Sepsis
  • Small bowel bacterial overgrowth and D-lactic acidosis due to stasis, causing encephalopathy, ataxia, and other neurologic symptoms

  Short Bowel Syndrome - patient-monitoring

  • When to expect improvement: Depends on site and extent of bowel resection
  • Signs to watch for:
    • Vomiting, diarrhea, weight loss, severe fluid and electrolyte abnormalities, sepsis, bowel dilatation, intestinal obstruction
    • Major cause of death: Sepsis and cholestatic liver disease

  Short Bowel Syndrome - bibliography

  1. Buchman AL, Scolapio J, Fryer J. AGA technical review on short bowel syndrome and intestinal transplantation. Gastroenterology. 2003;124(4):1111–1134.
  2. Goulet O. Irreversible intestinal failure. J Pediatr Gastroenterol Nutr. 2004;38:250–269.
  3. Goulet O, Ruemmele J. Causes and management of intestinal failure in children. Gastroenterology. 2006;130:S16–S28.
  4. Hwang S, Shulman R. Recent advances in neonatal gastroenterology, update on the management and treatment of short gut. Clin Perinatol. 2002;29:181–194.
  5. Sigalet DL. Short bowel syndrome in infants and children: An overview. Semin Pediatr Surg. 2001;10(2):49–55.
  Vanderhoof JA. Short-bowel syndrome including adaptation. In: Walker WA, Watkins JB, eds. Nutrition in Pediatrics. Philadelphia: BC Decker; 2000:771–789.
  6. Vanderhoof JA, Young RJ. Enteral and parenteral nutrition in the care of patients with short-bowel syndrome. Best Pract Res Clin Gastroenterol. 2003;17(6):997–1015.
  7. Vanderhoof JA, Young RJ, Thompson JS, New and emerging therapies for short bowel syndrome in children. Pediatr Drugs. 2003;5(8):525–531.
  8. Warner BW, Vanderhoof JA, Reyes JD. What’s new in the management of short gut syndrome in children. J Am Coll Surg. 2000;190(6):725–736.
  9. Westergaard H. Short bowel syndrome. Semin Gastrointest Dis. 2002;13(4):210–220.

  Short Bowel Syndrome - CODES

  Short Bowel Syndrome - icd9

  • 579.3 Postsurgical
  • 579.9 Malabsorption syndrome

  Short Bowel Syndrome - FAQ

  • Q: What are the favorable prognostic factors in short-bowel syndrome?
  • A: Poor prognosis is related to the greater length of the bowel resected, loss of the ICV, loss of jejunum and ileum, longer time to tolerate full enteral feeds, and development of severe TPN–liver disease. Neonates have greater chances of bowel adaptation than do adults.
  • Q: Are elemental formulas better than intact formulas in the management of patients with short-bowel syndrome in patients >1 year of age?
  • A: Recent studies have shown similar rates of absorption, stomal output, and electrolyte losses between elemental and intact formulas. The disadvantages of elemental formulas include high osmolality and cost.

  Book Source Details

  • Book Title: The 5-Minute Pediatric Consult
  • Author(s): M. William Schwartz MD; et al.
  • Year of Publication: 2008
  • Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
  
  

  More About Short Bowel Syndrome

  • Back to disease: Short Bowel Syndrome: Introduction
  • Next Book Extract About Short Bowel Syndrome: Surveys relating to Short Bowel Syndrome
  • More Book Extracts: All Online Books for Short Bowel Syndrome

  More Medical Textbooks Online about Short Bowel Syndrome

  Review other book chapters online related to Short Bowel Syndrome:

  Medical Books Excerpts

  • Short Stature
  • "In A Page: Pediatric Signs and Symptoms" (2007)
  • [ read ]

  • Shortness of Breath
  • "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
  • [ read ]

  • Short Bowel Syndrome
  • "The 5-Minute Pediatric Consult" (2008)
  • [ read ]

   

  Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

  
  

  ---

  
  

  More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

  
   » Next page: Surveys relating to Short Bowel Syndrome

  Rate This Website

  What do you think about the features of this website? Take our user survey and have your say:

  Website User Survey

  Medical Tools & Articles:

  Next articles:

  • Surveys relating to Short Bowel Syndrome
  • Ask Question about Short Bowel Syndrome

  Tools & Services:

  • Bookmark this page
  • Symptom Search
  • Symptom Checker
  • Medical Dictionary
  • Give your feedback

  Medical Articles:

  • Disease & Treatments Search
  • Misdiagnosis Center
  • Full list of interesting articles

  Forums & Message Boards

  • Ask or answer a question at the Boards:
  • I cannot get a diagnosis. Please help.
  • Tell us your medical story.
  • Share your misdiagnosis story.
  • What is the best treatment for my condition?
  • See all the Boards.

  Enter your search terms Submit search form
  Search The Web Search wrongdiagnosis.com

Common Health Mistakes

Symptom
Checker

Search Specialists by State and City