Pediatric Short Bowel Syndrome

Updated: Aug 15, 2016
  • Author: Carmen Cuffari, MD; Chief Editor: Jatinder Bhatia, MBBS, FAAP  more...
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Overview

Practice Essentials

Short bowel syndrome (SBS) results from the alteration of intestinal digestion and absorption that occurs after extensive bowel resection. It is a complex disorder with nutritional, metabolic, and infectious consequences. Bowel obstruction is a potential complication of SBS (see the image below).

Small-bowel obstruction caused by intussusception Small-bowel obstruction caused by intussusception in a 5-month-old patient is visible on plain radiograph.

Signs and symptoms

Although SBS may rarely be a congenital condition, most patients with SBS have undergone bowel resection for one of the following:

  • A congenital anomaly such as an omphalocele, gastroschisis, or intestinal atresia
  • Necrotizing enterocolitis associated with prematurity [1]
  • Intestinal ischemia from malrotation and volvulus
  • Crohn disease (infrequent with current treatment)

Children with cloacal exstrophy do not have SBS in the classical sense but often have all the signs and symptoms associated with functional intestinal failure.

Patients with SBS may present with any of the following:

  • Nutritional deficiencies
  • Malabsorptive diarrhea
  • Dehydration
  • Vomiting
  • Bloating
  • Gastroesophageal reflux
  • Failure to thrive
  • Drug toxicities
  • Sepsis

See Clinical Presentation for more detail.

Diagnosis

Children with SBS should undergo standard hematologic and biochemical studies, according to institutional guidelines. Studies may include the following:

  • Electrolytes, BUN, creatinine, calcium, magnesium, phosphorous - Biweekly in both the initial phase and the late period or at the time of presentation for instability
  • Comprehensive metabolic panel, CBC, triglycerides, cholesterol - Weekly in both the initial phase and the late period or at the time of presentation for instability
  • Folate, vitamin B-12, vitamin E, copper, zinc, selenium - Monthly in both the initial phase and the late period or at the time of presentation for instability

Carnitine

The workup for suspected sepsis in patients with SBS may include the following:

  • Blood cultures from both the central and peripheral sites
  • Urinalysis and blood culture specifically to search for fungal infection
  • Abdominal ultrasonography to search for fungal balls in the kidney (only in the case of persistent fungal infection)
  • CT scanning of the abdomen to identify persistent sepsis
  • Ultrasonography of Broviac tip

The workup for suspected bowel obstruction in patients with SBS may include the following:

  • Plain radiography of the abdomen
  • Barium imaging of the bowel

Laboratory procedures may include the following:

  • Upper endoscopy to assess for peptic ulcer disease and possible signs of liver disease (eg, esophageal varices, hypertensive gastropathy)
  • Liver biopsy to evaluate for total parenteral nutrition (TPN)-related liver disease (rarely performed unless the presentation is unusual)

See Workup for more detail.

Management

The management of SBS requires an aggressive multidisciplinary approach that is most often tailored to the individual needs of the patient. [2] Features of treatment include the following:

  • The institution of early and aggressive enteral therapy is the most important stimulus for intestinal adaptation and the eventual discontinuation of parenteral therapy
  • Aggressive resuscitation with fluids, parenteral nutrition, or both is often required in the first week after intestinal resection
  • Serum electrolytes and a comprehensive biochemical panel should be monitored daily In the early postoperative period and on a biweekly or triweekly basis once the values have stabilized
  • The hypersecretion noted within the first 12 months postresection is usually treated with H2 blockers or proton pump inhibitors
  • Parenteral fluid replacement needs may be ongoing, depending on the amount of stool or ostomy output
  • Bacterial overgrowth in the proximal small bowel may require antibiotic therapy, including metronidazole alternating with either kanamycin or oral gentamicin
  • Cholestyramine has been used to bind bile salts in patients with choleretic diarrhea
  • A diverting ileostomy can be used to prevent the stagnation of stool in patients with significant dysmotility
  • Small-bowel transplantation is used only if absolutely necessary in patients with associated severe advanced liver disease and those with major vascular access problems
  • Isolated orthotopic liver transplantation without small-bowel transplantation has been demonstrated to be effective

See Treatment and Medication for more detail.

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Background

Few conditions in pediatric gastroenterology pose as great a challenge as short bowel syndrome (SBS). [3] Short bowel syndrome is the result of the alteration of intestinal digestion and absorption that occurs following extensive bowel resection. It represents a complex disorder that affects normal intestinal physiology with nutritional, metabolic, and infectious consequences.

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Pathophysiology

The small intestine of the neonate is approximately 250 cm in length. By adulthood, the small intestine grows to approximately 750 cm. As a consequence, the infant and the young child have a favorable long-term prognosis compared to an adult in regards to potential intestinal growth after intestinal resection. Intestinal adaptation may take weeks to months to be achieved; in the interim, children who have had intestinal resection need nutritional support through various therapeutic measures, including parenteral nutrition. The duodenum and jejunum are responsible for the absorption of most dietary constituents except vitamin B-12 and bile acids.

The jejunum is characterized by long villi and a large absorptive area. The tight junctions are relatively large, rendering the epithelium more porous to larger molecules and the free and rapid flux of water and electrolytes. In comparison, the ileum has shorter villi and a less absorptive surface area than the jejunum. Furthermore, the tight junctions are tighter, permitting less flux of water and electrolytes from the vascular space into the intestinal lumen, and, consequently, the ileum is more efficient in the absorption of water. Although nutrients are less well absorbed in the ileum, it has site-specific receptors for the absorption of bile acids and vitamin B12. Moreover, many GI hormones that affect intestinal motility, including enteroglucagon and peptide YY, are produced in the ileum. The small intestinal sites of nutrient absorption are as follows:

  • Duodenum - Iron
  • Jejunum - Carbohydrates, proteins, fat, vitamins
  • Ileum - Bile acids, vitamin B-12

In general, virtually all digestion and absorption is completed within the first 100-150 cm of jejunum in a healthy individual. In the absence of an intact colon, the minimum length of healthy bowel necessary to avoid parenteral nutrition is approximately 100 cm. Patients who have less than 100 cm of jejunum exhibit significant malabsorption. Although the ileum is limited in its capacity to form chylomicrons compared to the jejunum, studies have shown that the ileum has greater adaptive function as far as improving its absorptive function in the presence of short bowel syndrome. Similarly, studies in animals have shown that intestinal transit time is more likely to improve (ie, increase) in patients with proximal small-bowel resection as opposed to patients with distal small-bowel resection.

The jejunum cannot develop site-specific carriers for the transportation of vitamin B12 and bile salts, and, consequently, these are malabsorbed permanently in patients, following ileal resection. Furthermore, the loss of enteroglucagon and peptide YY cannot be underscored in the regulation of small-bowel motility.

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Epidemiology

Mortality/Morbidity

The leading causes of death in infants with short bowel syndrome who are being treated with parenteral nutrition are central line sepsis and liver failure with the prolonged use of parenteral nutrition.

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