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Key Features

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  • Results from reduced intestinal absorptive surface that leads to alteration in intestinal function that compromises normal growth, fluid/electrolyte balance, or hydration status

  • Most common etiologies

    • Necrotizing enterocolitis (45%)

    • Intestinal atresias (23%)

    • Gastroschisis (15%)

    • Volvulus (15%)

  • Less common etiologies

    • Congenital short bowel

    • Long-segment Hirschsprung disease

    • Ischemic bowel

  • Many factors influence the process and likelihood of bowel adaptation and achievement of enteral autonomy, including

    • Patient's gestational age

    • Postsurgical anatomy (including residual small bowel length and presence of ileocecal valve and/or colon)

    • Presence of small bowel bacterial overgrowth

    • Underlying surgical disease

  • No specific anatomic bowel length measurements offer 100% certainty in predicting clinical outcomes

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Clinical Findings

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  • Typical symptoms are related to the underlying malabsorptive state, including

    • Diarrhea

    • Dehydration

    • Electrolyte or micronutrient deficiency states

    • Growth failure

  • Patients are also at risk for

    • Small bowel obstruction

    • Bowel dilation and dysmotility (with secondary small bowel bacterial overgrowth)

    • Hepatobiliary disorders, including cholelithiasis

    • Nephrolithiasis due to calcium oxalate stones

    • Oral feeding challenges

    • Gastrointestinal mucosal inflammatory problems, including noninfectious colitis and anastomotic ulcerations

  • Recurrent catheter-related bloodstream infections are relatively common

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Diagnosis

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  • Parenteral nutrition is required in many cases to provide adequate caloric, fluid, and electrolyte delivery in the setting of insufficient intestinal absorptive function

  • Intestinal failure can be diagnosed when supplemental parenteral nutrition is required for more than 2–3 months in the setting of short bowel syndrome or any other underlying disorder

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Treatment

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  • Management goal is to promote growth and adaptation while minimizing and treating complications of the underlying intestinal disorder or parenteral nutrition therapy

  • Enteral nutrition should favor absorption, commonly requiring continuous delivery of an elemental formula through a gastrostomy tube

  • Commonly prescribed pharmacologic adjuncts include

    • Acid suppressive therapy

    • Antimotility and antidiarrheal agents

    • Antibiotics for the treatment of small bowel bacterial overgrowth

  • Emerging therapies targeted to promote bowel adaptation include glucagon-like peptide 2 analogs

  • Autologous bowel reconstructive surgery (bowel lengthening) should be considered in a patient who is not advancing enterally and has anatomy amendable to surgical intervention, typically with regards to adequate bowel dilation

  • Both the serial transverse enteroplasty (STEP) procedure and longitudinal intestinal lengthening and tailoring (Bianchi) procedure have been successful in allowing weaning from total parenteral nutrition in up to 50% of patients

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