Short Bowel Syndrome

Key Features

• 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

Clinical Findings

• 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

Diagnosis

• 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

Treatment

• 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