Paediatric Short Bowel Syndrome (SBS)

Short Bowel Syndrome is a rare but serious condition that occurs when a child has an insufficient length of functional small intestine to absorb enough nutrients and fluids for normal growth and health. It most often follows extensive intestinal resection performed for conditions such as necrotising enterocolitis, volvulus, atresia, or severe Crohns disease.

1. Pathophysiology

The small intestine is the primary site for digestion and absorption of macronutrients, micronutrients, electrolytes, and water. When large segments are removed, several problems arise:

• Reduced absorptive surface area fewer villi and less mucosal mass.
• Transit time acceleration food moves quickly, limiting contact time.
• Loss of key hormones such as gastrin, motilin, and peptide YY, which regulate motility and secretion.
• Altered microbiome overgrowth of bacteria can further impair absorption.
• Fluid and electrolyte imbalance especially sodium and magnesium losses.

2. Clinical Presentation

Symptoms vary according to the remaining bowel length, location of the resection, and the childs age. Common features include:

• Chronic diarrhoea
• Weight loss or failure to thrive
• Dehydration and electrolyte disturbances
• Fatsoluble vitamin deficiencies (A, D, E, K)
• Metabolic bone disease
• Growth retardation

3. Diagnosis

Diagnosis is primarily clinical, supported by investigations:

• History & physical examination surgical history, bowel length, growth chart.
• Laboratory tests serum electrolytes, albumin, prealbumin, vitamin levels, liver function.
• Stool studies quantification of fat (72hour stool fat collection) and calprotectin.
• Imaging contrast studies or MRI enterography to assess residual bowel.
• Bone density scan when longterm deficiencies are suspected.

4. Management

Management is multidisciplinary, aiming to maximise nutrition, promote intestinal adaptation, and prevent complications.

4.1 Nutritional Support

4.2 Pharmacological Adjuncts

• GLP2 analogues (e.g., teduglutide) enhance mucosal growth and reduce PN dependence.
• Proton pump inhibitors decrease gastric hypersecretion that can worsen diarrhoea.
• Antimotility agents (e.g., loperamide) slow transit time.
• Antibiotics or probiotics manage bacterial overgrowth.
• Vitamin and mineral supplements especially fatsoluble vitamins, calcium, magnesium, zinc.

4.3 Surgical Options

When conservative measures fail, surgery may be considered:

• Intestinal lengthening procedures Serial transverse enteroplasty (STEP) or longitudinal intestinal lengthening and tapering (LILT).
• Isolated small bowel transplant reserved for PNrelated liver failure or recurrent sepsis.

4.4 Monitoring & Followup

Regular followup is essential:

• Growth parameters every 13months.
• Quarterly labs for electrolytes, liver function, and vitamin levels.
• Annual bone density scan after 2years of PN.
• Psychosocial support for families.

5. Prognosis

Outcomes have improved dramatically with modern PN, lipid emulsions, and bowel adaptation strategies. Approximately 4060% of children wean off PN completely within 25years, especially those with >30cm of jejunum and an intact ileocecal valve. Longterm complications include:

• PNassociated liver disease
• Catheterrelated bloodstream infections
• Metabolic bone disease
• Growth delay if adaptation is insufficient

6. Living with SBS

Families need education on catheter care, signs of dehydration, and nutrition management. School and daycare coordination is vital to ensure safe feeding practices. Many centres provide homePN training, allowing children to attend regular school and enjoy normal activities.

7. Key Resources

• International Society for Research on Short Bowel Syndrome (ISRSBS)
• American Society for Parenteral and Enteral Nutrition (ASPEN) guidelines
• National Institute for Health and Care Excellence (NICE) Guidance on paediatric PN
• Patient support groups such as SBS UK

This page is intended for educational purposes and should not replace professional medical advice.