Short bowel syndrome

Short bowel syndrome (SBS) is defined as loss of bowel mass from surgical removal, congenital anomaly or disease. A small number of long-gap OA/TOF patients fall into this definition as a result of the jejunal and colonic interpositions undertaken to repair the OA, in particular those where complications meant that more than one such procedure was undertaken. It is estimated that having less than 200cm of functional intestine is needed to develop SBS and less than 35cm for intestinal failure (where oral feeding plus supplements are not sufficient to meet nutritional needs and parenteral nutrition is necessary). (195)

Symptoms

Whilst any adults with OA/TOF will have had loss of bowel since childhood and some adaptation will have occurred over this time, some may present with ongoing symptoms in adulthood, particularly if any further surgery to the bowel has occurred, and some may have worsening problems due to gastrointestinal infection or may be debilitated by the symptoms they have lived with for many years.

• Diarrhoea and steatorrhoea (fat in the stools, meaning they are bulky, difficult to flush, oily and are foul smelling)
• Fatigue, malaise and lethargy
• Weight loss
• Signs of vitamin deficiency (vitamins A, D, E, K, B12, thiamine, calcium, magnesium and zinc deficiency can occur) (196)

Management

1. The great majority with SBS are managed with diet. This is a specialist area, needing dietician and specialist gastrointestinal surgeon/gastroenterologist input, but in general drinking and eating around 1.5x more than their nutritional requirement is encouraged so that the relative malabsorption can be overcome and they should eat throughout the day, not just at the three meal times.
2. Vitamin supplementation may be needed:

• Thiamine (thiamine absorption can also be affected by blind loop syndrome/ small intestinal bacterial overgrowth)
• B12 in all patients who have had more than 30cm ileum removed due to malabsorption
• Fat-soluble vitamins such as A, D and E may also be deficient due to malabsorption
• Zinc deficiency may also occur due to diarrhoea losses in SBS patients

3. Control of diarrhoea – this can include opiates and loperamide.
4. Severely affected patients may need parenteral nutrition. (196)