John K DiBaise Md Facg

Contents

1 Anatomical and Physiological Considerations

2 Intestinal Adaptation

3 Treatment

4 Role of Diet and Fluids

5 Role of Pharmacological Management

6 Role of Trophic Factors

7 Role of Surgical Management

8 Weaning of Parenteral Nutrition

9 Conclusion

Summary

Short bowel syndrome (SBS) results from extensive intestinal resection. It is associated with significant morbidity and mortality, a reduced quality of life and a high rate of health care resource utilization. The management of patients with SBS requires a multidisciplinary approach that includes dietary, fluid and pharmacological management, co-morbid disease management and, occasionally, surgery. An understanding of the physiological alterations that occur in SBS patients is useful to understand the therapeutic strategies employed. In the pages that follow, these physiological alterations are discussed as are the roles of diet and fluids, specialized nutrition support, medications including trophic factors and surgery in the care of these complex patients.

From: Clinical Gastroenterology: Nutrition and Gastrointestinal Disease Edited by: M.H. DeLegge © Humana Press Inc., Totowa, NJ

Short bowel syndrome (SBS) is a malabsorption syndrome resulting from extensive intestinal resection [1, 2]. In infants, necrotizing enterocolitis and congenital intestinal anomalies are frequently responsible. In older children and adults, multiple resections for Crohn's disease and massive resections due to catastrophic mesen-teric vascular events, radiation enteritis, adhesive obstruction and trauma represent the more common causes of SBS [3]. These patients frequently experience chronic diarrhea, dehydration, and macro-and micronutrient deficiencies often requiring enteral or parenteral nutrition support at home. It has been demonstrated, using nutrient absorption studies, that patients who absorb <1.4kg/day of wet weight or <84% of their calculated energy needs will likely require parenteral fluid and/or nutrition support [4]. This typically translates into a patient with <50-70 cm of small bowel when the colon is intact, or <100-150 cm of small bowel when the colon is absent [5]. For practical purposes, in adults, SBS can be defined as the presence of <200 cm of the remaining small intestine. In infants, the diagnosis of SBS relies less on an anatomical definition and more on a functional definition. The amount of resection required to produce malabsorption in infants varies with factors such as age, the presence or absence of an ileocecal valve and length of residual colon [6].

Key Words: Short Bowel Syndrome, Multidisciplinary Treatment, Parenteral Nutrition, Intestinal Transplantation

The prevalence of SBS is unclear. A 1997 European survey indicated a point prevalence of home parenteral nutrition (HPN) use of about 4 per million, of whom approximately 35% had SBS [7]. In the United States, the annual prevalence of HPN use was estimated at about 120 per million, of whom approximately 25% had SBS [8]. These numbers likely underestimate the prevalence of SBS as they do not reflect patients with SBS who never required HPN or were able to be successfully weaned from parenteral nutrition (PN). The difference in HPN use between the US and Europe may reflect differences in both the calculation of the prevalence of HPN patients and in the ease of accessibility of HPN in the US. Importantly, there is no single reliable database available to precisely capture the number of SBS patients on HPN in either the US or Europe.

While SBS is clearly uncommon, it remains an important clinical problem due to its effect on these patients' quality and duration of life, the high rate of associated complications and the subsequent high costs involved in the their care [8]. Quality of life has been shown to be worse in HPN patients, many of whom had SBS, compared to SBS patients not requiring PN [9]. Table 8.1 lists complications occurring

Table 8.1 Complications in Short Bowel Syndrome Patients

Central venous catheter-related Infection Occlusion Breakage

Central vein thrombosis

Parenteral nutrition-related

Hepatic Biliary

Bowel anatomy-related

Malabsorptive diarrhea Malnutrition

Fluid and electrolyte disturbances Micronutrient deficiency Essential fatty acid deficiency Small bowel bacterial overgrowth D-lactic acidosis Oxalate nephropathy Renal dysfunction Metabolic bone disease Acid peptic disease Anastomotic ulceration/stricture in SBS patients that can be related to either the altered bowel anatomy or its treatment [1, 5, 10]. With respect to survival, studies from France and the US have demonstrated 2-year and 5-year survival rates for SBS at over 80% and 70%, respectively [11, 12]. Furthermore, the study from France reported PN-dependency at 2 years of 49% and 45% at 5 years [12]. Survival rates were lowest in the end-jejunostomy and ultra-short small bowel groups. Other factors affecting survival include the patient's age, primary disease process, co-morbid diseases, presence of chronic intestinal obstruction and the experience of the team managing the patient [13].

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