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Treatment Options

Nutritional Therapies

All patients will undergo a complete nutritional analysis. Our physicians and dietitians work closely to determine recommendations regarding optimal Total Parenteral Nutrition (TPN) management, fluid and electrolyte management and special diet designed to meet the patient's specific needs. These recommendations are provided to the patient and referring physician to help achieve desired outcomes. Goals are to increase weight, decrease stool output, improve hydrations status and improve nutrient deficiencies.

    What is TPN?

    Total Parenteral Nutrition (TPN) is a method of feeding a person through an intravenous (IV) catheter. Physicians often order this method of feeding when a person is unable to absorb enough nutrition from the food they consume. TPN contains nutrients such as carbohydrates, lipids, proteins, vitamins, minerals, and water.

    What is Enteral Feeding?

    Enteral Feeding is a method of delivering liquid feedings though a tube and is used in instances where the gastrointestinal tract is functional, but the person cannot consume enough food to meet nutritional requirements.

Lipid-reduction Therapy

Our intestinal rehabilitation program has championed a lipid reduction therapy to treat TPN-related cholestasis. The graph shows the results of a recent group of neonates treated with lipid reduction, and demonstrates a significant reduction in parenteral nutrition-associated liver disease with this program.

Ethanol-lock Program

Our intestinal rehabilitation program has developed an aggressive ethanol lock therapy program to prevent the development of catheter-related infections. With the use of this program we have decreased intravenous catheter infections from 8.0 catheter infections per 1000 catheter days down to 1.3 catheter infections per 1000 catheter days. Please see the publication on this work by our group.

Surgical Options

Surgical options include a number of methods to taper the intestine in hopes of improving intestinal motility and preventing the development of bacterial overgrowth. Additionally, a number of other surgical methods to lengthen the intestine have also been introduced. These include the Bianchi method and the more recent STEP procedure. All of these options may be considered in patients with SBS, however, they are used only after it is clear that medical management is not effective in weaning the child off TPN.

A surgeon is available for consultation at the clinic and will make recommendations specific to the individual need and clinical presentation. Surgical procedures performed at Mott Children's Hospital include bowel lengthening either by a standard Bianchi or STEP procedure and bowel tapering. Additionally, routine gastrointestinal surgeries, including feeding tube placement and management, and antireflux procedures are provided.

    The Bianchi Method

    Figure 13 below shows a bowel lengthening procedure via the Bianchi method. Note that the bowel is split longitudinally by separating the mesentery to either one side of the intestine or the other. A stapling device then splits the bowel longitudinally. The bowel is then placed into continuity, with correct peristaltic direction.

    Figure 13

    The STEP Procedure

    Figure 14 below shows the current STEP procedure for intestinal lengthening. Note that the absolute length of bowel can be adjusted by altering the interval of creating linear staplings, and the luminal diameter can be corrected by the length of each stapling. Figure adapted from K. Georgenson. J Pediatr Surg. 2003, 38(6):881-5, and originally described by Bae and Jaksic, J Pediatr Surg. 2003 Jun;38(6):881-5

    Figure 14

Please take a look at a novel procedure our group developed by performing sequential STEP procedures in infants with short bowel syndrome:
http://www.ncbi.nlm.nih.gov/pubmed/17502191

Please also look at long-term outcomes of infants after a STEP procedure. This highlights important aspects of what patients may or may not benefit from such a procedure:
http://www.ncbi.nlm.nih.gov/pubmed/21238656