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428 Short Bowel Syndrome
Table 71.2: Evidence-based research.
Title Comparison of intestinal lengthening procedures for patients Outcome/ This review involved 64 patients, including 14 adults, who
with short bowel syndrome effect underwent 43 Bianchi and 34 STEP procedures between
1982 and 2007. Three patients had prior isolated liver
Authors Sudan D, Thompson J, Botha J, Grant W, Antonson D, transplants. The median (range) remnant bowel length before
Raynor S, Langnas A
first lengthening was 45 (11–150) cm overall (Bianchi = 44
Institution Department of Surgery, Nebraska Medical Center, Omaha, cm; STEP = 45 cm); and 68 (20–250) cm after lengthening
Nebraska, USA (Bianchi = 68 cm; STEP = 65 cm). Actual survival is 91%
overall (Bianchi, 88%; STEP, 95%), with a median follow-up
Reference Ann Surg 2007; 246(4):593–601; discussion 601–604 of 3.8 years (Bianchi = 5.9 years; STEP = 1.7 years). Average
Problem Outcome of bowel-lengthening procedures. enteral caloric intake in paediatric patients was 15 kcal/kg
before lengthening and 85 kcal/kg at 1 year after lengthening.
Intervention A review of the clinical results of 24 years of intestinal Sixty-nine percent of patients were off TPN at the most recent
lengthening procedures at one institution. follow-up, including 8 who were weaned from TPN after
Comparison/ A retrospective review of a single centre experience intestinal transplantation. Liver disease (when present) was
reversed in 80%. Surgical complications occurred in 10%,
control comparing the outcome of two intestinal-lengthening more commonly requiring reoperation after Bianchi than
(quality of procedures (Bianchi and STEP) in terms of survival, total STEP. Intestinal transplantation salvage was required in 14%
parenteral nutrition (TPN) weaning, and complications.
evidence) at a median of 2.9 years (range = 8 months to 20.7 years)
after lengthening.
Historical Surgical lengthening with both Bianchi and STEP
significance/ procedures results in improvement in enteral nutrition,
comments reverses complications of TPN, and avoids intestinal
transplantation in the majority, with few surgical
complications. Intestinal transplantation can salvage most
patients who later develop life-threatening complications or
fail to wean TPN.

Key Summary Points

1. Short bowel syndrome may be congenital or acquired. 5. Surgical options include tapering, bowel lengthening using the
Bianchi approach, the STEP procedure, or bowel transplant.
2. The effect of bowel resection depends on the site and length of
bowel resected and the bowel remaining. 6. Long-term survival without TPN depends on the remaining
3. Intestinal adaptation takes place by means of bowel dilatation, bowel length. With TPN availability, survival is related to
complications of TPN rather than to bowel length.
lengthening, and thickening to increase the efficiency of
absorption per unit of length.
4. Medical management is mainly supportive along with fluid and
electrolyte balance, nutritional support with or without TPN, and
infection control.

References

1. Gupte GL, Beath SV, Kelly DA, Millar AJW, Booth IW. Current 4. Goulet O. Short bowel syndrome in pediatric patients. Nutrition
issues in the management of intestinal failure. Arch Dis Child 1998; 14:784–787.
2006; 91:259–264.
5. Bianchi A. Intestinal loop lengthening: a technique for increasing
2. Duro D, Kamin D, Duggan C. Overview of pediatric short bowel small intestinal length. J Pediatr Surg 1980;15:145–151.
syndrome. J Pediatr Gastroenterol Nutr 2008; 47(suppl 1):533–
536. 6. Tannuri U. Serial transverse enteroplasty (STEP): a novel bowel
lengthening procedure, and serial transverse enteroplasty for short
3. Ching YA, Guruk K, Modi B, Jaksic T. Pediatric intestinal failure: bowel syndrome. J Pediatr Surg 2003; 38:1845–1846.
nutrition, pharmacologic and surgical approaches. Nutr Clin Pract
2007; 22:653–663.