Intermediate outcomes after serial transverse enteroplasty in children with short bowel syndrome.
ABSTRACT Serial transverse enteroplasty (STEP) is a novel intestinal lengthening procedure introduced in 2003. To date, no human studies exist that report objective assessment of intestinal absorptive capacity. The aim of this study was to report intermediate outcomes in patients who have received the STEP at our institution using clinical and biochemical assessment of intestinal function.
All 14 patients who received the STEP since May 2003 were reviewed. Clinical (weight gain, enteral tolerance, stool frequency) and biochemical (citrulline levels, D-xylose absorption, alpha-1 antitrypsin clearance, and fecal fat content) outcomes were performed pre-STEP and post-STEP at 1, 6, and 12 months, respectively. Data are presented as means with standard deviation. Paired t tests were used to compare post-STEP outcomes with pre-STEP values (P < .05 was significant). Three patients had a STEP as a newborn and are analyzed separately.
There were 14 patients (3 females; mean age, 24.8 months; range, 1 day-14 years). Serial transverse enteroplasty resulted in a mean increase in length of dilated bowel segment of 94% +/- 30% and increase in total small bowel length of 49% +/- 42% with mean application of 16 +/- 9 cartridges and cost of Can$2878.51 +/- 1406.22. Patients demonstrated improvement in both clinical parameters, as well as intestinal absorptive function. Complications included 2 patients with staple line leak and 1 patient with gastrointestinal bleeding from staple line ulcers. Three patients died (2 from liver failure and 1 from sepsis and congenital heart disease). Two patients received liver-intestinal transplants at 4 and 5 months post-STEP. Mean follow-up was 23 +/- 9 months, with 7 patients followed more than 2 years. Of 8 patients with post-STEP follow-up of more than 1 year, 7 have weaned from parenteral nutrition.
Clinical and objective biochemical outcomes of intestinal function after the STEP procedure show promise after intermediate follow-up.
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ABSTRACT: In human, citrulline (plasma concentration about 40 micromol/L) is an amino acid involved in intermediary metabolism and that is not incorporated in proteins. Circulating citrulline is mainly produced by enterocytes of the small bowel. For this reason plasma or serum citrulline concentration has been proposed as a biomarker of remnant small bowel mass and function. This article reviews this concept and its metabolic basis. Conditions in which there is a significantly reduced small bowel enterocyte mass and function and a plasma or serum citrulline were measured in adults and children. These studies included patients with a short bowel syndrome, villous atrophy states, Crohn's disease, during monitoring of digestive toxicity of chemotherapy and radiotherapy or follow-up of patients after small bowel transplantation. In all these situations, with more than 500 studied patients a decreased level of plasma citrulline correlated with the reduced enterocyte mass independently of nutritional and inflammatory status. A close correlation between small bowel remnant length and citrullinemia was found. In addition, diagnosis of intestinal failure was assessed through plasma citrulline levels in severe small bowel diseases in which there is a marked enterocyte mass reduction. The threshold for establishing a diagnosis of intestinal failure is lower in villous atrophy disease (10mumol/L) than in short bowel syndrome (20mumol/L). Compromised renal function is an important factor when considering plasma citrulline levels as a marker of intestinal failure as this potentially can increase circulating citrulline values. Reduced plasma citrulline levels are an innovative quantitative biomarker of significantly reduced enterocyte mass and function in different disease states in humans.Clinical nutrition (Edinburgh, Scotland) 07/2008; 27(3):328-39. · 3.27 Impact Factor
Article: Long-term nutritional and clinical outcomes after serial transverse enteroplasty at a single institution.[show abstract] [hide abstract]
ABSTRACT: Serial transverse enteroplasty (STEP) is a novel technique to lengthen and taper bowel in patients with intestinal failure. First described in 2003, initial data and reports have demonstrated favorable short-term outcomes, but there is limited published data on long-term outcomes of the procedure. Our aim was to assess clinical and nutritional outcomes after the STEP procedure. After obtaining institutional review board approval, we reviewed all records of patients (n = 16) who underwent the STEP procedure at our institution from February 2002 to February 2008. Patients were observed for a median time of 23 months (range, 1-71) postoperatively. Analyses of z scores for weight, height, and weight-for-height, and progression of enteral calories were performed using longitudinal linear models with random effects. Of the 16 patients (10 male), the median age at time of surgery was 12 months (interquartile range, 1.5-65.0). The mean increase in bowel length was 91% +/- 38%. After the STEP procedure, patients had increased weight-for-age z scores of 0.03 units per month (P = .0001), height for age z scores of 0.02 units per month (P = .004), and weight-for-height z scores of 0.04 units per month (P = .02). Patients had improved enteral tolerance of 1.4% per month (P < .0001). Six patients (38%) transitioned off parenteral nutrition (median, 248 days). Long-term complications included catheter-related bacteremia (n = 5), gastrointestinal bleeding (n = 3), and small bowel obstruction (n = 1). Two patients ultimately underwent transplantation. There were no deaths. In pediatric patients with intestinal failure, the STEP procedure improves enteral tolerance, results in significant catch-up growth, and is not associated with increased mortality.Journal of Pediatric Surgery 05/2009; 44(5):939-43. · 1.45 Impact Factor