Comparison of Intestinal Lengthening Procedures for Patients With Short Bowel Syndrome

Department of Surgery, Nebraska Medical Center, Omaha, Nebraska 68198-3285, USA.
Annals of Surgery (Impact Factor: 8.33). 11/2007; 246(4):593-601; discussion 601-4. DOI: 10.1097/SLA.0b013e318155aa0c
Source: PubMed


Review the clinical results of 24 years of intestinal lengthening procedures at one institution.
Retrospective review of a single center experience comparing the outcome of 2 intestinal lengthening procedures (Bianchi and serial transverse enteroplasty [STEP]) in terms of survival, total parenteral nutrition (TPN) weaning, and complications.
Sixty-four patients, including 14 adults, underwent 43 Bianchi and 34 STEP procedures between 1982 and 2007. Three patients had prior isolated liver transplants. The median (range) remnant bowel length before first lengthening was 45 (11-150) cm overall; (Bianchi=44 cm, STEP=45 cm) and 68 (20-250) cm after lengthening; (Bianchi=68 cm, STEP=65 cm). Actual survival is 91% overall (Bianchi 88%, STEP 95%) with median follow-up of 3.8 years (Bianchi=5.9 years, STEP=1.7 years). Average enteral caloric intake in pediatric patients was 15 kcal/kg before lengthening and 85 kcal/kg at 1 year after lengthening. Sixty-nine percent of patients are off TPN at most recent follow-up, including 8 who were weaned from TPN after intestinal transplantation. Liver disease (when present) was reversed in 80%. Surgical complications occurred in 10%, more commonly requiring reoperation after Bianchi than STEP. Intestinal transplantation salvage was required in 14% at a median of 2.9 years (range=8 months to 20.7 years) after lengthening.
Surgical lengthening with both Bianchi and STEP procedures results in improvement in enteral nutrition, 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.

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    • "The techniques of LILT and STEP have been described in detail previously [10] [11] [14]. Simple tapering of a dilated bowel segment was performed with a linear stapler along the long axis of a dilated bowel segment [11]. "
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    ABSTRACT: Aims: This is a descriptive study aiming to compare outcomes of intestinal rehabilitation surgery among pediatric and adult intestinal failure (IF) patients with either primary intestinal motility disorders or short bowel syndrome (SBS) treated by our nationwide program. Methods: Medical records of IF patients (n = 31, 71% children) having undergone autologous intestinal reconstructions (AIR) (n = 25), intestinal transplantation (ITx) (n = 5), or being listed for ITx (n = 2) between 1994 and 2014 were reviewed. Results: At surgery, median age was 3.4 (interquartile range, 1.0-22.1) in SBS (n = 22) and 16.5 (3.2-26.7) years in dysmotility patients (n = 9) who received median 60% and 83% of energy requirement parenterally, respectively. Median small bowel length was shorter in SBS than dysmotility patients (34 versus 157 cm, p < 0.001). Following AIR, none of the dysmotility patients achieved permanent intestinal autonomy, whereas 68% of SBS patients weaned off parenteral nutrition (PN) (p = 0.022) and none required listing for ITx. Five dysmotility patients who underwent ITx achieved intestinal autonomy. Regarding both AIR and ITx procedures, no significant difference in PN weaning was observed between the two subgroups. At last follow-up, 3.3 (0.6-8.0) years postoperatively, median plasma bilirubin was 6 (4-16) µmol/l, while liver biopsy showed fibrosis (Metavir stage 1-2) in 50% and cholestasis in 8%. Proportion of PN energy requirement had reduced significantly (p = 0.043) among PN-dependent SBS (n = 7) but not among dysmotility patients (n = 5). Overall survival was 90%. Conclusion: AIR surgery was beneficial among selected SBS patients, whereas in intestinal dysmotility disorders, permanent PN weaning was only achieved by ITx.
    Full-text · Article · Nov 2014 · Scandinavian Journal of Gastroenterology
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    • "In such cases, where the chance of spontaneous adaptation is minimal, creating controlled obstruction to actively generate bowel dilatation is suggested in order to be able to perform AGIR surgery later [39]. Careful patient selection is essential to avoid unnecessary operations, since some infants especially with low birth weight and necrotizing enterocolitis as the etiology leading to a very short bowel remnant may wean off PN without any AGIR procedures at all [38]. In a select group of patients, despite long remnants of small bowel, rapid transit results in increased ileostomy output due to absence of ileocecal valve (ICV) and colon. "
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    ABSTRACT: Short bowel syndrome (SBS) is a state of malabsorption resulting from massive small bowel resection leading to parenteral nutrition (PN) dependency. Considerable advances have been achieved in the medical and surgical management of SBS over the last few decades. This review discusses in detail the surgical approach to SBS. Widespread use of PN enables long-term survival in patients with intestinal failure but at the cost of PN-associated life-threatening complications including catheter-associated blood stream infection, venous thrombosis, and liver disease. The goal of management of intestinal failure due to SBS is to enable enteral autonomy and wean PN by means of a multi-disciplinary approach. Availability of modified enteral feeding formulas have simplified nutrition supplementation in SBS patients. Similarly, advances in the medical field have made medications like growth hormone and glucagon-like peptide (GLP2) available to improve water and nutrient absorption as well as to enable achieving enteral autonomy. Autologous gastrointestinal reconstruction (AGIR) includes various techniques which manipulate the bowel surgically to facilitate the bowel adaptation process and restoration of enteral nutrition. Ultimately, intestinal transplantation can serve as the last option for the cure of intestinal failure when selectively applied. SBS continues to be a challenging medical problem. Best patient outcomes can be achieved through an individualized plan, using various AGIR techniques to complement each other, and intestinal transplantation as a last resort for cure. Maximum benefit and improved outcomes can be achieved by caring for SBS patients at highly specialized intestinal rehabilitation centers.
    Full-text · Article · Jun 2014 · Viszeralmedizin / Visceral Medicine
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    • "leading to intestinal failure may include gastroschisis, necrotizing enterocolitis (NEC), malrotation with volvulus, jejunal atresia, and other more rare intestinal anomalies or enteropathies. Clinical interventions to minimize the damaging effects of PN to the immature pediatric liver include transitioning the child to enteral nutrition with a slowly increasing feed volume with the hope of adaptation, use of " hepatosparing " PN formulas, autologous gastrointestinal reconstruction, and minimization of the inflammatory effects of bacterial translocation [4] [5] [6]. Children with intestinal failure benefit from early referral to a center of excellence in intestinal rehabilitation for development of a comprehensive treatment plan [4]. "
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    ABSTRACT: Infants with parenteral nutrition dependence may develop liver dysfunction and progress to liver failure requiring transplantation. The aspartate aminotransferase-to-platelet ratio index (APRI) has good correlation with liver fibrosis progression in adults. This study applies APRI scoring to parenteral nutrition-dependant, short-gut infants to determine hepatic fibrosis progression. Laboratory values and biopsies were collected from initial intestinal resection (time 0) up to transplantation (end). Fibrosis scoring ranged from F0 (normal) to F4 (cirrhosis). Children were divided into 3 groups: (1) isolated intestine; and combined liver/intestine with gestational age (2) 34 weeks or greater and (3) 30 weeks or less. Liver function values over time, including calculated APRI, were analyzed as predictors of fibrosis. Fifteen children who had 33 biopsies were included. Median APRI by fibrosis grade was F < or = 2: 1.88, F3: 3.23, and F4: 14.16 (P < .01). Median APRI at transplant by study group was (1) isolated intestine: 2.47, (2) liver/intestine 35 weeks or longer EGA: 14.16, and (3) liver/intestine 30 weeks or less EGA: 14.74 (P = .04). Progression of APRI up to 60 days initially demonstrates similar values among study groups, but over time the score distinguishes those children with impending liver cirrhosis and differentiates fibrosis grade and study group.
    Full-text · Article · Jun 2010 · Journal of Pediatric Surgery
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