Surgical Rehabilitation Techniques in Children with Poor Prognosis Short Bowel Syndrome

Abstract

Intestinal failure (IF) requires a multidisciplinary management based on nutritional support, surgical and medical rehabilitation, and transplantation. The aim of this study is to review our experience with surgical rehabilitation techniques (SRTs: enteroplasty, Bianchi, Serial Transverse Enteroplasty Procedure [STEP]) in patients with short bowel syndrome (SBS) and poor prognosis due to complex abdominal pathology.

We performed a single-center retrospective study of patients with IF evaluated for intestinal transplantation in the Intestinal Rehabilitation Unit who underwent an SRT. Nonparametric tests were used for statistical analysis.

A total of 205 patients (107 males/98 females) with mean age of 25 ± 7 months were assessed for IF. A total of 433 laparotomies were performed on 130 patients including intestinal resection, enteroplasties, adhesiolysis, and transit reconstruction. SRT were performed in 22 patients: 12 enteroplasties, 8 STEPs, and 4 Bianchi procedures. All patients were parenteral nutrition (PN) dependent with different stages of liver disease: mild (13), moderate (5), and severe (4). The adaptation rate for patients who underwent enteroplasty, STEP, and Bianchi were 70, 63, and 25%, respectively, although the techniques are not comparable. Overall, intestinal adaptation was achieved in nine (41%) patients, and four (18%) patients showed significant reduction of PN needs. One child did not respond to SRT and did not meet transplantation criteria. The remaining eight (36%) patients were included on the waiting list for transplant: four were transplanted, two are still on the waiting list, and two died. Better outcomes were observed in milder cases of liver disease (mild 77%, moderate 40%, severe 25%) (p < 0.05). Conversely, a trend toward a poorer outcome was observed in cases with ultrashort bowel (p > 0.05). One patient required reoperation after a Bianchi procedure due to intestinal ischemia and six needed further re-STEP or adhesiolysis procedure several months later. The median follow-up was 62 (3–135) months. Overall mortality was 19%, and was due to end-stage liver disease and/or central venous catheter–related sepsis. SRT led to intestinal adaptation in a significant number of patients with poor prognosis SBS referred for intestinal transplantation. However, SRT requires a multidisciplinary evaluation and should be attempted only in suitable cases. Careful assessment and optimal surgical timing is crucial to obtain a favorable outcome.

Keywords

surgical rehabilitation techniques - intestinal failure - short bowel syndrome

• References

• 1 Kaufman SS, Matsumoto CS. Management of pediatric intestinal failure. Minerva Pediatr 2015; 67 (4) 321-340
• 2 Coletta R, Khalil BA, Morabito A. Short bowel syndrome in children: surgical and medical perspectives. Semin Pediatr Surg 2014; 23 (5) 291-297
• 3 Pakarinen MP. Autologous intestinal reconstruction surgery as part of comprehensive management of intestinal failure. Pediatr Surg Int 2015; 31 (5) 453-464
• 4 Sudan D, Thompson J, Botha J , et al. Comparison of intestinal lengthening procedures for patients with short bowel syndrome. Ann Surg 2007; 246 (4) 593-601 , discussion 601–604
• 5 Kim HB, Fauza D, Garza J, Oh J-T, Nurko S, Jaksic T. Serial transverse enteroplasty (STEP): a novel bowel lengthening procedure. J Pediatr Surg 2003; 38 (3) 425-429
• 6 Kim HB, Lee PW, Garza J, Duggan C, Fauza D, Jaksic T. Serial transverse enteroplasty for short bowel syndrome: a case report. J Pediatr Surg 2003; 38 (6) 881-885
• 7 Millar AJW. Non-transplant surgery for short bowel syndrome. Pediatr Surg Int 2013; 29 (10) 983-987
• 8 Jones BA, Hull MA, McGuire MM, Kim HB. Autologous intestinal reconstruction surgery. Semin Pediatr Surg 2010; 19 (1) 59-67
• 9 Beath SV, Woodward JM. Intestinal failure-associated liver disease. In: Lanhas AN, Goulet O, Quigley EMM, Tappenden KA, eds. Intestinal Failures: Diagnosis, Management and Transplantation. 1st ed. Malden, MA: Blackwell Publishing; 2008: 191-200
• 10 Fusaro F, Hermans D, Reding R. Autologous gastrointestinal reconstructive surgery: complement or alternative to intestinal transplantation. Curr Opin Organ Transplant 2013; 18 (3) 286-290
• 11 King B, Carlson G, Khalil BA, Morabito A. Intestinal bowel lengthening in children with short bowel syndrome: systematic review of the Bianchi and STEP procedures. World J Surg 2013; 37 (3) 694-704
• 12 Yannam GR, Sudan DL, Grant W, Botha J, Langnas A, Thompson JS. Intestinal lengthening in adult patients with short bowel syndrome. J Gastrointest Surg 2010; 14 (12) 1931-1936
• 13 Miyasaka EA, Brown PI, Teitelbaum DH. Redilation of bowel after intestinal lengthening procedures—an indicator for poor outcome. J Pediatr Surg 2011; 46 (1) 145-149
• 14 Frongia G, Kessler M, Weih S, Nickkholgh A, Mehrabi A, Holland-Cunz S. Comparison of LILT and STEP procedures in children with short bowel syndrome — a systematic review of the literature. J Pediatr Surg 2013; 48 (8) 1794-1805
• 15 Bueno J, Redecillas S, GarcÍa L , et al. Duodenal lengthening in short bowel with dilated duodenum. J Pediatr Surg 2015; 50 (3) 493-496
• 16 Andres AM, Thompson J, Grant W , et al. Repeat surgical bowel lengthening with the STEP procedure. Transplantation 2008; 85 (9) 1294-1299
• 17 Piper H, Modi BP, Kim HB, Fauza D, Glickman J, Jaksic T. The second STEP: the feasibility of repeat serial transverse enteroplasty. J Pediatr Surg 2006; 41 (12) 1951-1956
• 18 Ba'ath ME, Almond S, King B, Bianchi A, Khalil BA, Morabito A. Short bowel syndrome: a practical pathway leading to successful enteral autonomy. World J Surg 2012; 36 (5) 1044-1048
• 19 Jones BA, Hull MA, Potanos KM , et al; International STEP Data Registry. Report of 111 consecutive patients enrolled in the International Serial Transverse Enteroplasty (STEP) Data Registry: a retrospective observational study. J Am Coll Surg 2013; 216 (3) 438-446
• 20 Modi BP, Javid PJ, Jaksic T , et al; International STEP Data Registry. First report of the international serial transverse enteroplasty data registry: indications, efficacy, and complications. J Am Coll Surg 2007; 204 (3) 365-371