Serial Transverse Enteroplasty Procedure Research Articles

A new surgical technique for short bowel syndrome

ObjectiveShort bowel syndrome (SBS) is a severe intestinal disease that causes malabsorption. Long-term parental nutrition therapy induces infection and liver failure. For the surgical management of intestinal rehabilitation, the intestinal loop lengthening method and serial transverse enteroplasty (STEP) method have been reported, although their effects have proven limited. We herein report a new surgical technique, Saeki–Spiral–Shark (3S) method for SBS using biomimetics of shark intestine.MethodsIn the 3S method, a spiral valve is formed inside the intestine by external sutures. Using a 25 cm length intestinal organ model, we performed both the 3S method and STEP procedure. We then compared the length and fluid passage times of the subsequently formed intestine.ResultsAfter the 3S method was performed, the length of the intestinal model changed to 22 cm, and after the STEP procedure, that was elongated to 30 cm. Although the water passage times did not change markedly, the semi-digestive nutritional supplement passage time slowed down in the model with the 3S method. There was slight leakage in the STEP procedure model.ConclusionsThe 3S method is a unique method of treating SBS based on biomimetics. This procedure does not require an incision of the intestine, which thereby enabling clean and less-invasive surgery. We plan to conduct animal experiments in the future.

213.7: A Data-driven Predictive Analytics Framework for the Analysis of Prognostic Factors and Health Outcomes in Paediatric Intestinal Failure Patients

Introduction: Machine learning (ML) involves computational methods and learning mechanisms that can help generate new knowledge from large databases. These techniques are uniquely suited to analyses of paediatric intestinal failure (IF) where classification, prognostication, identification of risk factors, and knowledge extraction can be challenging. Heterogeneous patient populations, small sample sizes, and variations in treatments may complicate our ability to manually identify trends in data. Factors that predict enteral autonomy (EA) and patient management (optimal intravenous lipid emulsion [ILE] solutions; central venous catheter [CVC] complications) have been described, yet a systematic approach that may be prospectively applied to large datasets has never been performed in this patient population. Our study aims to identify predictors of EA through machine learning (ML) and assess their impact on TPN duration. For patients on TPN, assessments of lipid emulsion (ILE) and central venous catheter (CVC) type were also performed. Methods: Demographics, procedures, and outcomes for patients (N=17) from McMaster Children’s Hospital’s (MCH) Intestinal Rehabilitation Program (1999-2021) were collected for a retrospective chart review. Predictors of EA status were obtained using a decision tree ML model. Performance was evaluated as area under the receiver operator characteristics (AUC) and precision-recall (PR) curves. These predictors were assessed for ability to impact TPN duration with Kaplan-Meier (KM) estimates and Cox proportional-hazards (CPH) models. ILE treatments were compared using the Mann-Whitney U test, and CVC types were compared with KM, CPH, and analysis of variance (ANOVA). Results: The model retrieved 10 predictors of EA status. AUC and PR curves were 91% and 71% respectively. Of these 10 predictors: sex, serial transverse enteroplasty (STEP) procedure, ileocecal valve (ICV) presence, and remnant small bowel length were significant for predicting EA status under KM, but not CPH. For ILE treatment, patients on SMOF Lipid® had lower total bilirubin values compared to Intralipid® at six months (4.0 versus 10.0 uMol/L). Longevity of peripherally inserted central catheters (PICC) was 847 days versus 268 days for tunnelled CVCs, and 181 days for ports (95% CI: 0-2044, 93-443, 144-218). Types of complications were not significantly different across CVC. Conclusions: Our study supports the role for ML frameworks in advanced pattern recognition, and promoting adaptive clinical management. Validation of this model using our single-centre dataset supports similar conclusions derived from larger, multi-center registries. Ongoing use of our ML framework offers a novel contribution to intestinal failure analyses by helping identify unique risk factors and protective factors for EA, and approaches to clinical management in the paediatric IF population.

Serial transverse enteroplasty (STEP) in case of short bowel syndrome: did we achieve our goal? A systematic review.

Surveys on Serial Transverse Enteroplasty (STEP) published in international literature (1 January 2003- 31 May 2021) were searched. Articles were included from 17 countries: 1/23 comparative and 22/23 cohort studies. STEP was performed on 308 patients: pediatrics, adults, and mixed ages. Pediatric group included 16 studies and the adult 6. Pre-STEP residual small bowell (SB) length for pediatrics and adults ranged from 18 to 26cm and from 30 to 70cm, respectively. Post-STEP increased SB length for pediatrics and adults ranged between 42 and 100% and 50% and 176%, respectively. For pediatrics, enteral autonomy was reached in 32.22% of cases, parenteral nutrition (PN) dependence was 36.11%, a repeated STEP procedure (Re-STEP) was needed in 17.22%, and a bowel transplant was performed in 6.11%. In adults, enteral autonomy was achieved in 52.38%, while PN dependence was 37.1%, and no Re-STEP or transplantation were required. For the mixed group, post-STEP bowel length increased from 2 to 50cm, enteral autonomy was obtained in 43%, PN dependence was 57%, without reported Re-STEP or transplantation. Mortality rates were between 5.55% (pediatric) and 7.14% (adults). Preoperative length with preservation of ileocecal valve represented the main predictive factors to achieve enteral autonomy.

Comparing bowel lengthening procedures: which, when, and why?

Purpose of reviewIntestinal failure secondary to short bowel syndrome is still a very serious condition. Treatment consists of parenteral nutrition to provide nutrients and maintain body weight. During the last decades, intestinal lengthening procedures have become more available. The goal of this review is to discuss the results of the literature on the most commonly performed intestinal lengthening procedures.Recent findingsLongitudinal Intestinal Lengthening, Serial Transverse Enteroplasty (STEP), and Spiral Intestinal Lengthening and Tailoring (SILT) are currently the most frequently reported intestinal lengthening procedures. The most recent literature of these procedures is described with respect to indication, technical details, complications, short and long-term outcome, and PN independence.SummaryOn the basis of indication, surgical complexity, complications, and clinical success, we conclude that the STEP procedure is probably the best choice for most centers.

Long-Term Outcome of Children with Short Bowel Syndrome Treated with a Modification of the STEP Technique Avoiding Mesenteric Defect.

The Serial Transverse Enteroplasty Procedure (STEP) Registry has reported a 47% success to achieve enteral autonomy in pediatric short bowel syndrome (SBS). We have performed the STEP with a technical modification (MSTEP) consisting in stapler application without mesenteric defects that can also be applied to the duodenum. Our experience with this technique is described. In this study, 16 children with SBS underwent MSTEP (2005-2019). Indications were nutritional autonomy achievement (n = 11, with duodenal lengthening in 5/11) and bacterial overgrowth treatment (n = 5). With a median follow-up of 5.8 years (0.7-13.7 years), 5 of 11 (45%) patients achieved enteral autonomy, 4 of them with duodenal lengthening. Four of four who preserved > 50% colon, while only one of seven with < 50% of colon achieved enteral autonomy (p < 0.05). After redo procedures, three of four attained enteral autonomy. Thus, 8 of 11 (73%) progressed to enteral autonomy, including all with duodenal lengthening. One child, already parenteral nutrition free, died due to central line sepsis. All the patients from the bacterial translocation group improved their metabolic/nutritional status, but one required subsequent enterectomy of the lengthened intestine due to multiple ulcers in the staple lines. The effectiveness of MSTEP to achieve enteral autonomy seems similar to the classical STEP. It can be applied to the duodenum. The retained colon length may influence the post-STEP enteral autonomy achievement.

Experiences with intestinal failure from an intestinal rehabilitation unit in a country without home parenteral nutrition.

For the first time, we reported experiences with an intestinal rehabilitation unit (IRU) from a country without home parenteral nutrition (HPN). We included patients with a diagnosis of intestinal failure (IF) since the establishment of our IRU from February 2018 to February 2020. We further report on our protocols for management of enterocutaneous fistulas (ECFs), short-bowel syndrome (SBS), chronic intestinal pseudo-obstruction and motility disorders. Among a total of 349 patients who have been admitted, 100 patients had IF and were included . Mean (SD) age of patients was 46.3 ± 16.1 years. Most common cause of IF was ECFs (32%), SBS (24%), and SBS + fistula (22%). Most common causes of SBS were mesenteric ischemia (63.3%) and repeated surgery (22.4%). Median (interquartile range [IQR]) duration of parenteral nutrition (PN) for patients was 32 (18-60) days. The most common reconstructive surgery performed was resection and anastomosis (75.4%), followed by serial transverse enteroplasty procedure (10.5%) and closure of ostoma (7%). Patients were hospitalized for a median (IQR) of 33 (17.5-61) days. Most common complications were sepsis (45%), catheter infections (43%), and catheter thrombosis (20%), respectively. At the final follow-up, 61% stopped receiving PN, 23% became candidates for transplantation, and 16% died. Considering that most countries lack facilities for HPN, by establishing IRUs using specific treatment protocols and autologous gastrointestinal reconstruction techniques will provide a means to manage patients with IF, thus decreasing death rates and number of patients who require intestinal transplantations due to IF.

Use of Lumen-Apposing Metal Stents for Inflammatory Bowel Disease-Related Strictures at a Pediatric Center: A Case Series

Use of Lumen-Apposing Metal Stents for Inflammatory Bowel Disease-Related Strictures at a Pediatric Center: A Case Series

Repeat serial transverse enteroplasty procedure (reSTEP): Is it worth it?

Background: The utility of repeat serial transverse enteroplasty (reSTEP) has been questioned after patients failed to achieve enteral autonomy. We compared the outcomes after reSTEP to one lifetime STEP (oneSTEP), and tried to identify patients at risk for reSTEP failure.Methods: All STEPs done at our institution were reviewed. Growth, enteral autonomy, bowel-derived bloodstream infection hospital admissions, complications, and need for bowel transplantation were evaluated (p<0.05 considered significant).Results: 24 patients underwent 32 STEP (16 oneSTEP, 8 reSTEP). reSTEP patients were younger at first surgery (1.01±1.05 vs. oneSTEP 3.06±4.73 years, p = 0.24). Median time to reSTEP was 1.1 year (0.6–5.7). Weight-for-length z-scores improved after reSTEP (0.02±1.40 to 0.22±1.42, p = 0.81). Bowel-derived bloodstream infections decreased after surgery (oneSTEP 1.50±2.25 to 0.94±1.73, p = 0.50; reSTEP 1.88±2.10 to 1.66±2.32, p = 0.52). 37.5% (9/24) patients achieved enteral autonomy at last follow-up: 7/16 oneSTEP, 2/8 reSTEP (p = 0.37). Two complications occurred after oneSTEP (staple line ulcer, leak), none following reSTEP. Three patients (oneSTEP 1/16, reSTEP 2/8, p = 0.19) underwent bowel transplantation (all gastroschisis).Conclusion: Similar postoperative outcomes after reSTEP and single STEP (improved enteral tolerance, reduced rates of infections) support the use of reSTEP when clinically indicated, although reSTEP in young infants with a history of gastroschisis may need further evaluation.

Repeat serial transverse enteroplasty leads to reduction in parenteral nutrition in children with short bowel syndrome

Background/purposeFollowing a serial transverse enteroplasty (STEP) procedure some children develop redilation of the small intestine leading to impaired enteral tolerance and inability to wean parenteral nutrition (PN). The benefit of a second STEP procedure (2STEP) has been controversial. MethodsWe performed a retrospective review of our experience (2008–2018) performing 2STEP, with comparative analysis of nutritional outcomes pre- and postsurgery. ResultsDuring this period 2STEP was performed in 23 patients (13 F:10 M) at a median (25%–75%) age of 2.2 (1.2–3.6) years. Median intestinal length was 68 (40–105) cm before and 85 (40–128) cm after 2STEP. Leading up to 2STEP, PN provided almost 75% of estimated calorie needs. By 24 weeks following 2STEP drops in mean PN percent approached statistical significance (p = 0.07) and at most recent follow up the mean PN percentage was statistically better than at the time of operation or 4 weeks prior to 2STEP, and was nearly significant compared with 12 weeks (p = 0.07) and 24 weeks (p = 0.06) prior. Thirteen children were completely off parenteral support. ConclusionWhen small intestine redilation occurs following a STEP procedure and where PN cannot otherwise be weaned we believe these data support performing a 2STEP. We cannot predict preoperatively which children will ultimately benefit. Level of evidence3 (retrospective comparative study).

P4.25: Serial Transverse Enteroplasty in Children with Short Bowel Syndrome

Introduction: Serial transverse enteroplasty (STEP) is an intestinal lengthening procedure used for short bowel syndrome (SBS) that was first introduced in 2003. This study aimed to report clinical outcomes in children who underwent the STEP procedure at our institution. Methods: STEP procedures were performed according to the guidelines of Kim et al. Since March 2008 at our hospital, five children dependent on parenteral nutrition (PN) underwent the STEP procedure. Pre- and post-STEP clinical outcomes were reviewed retrospectively. Results: Our cohort included three girls and four boys with SBS. The causes of SBS were jejunoileal atresia (2), necrotizing enterocolitis (4), and total colonic aganglionosis (1). The median age on STEP was 37months (range, 7–59). STEP procedures were performed 1.71 times/patient, three times in one patient, three patients for two times, and one times for four patients. Intestinal length was increased in all cases. There were no perioperative complications and no evidence of intestinal leak or obstruction. Three patients have been completely weaned from PN. Other three patients have remained on PN, but their PN requirement has been decreased. In one patient with total colonic aganglionosis with small bowel involvement, the PN requirement has not been changed. Conclusions: STEP is a simple bowel-lengthening procedure associated with promising early PN weaning and decrease in PN requirement. But in case of SBS caused by total colonic aganglionosis, it may not be so helpful. Further data from a multicenter registry are needed to evaluate its long-term efficacy and proper candidate.

P1.31: Salvage therapy with Infliximab for anastomotic ulcers- a potential treatment for a challenging complication

Background:Children with short bowel syndrome (SBS) may require bowel lengthening procedures such as the Serial Transverse Enteroplasty (STEP) procedure. STEP may be associated with severe complications such as anastomotic and staple line ulcers with gastrointestinal (GI) bleeding. These complications pose a therapeutic challenge and are often refractory to medical therapy. We present a child with recurrent GI bleeds from staple line ulcers responsive to treatment with Infliximab (IFX). Case:An 11 year old male with SBS and intestinal failure secondary to gastroschisis with midgut atresia underwent STEP procedures at 4 months of age and again at 4 years. He developed recurrent GI bleeds and was found to have multiple ulcers along staple lines on repeat endoscopies. Initial treatment included mesalamine (65mg/kg/day), oral budesonide (9mg), pantoprazole, omega-3, and cycling antibiotics with no improvement. Due to failure of all treatment attempts by 8 years old, he was started on IFX (6.5mg/kg/dose at week 0, 2, and 6 weeks then every 8 weeks). IFX level at dose #4 was <0.035ug/mL, anti-IFX antibodies <2AU/mL. Almost complete mucosal healing was found on endoscopy after dose #4. At infusion #6, anti-IFX antibodies were 70AU/mL, the child developed a rash and fever during the infusion and IFX was stopped. Other bioplogic therapies attempted included Adalimumab and Golimumab, not effective in preventing recurrent GI bleeding. The failure of medical therapies led to massive small bowel resection with removal of all areas involved with the previous STEP procedures. The most proximal staples were left in situ. Despite resection, new ulcers and GI bleeding occurred at the remaining staple site and in the duodenum. As a result IFX was reintroduced with methylprednisolone for premedication. The patient received IFX (10mg/kg/dose, week 0 and 2), repeat induction at week 4 due to low levels of IFX <0.035ug/mL. Follow up endoscopy showed excellent mucosal healing after 3 doses of IFX. Due to high anti-IFX antibodies, 190AU/mL, IFX was held at dose #4. Conclusion:Anastomotic and staple line ulcers post-STEP procedures were responsive to treatment with IFX in this case. Development of anti-IFX antibody can be a barrier for a successful outcome and should be monitored. The response to IFX suggests a potential role for innate immunity and TNF alpha in the pathogenesis of anastomotic ulcers.

Autologous intestinal reconstruction: a single institution study of the serial transverse enteroplasty (STEP) and the longitudinal intestinal lengthening and tailoring (LILT)

To review the effectiveness of the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) operations in a cohort of patients with short bowel syndrome (SBS). We conducted a retrospective analysis of children with SBS treated at our institution from 2004 until 2014. Children aged 0days to 18years with SBS who underwent autologous intestinal reconstruction were included in the study. Twenty-two SBS patients underwent 31 different lengthening procedures (LP). Seventeen patients underwent their primary lengthening procedures at our institution: 9 (53%) patients underwent a LILT, 7 (41%) underwent a STEP and 1 (6%) had a simultaneous LILT and STEP procedure. 12/22 patients had a second STEP, two had a third STEP and one patient had an intestinal transplantation after the LP. Median intestinal length at the time of surgery was 25cm (range 12-90cm). There was no difference in gain of intestinal length after LILT vs. STEP (p = 0.74). Length of stay and initiation of feeds were similar. Serum albumin increased after autologous bowel lengthening (p < 0.001). 50% were weaned off parenteral nutrition (PN) (5/9 of the LILT, 1/7 of the STEP, 1/1 of the combined LILT/STEP). There were no surgical complications or deaths. In patients with SBS, LILT and STEP procedures are effective for autologous intestinal reconstruction and enable intestinal rehabilitation.

“First STEP” or Nature Deserves a Second Chance! Systematic Literature Review and Meta-analysis

Aim: Surgical management of short bowel syndrome (SBS) in children is challenging. Recently, more authors are advocating for the neonatal serial transverse enteroplasty procedure (STEP) in SBS quoting the term “primary STEP” or “first STEP.” This review sought to identify the current published indications for neonatal STEP and to analyze their subsequent outcomes.Methods: We performed an OVID MEDLINE/ EMBASE search using the keywords: (Bowel, enteroplasty, intestinal lengthening, STEP, and short bowel) limited to children since the introduction of STEP in 2003 prospero systematic review registration number (CRD42017076955).Results: Thirteen papers matched our search criteria, and accurate data were available from 10 papers. A total of 26 cases had a STEP procedure at a median age of 2.5 days. The primary diagnosis was Jejunal atresia (62%), gastroschisis (19%), gastroschisis with atresia (15%), and midgut volvulus (4%). Almost a third (7/23) of the cases did not meet the anatomical definition of SBS and had a pre STEP residual small bowel (SB) length of ≥50 cm. Only 6 cases (26%) achieved enteral autonomy after the “first STEP,” interestingly in half the pre STEP SB length was ≥90 cm, 13 (56%) required a second STEP, 9 (40%) are still parenteral nutrition dependant, 4 more cases achieved enteral autonomy following a second STEP, 3 infants died, and one required SB transplantation. Significant post-operative complications were reported in four cases, and bowel redilatation occurred in almost all true SBS cases.Conclusion: Redilatation following “first STEP” is very common, may influence the ability to achieve enteral autonomy and generally necessitates further surgical intervention. The limited current evidence does not support the widespread use of STEP in the neonatal period. STEP can be a method of mucosal-sparing tailoring procedure; however, its outcomes in primary bowel lengthening in the neonatal period are yet to be established, and further studies are required before it is widely adopted.

Short bowel mucosal morphology, proliferation and inflammation at first and repeat STEP procedures

BackgroundAlthough serial transverse enteroplasty (STEP) improves function of dilated short bowel, a significant proportion of patients require repeat surgery. To address underlying reasons for unsuccessful STEP, we compared small intestinal mucosal characteristics between initial and repeat STEP procedures in children with short bowel syndrome (SBS). MethodsFifteen SBS children, who underwent 13 first and 7 repeat STEP procedures with full thickness small bowel samples at median age 1.5 years (IQR 0.7–3.7) were included. The specimens were analyzed histologically for mucosal morphology, inflammation and muscular thickness. Mucosal proliferation and apoptosis was analyzed with MIB1 and Tunel immunohistochemistry. ResultsMedian small bowel length increased 42% by initial STEP and 13% by repeat STEP (p=0.05), while enteral caloric intake increased from 6% to 36% (p=0.07) during 14 (12-42) months between the procedures. Abnormal mucosal inflammation was frequently observed both at initial (69%) and additional STEP (86%, p=0.52) surgery. Villus height, crypt depth, enterocyte proliferation and apoptosis as well as muscular thickness were comparable at first and repeat STEP (p>0.05 for all). Patients, who required repeat STEP tended to be younger (p=0.057) with less apoptotic crypt cells (p=0.031) at first STEP. Absence of ileocecal valve associated with increased intraepithelial leukocyte count and reduced crypt cell proliferation index (p<0.05 for both). ConclusionsNo adaptive mucosal hyperplasia or muscular alterations occurred between first and repeat STEP. Persistent inflammation and lacking mucosal growth may contribute to continuing bowel dysfunction in SBS children, who require repeat STEP procedure, especially after removal of the ileocecal valve. Level of evidenceLevel IV, retrospective study.

Case Report of an Adult Patient Who Underwent a Serial Transverse Enteroplasty Procedure for Short Bowel Syndrome Following an Esophagectomy and Total Gastrectomy

Since its introduction as an alternative intestinal lengthening technique, the serial transverse enteroplasty (STEP) procedure has been used increasingly as the surgical treatment of choice for children with short bowel syndrome (SBS). On the other hand, there are few report of its efficacy in adults with SBS, particularly those who have previously undergone a gastrectomy. This case report describes a 34-year-old woman with a short bowel after an esophagectomy and total gastrectomy due to lye ingestion followed by an extensive intestinal resection due to small bowel strangulation. The STEP procedure was performed successfully and the small intestine was lengthened from 55 to 75 cm. The patient tolerated the procedure well and was weaned off total parenteral nutrition. The frequency and characteristics of diarrhea improved, and her weight remained acceptable via management with intermittent parenteral nutritional support for 6 months postoperatively. This case suggests that the STEP procedure should be considered for gastrectomized patients with SBS.

Serial Transverse Enteroplasty Procedure in the New Era of Gut Rehabilitation

Serial Transverse Enteroplasty Procedure in the New Era of Gut Rehabilitation

Factors Related to the Development of Small Bowel Bacterial Overgrowth in Pediatric Intestinal Failure

Background: Small bowel bacterial overgrowth (SBBO) is a challenge in the management of pediatric intestinal failure (PIF). The development of SBBO may delay or prevent weaning of parenteral nutrition (PN) contributing to other complications. Our goal was to determine the proportion of patients treated for SBBO by an intestinal rehabilitation program and factors related to its development. Methods: A retrospective analysis of PIF patients referred between 2008–2014. Data was collected on factors related to IF and SBBO. Patients were also evaluated for refractory SBBO (use of 3 or more antimicrobial agents). Diagnosis of SBBO was based on clinical symptoms. Statistical testing completed using T-test, Chi square and logistic regression. Results: 35 of 102 patients followed by our (IRP) developed SBBO (34%) and 16 (16%) had refractory SBBO. Children with SBBO had an older gestational age (34.8 vs 32.3 weeks, p=0.01) and higher birth weight (2345 vs 1843g, p=0.001). Those with gastroschisis were more likely to develop SBBO (40.0 vs 19.4%, p=0.025). The development of SBBO was more likely in those with shorter percentage residual small bowel (SB) (45.4 vs 66.5%, p=0.004). Those with SBBO were more likely to have had a serial transverse enteroplasty (STEP) (22.9 vs 3.0%, p=0.004). Patients with SBBO were less likely to wean from parenteral nutrition (PN) (51.4 vs 85.1%, p < 0.0001). Similar to those with SBBO, refractory SBBO patients had a higher birth weight (2557 vs 1903g, p=0.007) and were likely to have gastroschisis (50.0 vs 22.1%, p=0.020). Refractory SBBO patients had shorter percentage small and large bowel remaining (23.2 vs 65.9%, p < 0.0001 and 60.6 vs 79.4%, p=0.03), more likely to have had a STEP procedure (50.0 vs 2.3%, p < 0.0001) and less likely to wean from PN (37.5 vs 80.2%, p=0.001). Logistic regression for development of SBBO demonstrated that longer small bowel residual was protective (p=0.001, OR 0.95; CI 0.93-0.99) and IF category (SBS) was as risk factor (p=0.001, OR 0.04; CI 0.01-0.27). The presence of an ileocecal valve (ICV), stoma and method of delivery (vaginal vs cesarian section) was not significant. Conclusion: SBBO has management challenges in PIF. A longer SB remnant was protective against the development of SBBO. Patients with SBBO were more likely to have a diagnosis of SBS and remain PN dependent. The presence of the ileocecal valve, stoma or method of delivery did not predict development of SBBO in our cohort.

Taking a STEP back: Assessing the outcomes of multiple STEP procedures

PurposeShort bowel syndrome (SBS) is a highly morbid condition primarily because of parenteral nutrition (PN)–associated complications. Bowel lengthening via serial transverse enteroplasty (STEP) has become standard of care. While initial STEPs have resulted in weaning from PN, outcomes of repeated STEPs (ReSTEPs) are not well described. We investigated outcomes of initial STEP compared to ReSTEP procedures. MethodsThis retrospective review of STEPs included 17 children and a total of 24 procedures. Demographics, complications, hospital readmission rates, postoperative costs, and PN weaning were analyzed. ResultsNeither patient-specific data nor the etiology of SBS was predictive of requiring a ReSTEP. PN weaning was more likely in the year following a first STEP (18% wean rate vs. 0% for ReSTEP, p>.05). No ReSTEP patients reached enteral autonomy. Enteral nutrition (%EN) increases were greater after first STEP compared to ReSTEP (26.0% vs. 4.7%, p=0.03). This trend was true for bowel length as well, where first STEPs resulted in a 51% increase in bowel length compared to a 20% increase after in ReSTEP (p=0.02). ConclusionsReSTEPs failed to result in significant PN weaning, with no ReSTEP patients achieving enteral autonomy during follow-up. Given its higher costs, smaller bowel length gains, and limited ability to produce enteral autonomy, surgeons should carefully consider performing ReSTEP procedures. Level of evidenceLevel III.

The Long-Term Treatment of Short Bowel Syndrome

Short bowel syndrome (SBS) is the most common cause of intestinal failure in the pediatric population. Functionally, SBS is defined as the spectrum of malabsorption features occurring after extensive surgical resection. Congenital SBS is a rare entity, with an estimated prevalence of 0.02-0.1% among all live births. The mainstay of treatment consists of parenteral nutrition and intestinal rehabilitation, comprising surgical reconstruction. The two main procedures are Longitudinal Intestinal Lengthening and Tailoring procedure (LILT) and Serial Transverse Enteroplasty Procedure (STEP). Transplantation is reserved as treatment of last resort, when intestinal rehabilitation has been unsuccessful. Short bowel syndrome is a debilitating disease, whose management stretches over the course of years. However, with the innovation of surgical techniques such as LILT and STEP, as well as advances in multidisciplinary intestinal rehabilitation centers, long-term survival rates are approaching 90%. In this review we discuss the current and future treatment options for SBS.

Improved enteral tolerance following step procedure: systematic literature review and meta-analysis.

Surgical management of children with short bowel syndrome (SBS) changed with the introduction of the serial transverse enteroplasty procedure (STEP). We conducted a systematic review and meta-analysis using MEDLINE and SCOPUS to determine if children with SBS had improved enteral tolerance following STEP. Studies were included if information about a child's pre- and post-STEP enteral tolerance was provided. A random effects meta-analysis provided a summary estimate of the proportion of children with enteral tolerance increase following STEP. From 766 abstracts, seven case series involving 86 children were included. Mean percent tolerance of enteral nutrition improved from 35.1 to 69.5. Sixteen children had no enteral improvement following STEP. A summary estimate showed that 87% (95% CI 77-95%) of children who underwent STEP had an increase in enteral tolerance. Compilation of the literature supports the belief that SBS subjects' enteral tolerance improves following STEP. Enteral nutritional tolerance is a measure of efficacy of STEP and should be presented as a primary or secondary outcome. By standardizing data collection on children undergoing STEP procedure, better determination of nutritional benefit from STEP can be ascertained.