Bowel Lengthening Procedures Research Articles

Serial Transverse Enteroplasty (STEP) versus Longitudinal Intestinal Lengthening and Tailoring (LILT): Systematic Review and Meta-Analysis of Outcomes and Complications

Abstract Background The prevalence of SBS is 3-4 per million. It occurs in about 15% of adult patients who undergo intestinal resection, with 3/4th of these cases resulting from massive intestinal resection and 1/4th from multiple sequential resections. About 70% of patients in whom SBS develops are discharged from the hospital and a similar percentage remain alive a year later. This improved survival rate has been achieved primarily by the ability to deliver long-term nutritional support. Objective To compare between Serial Transverse Enteroplasty (STEP) and Longitudinal Intestinal Lengthening and Tailoring (LILT) in the incidence of complications and clinical prognosis regarding morbidity and mortality in patients with Short Bowel Syndrome (SBS). Patients and Methods The search was carried out using the electronic national library of medicine PubMed database, Cochrane library and manual reference of articles published on serial transverse enteroplasty (STEP) versus longitudinal intestinal lengthening and tailoring (LILT) in management of patients with SBS. Conclusive search was done and duplicate studies were excluded. Data Sources Medline databases (PubMed, Medscape, ScienceDirect. EMF-Portal) and all materials available on the Internet till 2022. Results LILT and STEP are both valuable treatment strategies used in the management of pediatric SBS. However, currently, it is not possible to advise surgeons on accurate patient selection and to predict the result of either intervention. There is very limited published data on bowel lengthening procedure exclusively in adults. Most reported series exclusively or predominantly discuss the pediatric population with some adults included in one series and two additional single case reports. Conclusion Both STEP and LILT are potentially safe and effective methods for the treatment of SBS, under the conditions that the surgical techniques required have been mastered and the indications for surgery are observed. This review should be a gateway to open the scope on the field of Autologous Intestinal Reconstruction Surgeries (AIRS) and plan for a more detailed prospective studies with close monitoring of the data regarding outcomes, complications and follow up.

Outcomes of Children With Short Bowel Syndrome: Experiences in a Multidisciplinary Intestinal Rehabilitation Unit Over Two Decades

BackgroundShort bowel syndrome (SBS) is a rare but serious form of organ failure, and patients with SBS depend on total parenteral nutrition (PN) to maintain growth and development. The present study aimed to evaluate the experiences and outcomes of children with SBS managed by a multidisciplinary intestinal rehabilitation programme in a tertiary paediatric centre. MethodsA retrospective single-centre analysis of all paediatric patients with a clinical diagnosis of SBS between 2001 and 2022 was performed. Clinical outcomes and their predictors were extracted and analysed. ResultsOf the 64 children included in the study, 43 (67%) had extensive necrotising enterocolitis. The median bowel length was 45 cm (interquartile range (IQR) = 18–65) and 18.9% (IQR = 10–28.5) of the expected length based on age. Over a mean follow-up period of 8.9 years, 57 patients (89%) survived, and 50 (78%) weaned off PN. The presence of intestinal failure-associated liver disease (IFALD) (OR = 6.375, p = 0.02) and patients managed before the introduction of fish oil-based PN in 2007 (OR = 5.895, p = 0.001) were significant predictors of mortality. There was an overall improvement in survival over time (p = 0.003). Ultrashort bowel length was not associated with significantly higher mortality (OR = 1.1, p = 0.65) but was a poor prognostic factor for weaning off PN (OR = 3.57, p = 0.004). Among all patients who weaned off PN, two had bowel lengthening procedures and one received a glucagon-like peptide 2 (GLP-2) analogue. ConclusionsA multidisciplinary intestinal rehabilitation programme offers a comprehensive approach for patients with SBS and has been shown to be effective with favourable outcomes. Improvements in the choice of PN and the development of new treatment strategies potentially improved the survival and enteral autonomy of SBS patients. Level of EvidenceIII.

Nutritional Outcomes of Bowel Lengthening Procedure in Patients with Short Bowel Syndrome.

Although parenteral nutrition (PN) significantly improves mortality rates in pediatric short bowel syndrome (SBS), long-term PN has many possible complications and impacts quality of life. Bowel lengthening procedures (BLPs) increase the contact surface of food and the intestinal mucosa and enable the better absorption of nutrients and liquids, possibly leading to a PN decrease. We retrospectively reviewed the data of patients with short bowel syndrome who underwent BLPs in the period from January 2016 to January 2022. Overall, eight patients, four male, five born prematurely, underwent BLPs. There was a significant decrease in the percentage of total caloric intake provided via PN and PN volume after the BLPs. The more evident results were seen 6 months after the procedure and at the last follow-up, which was, on average, 31 months after the procedure. Two patients were weaned off PN after their BLPs. Patients remained well nourished during the follow-up. The BLP led to a significant decrease in PN needs and an increase in the food intake; however, significant changes happened more than 6 months after the procedure.

Intestinal Bowel Lengthening within the First 6 Months of Life: Institutional Experience and Review of the Literature.

Management of short bowel syndrome in children has been surrounded by much debate with timing of the lengthening procedure still controversial. Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before 6 months of age. The purpose of this paper is to report the institutional experience in EBLP and to review the literature on this subject to identify common indications. An institutional retrospective analysis of all the intestinal lengthening procedures was performed. Furthermore, an Ovid/Embase search regarding children who underwent bowel lengthening in the past 38 years was conducted. Primary diagnosis, age at procedure, type of procedure, indication, and outcome were analyzed. Ten EBLP were performed in Manchester from 2006 to 2017. Median age at surgery was 121 days (102-140), preoperative small bowel (SB) length was 30 cm (20-49) while postoperative SB length was 54 cm (40-70), with a median increased bowel length of 80%. Ninety-seven papers were reviewed, with more than 399 lengthening procedures performed. Twenty-nine papers matched criteria with more than 60 EBLP were observed of which 10 were performed in a single center from 2006 to 2017. EBLP was performed due to SB atresia, to excessive bowel dilatation or failure to enteral feeds, at a median age of 60 days (1-90). Serial transverse enteroplasty was the most frequent procedure used lengthening the bowel from 40 cm (29-62.5) to 63 cm (49-85), with a median increased bowel length of 57%. This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered, only in cases of actual necessity after review of qualified intestinal failure center.

Transluminal stenting: An intraoperative adjunct for preserving bowel length in the surgical management of necrotizing enterocolitis

Surgical management of necrotizing enterocolitis (NEC) presents a morbid dilemma: it requires maximal preservation of the small bowel length to prevent complications such as short bowel syndrome while ensuring the resection of all necrotic and non-viable bowel segments. We performed a retrospective review of four single institution cases that underwent multiple small bowel resections and anastomoses for NEC. Transluminal stenting was used prior to restoring continuity to preserve small bowel length. All four infants had >40% of predicted bowel length after their procedure, and none of the infants in the first three cases required repeat surgery after the stent removal and returned and restored small bowel continuity. The stent in the fourth case is the most recent and will remain in place for six weeks. The mean time to stent removal was 58 days for the first three cases. All four infants are alive with only mild developmental delays and are tolerating oral feeds without any parental nutrition dependency. Our experience shows that transluminal stenting is an adequate bowel lengthening procedure to lessen the risk of short bowel syndrome without increasing mortality.

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.

Early Bowel Lengthening Procedures: Bi-Institutional Experience and Review of the Literature.

Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before six months of age. The purpose of this paper is to compare our experience with literature on this subject to identify common indications. A bi-institutional retrospective analysis was performed. Diagnosis, type of surgery, age at procedure and outcomes were analysed. Eleven EBLP were performed in Manchester and Florence from 2006 to 2021. The median age at surgery was 126 days (102–180), pre-operative median short bowel (SB) length was 28 cm (17–49) with a post-operative median increase of 81%. Furthermore, a PubMed/Embase search was undertaken regarding bowel lengthening procedures performed in the last 40 years. Sixty-one EBLP were identified. The median age was 60 days (1–90). Serial transverse enteroplasty (STEP) was the most frequent procedure used, with a median increased bowel length of 57%. This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered only in cases of actual necessity and performed in a qualified intestinal failure centre.

A Comprehensive Review of Intestinal Atresias

A Comprehensive Review of Intestinal Atresias

Atretic midgut; a rare congenital cause of short bowel syndrome: Case report from Ethiopia

Abstract Background Neonatal short bowel syndrome is a devastating condition that carries a high mortality in the developing world. Among the congenital causes of this condition is midgut atresia which is exceedingly rare and often associated with gastroschisis. Case presentation 7days old male neonate born to 34 yrs old Para II mother who had uneventful antenatal follow up at gestational age of 32+6 weeks with a birth weight of 1600 gm. He presented with bilious vomiting and abdominal examination revealed no evidence of abdominal wall defect. Abdominal x-ray showed triple bubble appearance. After resuscitation he was explored with a finding of atretic midgut located between blindly ending duodenum and distal third of the transverse colon for which duodeno transverse anastomosis was done and he was put on progressive oral feeding postoperatively despite which he passed away after developing several complications of short bowel syndrome. Conclusion This case report represents a rare congenital cause of neonatal short bowel. The outcome of such neonates in the absence of bowel lengthening procedures, parenteral nutrition and intestinal rehabilitation centers is expected to be dismal in the developing countries emphasizing the importance of proper prenatal counseling.

320.9: The Double-Barrel Enteroplasty: A Novel Intestinal Lengthening Procedure for Short Bowel Syndrome

Advances in the management of Short bowel syndrome (SBS) has resulted in children with shorter lengths of small bowel coming off TPN and achieving enteral autonomy. Although existing bowel lengthening procedures such as the Bianchi Longitudinal Intestinal Lengthening and Tailoring (LILT) procedure and Serial Transverse Enteroplasty (STEP) have enjoyed moderated degrees of success, they have not been without complications and better alternatives are still being sought after. We present our experience with a novel bowel-lengthening procedure for SBS termed the double-barrel enteroplasty (DBE) that is simpler to perform, is less disruptive to anatomy and has the potential to achieve similar to superior results to existing bowel lengthening procedures. Methods: Ten patients have undergone the DBE at the Children’s Hospital at Westmead between January 2011 and November 2018. Baseline characteristics, complications, time to TPN weaning and growth parameters were recorded prospectively. Results: The mean age at operation was 21 months (range 4–41 months). Mean pre-operative small bowel length was 74.6cm (36-167cm) with a mean length of 35.6cm (17-60cm) undergoing enteroplasty. Mortality and progression to transplantation remains zero. Six patients have achieved complete enteral autonomy within 2.8 months (0.5–5 months). The most recent patients are still weaning TPN. All patients have normalising growth parameters. One patient required an extension of the DBE for on-going proximal dilatation. Discussion: The DBE is a safe, effective and potentially superior alternative to existing bowel lengthening procedures for SBS. Advantages include its simplicity to perform, less disruption of bowel and mesentery, less anastomoses, decreased possibility of stenosed segments and maintenance of neuromuscular integrity.

200.4: Serial Transverse Enteroplasty (STEP) for the Short Gut Syndrome (SGS) Patients with Gut Failure (GF)

Introduction: With recent evolution of gut rehabilitation, bowel lengthening is increasingly utilized to restore nutritional autonomy in patients with GF. This is the first largest single center experience with STEP particularly among adults with different causes of SGS including Crohn’s and allograft rejection. Methods: Over 7 years, 65 SGS-GF-patients received a total of 79 STEP procedures. Sixty one(94%) patients were adults and 4(6%) were children with an age ranging from 2 to 79 years. All patients suffered loss of nutritional autonomy with the requirement for total parenteral nutrition (TPN), or IVF-micronutrient replacement. The leading causes were vascular occlusion (n=22), surgical adhesions (n=13), and Crohn’s disease (n=12). Interestingly, the procedure was performed in an isolated intestinal allograft after partial recovery from acute rejection. The mean pre-STEP length of the residual bowel was 71 ± 13 cm with partially or fully preserved colon in 54(83%) and intact ileo-cecal valve in 14(22%) Reestablishment of gut continuity was accomplished preoperatively in 24(37%) and simultaneously performed in 41(63%). The procedure was reproducible in a total of 13(20%) patients. GLP2 was used as an adjunct therapy before and/or after the STEP procedure in 11(17%) patients. Results: With a total of 79 STEP-procedures and overall total number of 2 to 36 cuts, there was an increment in the bowel length ranging from 2 cm to 50 cm. The increase in the bowel length was influenced by length of the residual bowel, intestine diameter and the cumulative number of the transverse cuts. With a mean follow up of 19 ± 7 months, a total of 28(43%) patients achieved full nutritional autonomy with discontinuation of TPN therapy. The remaining 37(57%) experienced partial reduction in the TPN and IVF support. Length of residual bowel, volume/caloric contents of initial TPN therapy, and presence of the ileo-cecal valve were significant predictors of successful outcome. Conclusions: STEP along with autologous gut reconstruction is an effective bowel lengthening procedure for the management of SGS –GF patients. The operation is also technically feasible in Crohn’s disease patients and recipients of intestinal allografts.

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.

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.

Ultra-short bowel syndrome during infancy: improving outcomes and novel therapies.

Ultra-short bowel syndrome is relatively rare and has not yet been extensively reported. In ultra-short bowel syndrome, poor absorption of nutrients and dysmotility, interfere with fluid, energy, electrolyte and micronutrient balance. Patients with this disorder are managed through prolonged parenteral nutrition with the ultimate goal of achieving enteral autonomy. Overall outcomes of these patients are dependent on postsurgical bowel anatomy (residual length, intact colon, ostomy closure timing), incidence of sepsis, and care by a multidisciplinary specialized team. Over the years, standardization of management has improved outcomes. This includes central line care, lipid alternatives, enteral therapy, medications (antidiarrheal agents, acid suppression medications, bile acid binding salts, and enteral antibiotics) including Teduglutide. Bowel lengthening procedures have also proven beneficial, and finally bowel transplant does remain an option for a patient in whom rehabilitation has failed. Although there are many factors that influence outcomes of ultra-short bowel patients, novel therapies such as Teduglutide have been introduced with the aim of improving intestinal adaptation. Surgical lengthening and transplant are viable options in the setting of failed rehabilitation.

Bianchi’s bowel lengthening procedure for short bowel syndrome: Report of two cases and approach of pediatric surgeons in Turkey

Bianchi’s bowel lengthening procedure for short bowel syndrome: Report of two cases and approach of pediatric surgeons in Turkey

Autologous intestinal reconstruction surgery as part of comprehensive management of intestinal failure.

Pediatric intestinal failure (IF) remains to be associated with significant morbidity and mortality, the most frequent underlying etiologies being short bowel syndrome (SBS), and primary motility disorders. Management aims to assure growth and development, while preventing complications and facilitating weaning off parenteral support (PS) by fully utilizing adaptation potential of the remaining gut. Probability of survival and weaning off PS is improved by coordinated multidisciplinary intestinal rehabilitation combining individualized physiological enteral and parenteral nutrition (PN), meticulous central line care and medical management with carefully planned surgical care. Increasing evidence suggests that autologous intestinal reconstruction (AIR) surgery is effective treatment for selected short bowel patients. Bowel lengthening procedures normalize pathological adaptation-associated short bowel dilatation with potential to support intestinal absorption and liver function by various mechanisms. Although reversed small intestinal segment, designed to prolong accelerated intestinal transit, improves absorption in adult SBS, its feasibility in children remains unclear. Controlled bowel obstruction to induce dilatation followed by bowel lengthening aims to gain extra length in patients with the shortest duodenojejunal remnant. Reduced PS requirement limits the extent of complications, improving prognosis and quality of life. The great majority of children with SBS can be weaned from PS while prognosis of intractable primary motility disorders remains poor without intestinal transplantation, which serves as a salvage therapy for life-threatening complications such as liver failure, central vein thrombosis or recurrent bloodstream infections.

Short bowel syndrome – surgical perspectives and outcomes

Abstract Short bowel syndrome is a rare but challenging problem. A functional definition of the condition is that the patient has insufficient bowel to enable absorption of adequate fluids and nutrients to sustain life and growth. Aetiology may be congenital – via multiple atresias or gastroschisis; or acquired secondary to NEC or malrotation and volvulus. Treatments such as parenteral nutrition, surgical bowel lengthening procedures and intestinal transplant have improved prognosis for these patients significantly over the last decade. However, serious nutritional and infective complications remain. A structured feeding programme and timely interventions will produce the best outcomes for this challenging group of patients.

Clinical problem-solving: Short bowel syndrome in an infant

The present case report describes the clinical problems encountered over a five-month period in an infant born with jejunal atresia, extensive midgut volvulus and a microcolon. After an initial surgical resection, the patient had no remaining ileum and his ileocecal valve was also removed. The patient had 35 cm of jejunum, which was successfully lengthened to 60 cm using enteral nutrition and two bowel-lengthening procedures (serial transverse enteropathy procedures). Bouts of cholestatic liver disease, sepsis and small bowel bacterial overgrowth were vigorously treated. The patient was discharged at 5.5 months of age and is now 40 months of age. He is at the 50th percentile for both height and weight, and is developing normally. The outcome for infants with short bowel syndrome has improved significantly in the past few years due to intestinal rehabilitation programs, which integrate nutritional, surgical and pharmacological approaches tailored to individual needs.

The Use of Multiple Serial Transverse Enteroplasty (STEP) Procedures for the Management of Intestinal Atresia and Short Bowel Syndrome

The serial transverse enteroplasty procedure (STEP) was introduced as a bowel-lengthening procedure to reduce complications related to short bowel syndrome (SBS). Although some have described it as a useful adjunct to the Bianci procedure, others have acknowledged it as a primary procedure. We present a case of jejunal atresia in which two STEP procedures were performed 7 months apart to increase small bowel length. A 1-day-old, term girl presented with a known bowel obstruction diagnosed in utero. A laparotomy revealed a Type IIIb jejunal atresia with no remaining small bowel or cecum. A STEP procedure with an end jejunostomy and ascending colon mucous fistula lengthened the small bowel from 35 to 50 cm. A repeat procedure 7 months later lengthened it to 89 cm. The STEP procedure results in slower intestinal transit time and increases enterocytes contact with oral intake. We performed it during our initial exploration to increase small bowel size by 30 per cent. A repeat procedure 7 months later increased length to 89 cm. The use of multiple, staged STEP procedures avoided the need for bowel transplantation and long-term total parenteral nutrition dependence, demonstrating its effectiveness as a primary procedure for the surgical management of SBS.

Clinical experience of complex jejunal atresia

Small intestinal atresia is relatively common anomaly that causes intestinal obstruction in neonates. Although surgical interventions are usually successful, critical problems could raise in certain cases. This study aimed to identify the distinct clinical characteristics of complex cases of jejunal atresia by retrospective analysis. Overall, 91 cases of small intestinal atresia, which occurred in infants between 2001 and 2010 at Pusan National University Children's Hospital, were reviewed retrospectively. The clinical characteristics of complex jejunal atresia were analyzed. Of the 91 small intestinal atresias, 11 cases of complex jejunal atresia were found: high jejunal atresia with distal deletion, 3; high jejunal atresia with distal multiple atresias, 4; jejunal atresia with distal apple peel appearance, 1; jejunal atresia with colonic atresia, 1; jejunoileal atresia with distal volvulus, 2. Short bowel syndrome was found in four patients and bowel-lengthening procedure was performed in all. Three patients presented with an adhesive intestinal obstruction during the early postoperative period. Postoperative mortality occurred in one patient with distal volvulus. From a surgical perspective, complex jejunal atresia can cause many critical problems after the correction operation. An aggressive and multidisciplinary approach is necessary for managing this condition.