Longitudinal Intestinal Lengthening And Tailoring 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.

Short Bowel Syndrome in neonates and early infancy

Short bowel syndrome (SBS) is the commonest cause of intestinal failure in neonates. SBS results from widespread damage to the small intestine, leading to loss of functional capacity of this organ. This is generally secondary to conditions like necrotizing enterocolitis, gastroschisis, intestinal atresia, and midgut volvulus. The small bowel usually adapts to this damage in due course of time. The clinician's role usually entails the management of parenteral nutrition and the fluid and electrolyte balance to tide over this phase. The management should be initiated as soon as the diagnosis is suspected, especially post-surgical resection of the bowel. This should comprise enteral nutrition, with proactive monitoring and supplementation of electrolytes and micronutrients. Intestinal lengthening procedures like the Serial transverse enteroplasty (STEP), and Longitudinal intestinal lengthening and tailoring (LILT) may be considered in infants, where medical therapy fails to correct the pathology. The intricate nature of the condition warrants a multi-disciplinary approach, involving clinicians, intensivists, and surgeons, which ensures the best neonatal outcomes, in terms of the survival rates in these babies.

Factors influencing enteral autonomy after autologous gastrointestinal reconstructive surgery: A two-centre UK perspective

Background and AimsThe implementation of multidisciplinary care and improvements in parenteral nutrition (PN) in patients with short bowel syndrome (SBS) have led to better outcomes and higher survivability. Autologous gastrointestinal reconstructive (AGIR) surgery can reduce the duration on PN and lead to earlier enteral autonomy (EA). Our aim was to investigate the effect of SBS aetiology and other predictors on the achievement of enteral autonomy following AGIR surgery. MethodsRetrospective review of all patients undergoing AGIR surgery in two tertiary paediatric surgical units, between 2010 and 2021. Continuous data is presented as median (range). ResultsTwenty-seven patients underwent 29 AGIR procedures (20 serial transverse enteroplasties (STEP), 9 longitudinal intestinal lengthening and tailoring (LILT)) at an age of 6.6 months (1.5 – 104.5). EA rate was 44% at 13.6 months after surgery (1 – 32.8). AGIR procedures achieved an increase in small bowel length of 70% (pre-operative 46.5 vs 77 cm, p = 0.003). No difference was found between STEP and LILT (p = 0.84). Percentage of expected small bowel length (based on the child's weight) was a strong predictor of EA (bowel length >15% – EA 80% vs bowel length ≤15% – EA 17%, p = 0.008). A diagnosis of gastroschisis showed a negative non-significant correlation with the ability to achieve EA (25% vs 60%, p = 0.12). Overall survival rate was 96%. ConclusionAGIR surgery is an important tool in the multidisciplinary management of children with SBS. Percentage of expected small length and aetiology of SBS are likely predictors of achievement of EA in patients undergoing AGIR surgery. Level of Evidence: IVRetrospective Case-Series

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.

Double barrel enteroplasty for the management of short bowel syndrome in children.

Currently, there are two well-established methods of bowel lengthening in patients with short bowel syndrome (SBS)-longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) [1-4]. Both procedures may carry a high reported morbidity and mortality of 30.2% and 14.4%, respectively [5]. We report the outcomes of a novel technique: double barrel enteroplasty (DBE) for autologous intestinal reconstruction. We performed a retrospective review of all ten patients who underwent DBE at our institution since 2011. All patients have SBS and were dependent on parenteral nutrition (PN) at the time of surgery. Etiologies were gastroschisis (n = 4), bowel atresia (n = 3), necrotising enterocolitis (n = 1), volvulus (n = 1), and near-total intestinal aganglionosis (n = 1). Patient survival, complications, and subsequent enteral autonomy were evaluated. All patients are alive with normal liver function. Five children achieved enteral autonomy, while the remaining are on weaning PN. There was no bleeding, anastomotic leak, perforation, infective complications, or intestinal necrosis. No patient has required a liver and/or intestinal transplant. Double barrel enteroplasty is technically feasible and safe. It has similar efficacy and may have fewer complications when compared with other methods of autologous intestinal reconstruction.

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.

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.

PO-0141 Overview On The Physiology Of Longitudinal Intestinal Lengthening And Tailoring In Short Bowel Syndrome

Background Multidisciplinary treatment of short bowel syndrome (SBS) has been a success story for paediatric surgery. Longitudinal intestinal lengthening and tailoring (LILT) is the archetypal autologous gastrointestinal reconstruction procedure, but despite it being performed routinely in intestinal rehabilitation departments, the physiological basis behind it is poorly understood. We attempt to analyse the features that improve intestinal adaptation and offer a LILT theoretical model. Methods Based on our clinical experience on 59 LILT procedures over 30 years, we set up a concise theoretical model that describes post-LILT bowel adaptation in a holistic way. Results Intestinal adaptation is affected by many factors, including remaining bowel length, the presence of the ileocaecal valve and colon, underlying disease process, nutritional status, hepatobiliary function, and bacterial flora. Lengthening techniques do not affect the absorptive surface, although changes in the microvilli have been observed in animal models. Dilated dysmotile intestinal segments benefit from tapering because reduction in the intestinal lumen calibre allows better peristalsis, as demonstrated by improvements in intestinal transit time. Tapering also helps prevention of stasis and subsequently reduction of bacterial overgrowth. Additionally, reduction in bowel diameter decreases the volume to surface ratio, theoretically allowing for more effective contact of the chime with the absorptive surface. Conclusions LILT has become an essential component in the management of SBS. The physiological principles described above provide a theoretical basis that explains the absorptive advantage offered by the LILT. Further research is necessary to quantify the effect of these procedures on the microscopic and hormonal levels.

PO-0912 Short Bowel State: Does Autologous Gastrointestinal Reconstruction Reduce Catheter-related Blood Stream Infections?

Background and aims Catheter-related blood stream infections (CRBSI) occur frequently in Short Bowel Syndrome (SBS) children on parenteral nutrition (PN). Central venous catheter (CVC) complication and complete loss of central venous access are indication for intestinal transplantation. Autologous gastrointestinal reconstruction surgery (AGIR) is mandatory in any chronically PN-dependent patient when there is substantial bowel dilation to reduce bacterial translocation. We reviewed patients who underwent lengthening surgery and calculated the rate of CRBSI pre and post surgery. Methods PN dependent children with SBS were identified. Inclusion criteria were CVC for PN administration pre and post-operatively, CVC removed after weaned off PN and having gained enteral autonomy. CRBSI episodes were defined as temperature above 38.0 °C, along with positive blood culture microbiological infection from the CVC. Results Nineteen patients were identified (male n = 13). Median gestation was 35 (33.5–36.5) completed weeks and birth weight 2080 g (1725–2374). Ten patients underwent tapering enteroplasty, eight Longitudinal Intestinal Lengthening and Tailoring (LILT) procedure, and one Serial Transverse Enteroplasty (STEP) procedure. Median duration of PN was 5.3 months (2.9–6.6) pre and 9.0 months (4.2–10.9) post surgery. A total of 115 septic episodes were confirmed (70 prior to surgery and 45 post surgery). The total rate of catheter related sepsis was significantly lower after AGIR compared to before it (p = 0.016). Conclusions CRBSI frequency in PN dependent patients with dilated bowel reduces after AGIR. AGIR appears associated with significantly reduced frequency of CRBSI in PN dependent children with bowel dilatation. These findings warrant further exploration in larger, preferably controlled studies.

Surgical Management of Short Bowel Syndrome

For patients with short bowel syndrome (SBS), surgery can play an important role in preventing, mitigating, and, in some cases, reversing intestinal failure (IF). During intestinal resection, bowel length should be conserved to the fullest extent possible to avoid dependence on parenteral nutrition (PN). Bowel salvage may be improved by initially preserving tissue of questionable viability and later reevaluating during "second-look" procedures. Once the patient is stabilized, ostomy reversal and recruitment of distal unused bowel should be prioritized whenever feasible. Following progression to IF, surgical management of SBS depends on the symptoms and anatomical characteristics of the individual patient. For carefully selected patients with rapid intestinal transit and dilated bowel, longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) procedures may provide benefit. Outcomes following STEP and LILT are generally similar, and the choice between these procedures may rest on surgeon preference. For patients with rapid intestinal transit in the absence of bowel dilation, segmental reversal of the small bowel may reduce PN requirements. Intestinal transplantation is the standard of care for patients in whom intestinal rehabilitation attempts have failed and who are at risk of life-threatening complications of PN. Because patients awaiting isolated intestine transplant show increased survival compared with patients awaiting combined intestine-liver transplant, early referral of appropriate patients, before the development of advanced liver disease, is critical to enhancing patient outcomes.

Altersabhängige Viszeralmedizin: pädiatrische Viszeralmedizin - viszeralmedizinische Pädiatrie - Überlegungen zum Kurzdarmsyndrom im Kindesalter

There are several reasons for the possible development of a short bowel syndrome, which, however, occurs only rarely. The main causes consist of extended intestinal resections in cases of congenital anomalies (e.g., gastroschisis, intestinal atresia or dysplasia) or ischaemic lesions due to a volvulus. In addition, an intestinal stoma at a more upper segment of the GI tract can result in the functional manifestation of a short bowel syndrome. The differentiation between temporary and persisting types is essential for initiation of an adequate treatment. Loss or exclusion of organic resorption area at the inner surface of the (small) intestine can be associated with numerous pathological consequences requiring treatment. As a principle consideration from the paediatric point of view, the potential of intestinal adaptation needs to be assessed. Basic conservative treatment options are parenteral and enteral nutrition regimens, in particular, to prevent complications (such as D-lactate acidosis). The main surgical approaches are the procedures called LILT (longitudinal intestinal lengthening and tailoring) according to Bianchi and STEP (serial transverse enteroplasty). The technique to create intestinal segments of antiperistalsis has been abandoned. Because of the encouraging results of intestinal transplantation, this novel treatment option has gained greater attention over the past few years and is now also an option for paediatric patients. The limiting factor and thus major complication is the central venous catheter for long-term treatment. Catheter-related complications are still the main reason for a considerable mortality in these children.

Redilation of bowel after intestinal lengthening procedures—an indicator for poor outcome

Background/Purpose Despite a good understanding of short-term outcomes of the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) procedures, limited data exist on long-term complications. Methods This is a 15-year single-institution retrospective chart review of patients who underwent an intestinal lengthening procedure (ILP). Long-term ILP-related complications, their interval to development, patients' ability to wean off parenteral nutrition (PN), and the need for further procedures were analyzed. Results Of 119 patients with short bowel syndrome, 14 had undergone an ILP. Seven patients had an LILT, and 9 patients had a STEP, including repeat ILPs on the same patient. Overall, 93% of patients had complications. Four patients in the LILT group and 3 patients in the STEP group weaned off PN. Eight patients (57%) experienced bowel redilation after their ILP. The 2 deaths in the study came from this group. Seven required another abdominal operation and only one weaned off PN. There were no significant differences in mean bowel length between the redilated group and the non–re-dilated group. Conclusions Complications are common after ILPs, and patients who redilated their bowel after ILP did clinically worse than those who did not.

Autologous intestinal reconstruction surgery for intestinal failure management

Multidisciplinary management of intestinal failure has progressed over the past 30 years, facilitating the transition to enteral autonomy in many pediatric patients. However, there remains a select group of patients who reach a plateau in advancement of their enteral nutrition. Numerous surgical options have been pursued to attempt to slow intestinal transit, taper dilated bowel, and promote intestinal adaptation. This review highlights the current literature on autologous intestinal reconstruction surgery, focusing on the two most commonly performed procedures, the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP). LILT and STEP remain viable options to treat medically refractory short bowel syndrome. There is over 20 years of experience with the LILT procedure in the literature, with one large series showing that 67% of patients eventually transitioned to full enteral nutrition. The International STEP Data Registry reported a weaning rate of 34% after median follow-up of 12.6 months. Repeat STEP has been described as a possible treatment for the redilation that occurs after both LILT and STEP. LILT and STEP may facilitate enteral feeding advancement in patients with medically refractory short bowel syndrome.

Long‐Term Outcome in Patients With Short Bowel Syndrome After Longitudinal Intestinal Lengthening and Tailoring

Longitudinal intestinal lengthening and tailoring (LILT) is a well-established surgical treatment for short bowel syndrome. It has been shown to enhance peristalsis, decrease bacterial overgrowth, and extend mucosal contact time for nutrients. We present the results of a long-term follow-up of patients who underwent LILT and define prognostic parameters for the survival of these patients. Between 1987 and 2006, 53 patients underwent LILT in our institution. The main diagnoses were gastroschisis, intestinal volvulus, intestinal atresias, and necrotizing enterocolitis. LILT was performed at a mean age of 24 months (range 4144 months). The follow-up time was 79.76 months (range 6234 months). After LILT, 41 of 53 patients survived, and 36 of 41 surviving patients were successfully weaned from parenteral nutrition (PN). In long-term follow-up 79% stayed free of PN. The overall survival rate was 77.36%. Weight gain occurred in 58% of the patients after LILT. The quality of life after LILT is on a high level, with most patients having normal physical strength and participating in normal social life and education. Prognostic factors for survival after LILT in short bowel syndrome are length of small intestine (0.06582 + 0.0131 x bowel cm), length of large bowel (P = 0.039), preoperative liver function, and successful weaning from PN within 18 months postoperatively (P = 0.0032). Patients undergoing LILT in short bowel syndrome have a high survival rate, weight gain, and a high quality of life. Autologous gastrointestinal reconstruction remains therefore the first choice in the treatment of patients with short bowel syndrome.

The Impact of Longitudinal Intestinal Lengthening and Tailoring on Liver Function in Short Bowel Syndrome

Short bowel syndrome is a functional or anatomic loss of major parts of the small bowel leading to severe malnutrition. The limiting factor for the survival of these patients remains parenteral nutrition-related liver damage leading to end-stage liver failure. Longitudinal intestinal lengthening and tailoring (LILT) has been proven to enhance peristalsis, to decrease bacterial overgrowth and to extend the mucosal contact time for the absorption of nutrients. The aim of this study was to show the impact of LILT on the development of parenteral nutrition-related liver damage. A cohort of 55 patients with short bowel syndrome managed with LILT in our institution between 1987 and 2007 was retrospectively reviewed. LILT was performed at a mean age of 24 months (range 4 - 150 months). Mean follow-up time was 83.76 months (range 5 - 240 months). We obtained reliable data from 31 patients with regard to liver enzymes and function parameters in blood samples before LILT and at the present time. Liver biopsy was performed in 14 patients prior to LILT. Liver enzymes ALAT (mean 121 U/l), ASAT (mean 166 U/l) and bilirubin (mean 2.49 mg/dl) were elevated preoperatively in 27/31 children. After the lengthening procedure, ALAT (mean 50 U/l), ASAT (mean 63 U/l) and bilirubin (mean 1.059 mg/dl) normalized except in 5 of 8 patients who could not be weaned from parenteral nutrition after LILT. Liver function parameters such as the international normal ratio (INR) were slightly elevated in 5/31 patients. Albumin was generally low, probably due to parenteral nutrition. Liver biopsy was performed in 14 patients preoperatively, showing 4 patients with low-grade, 6 patients with intermediate and 4 patients with high-grade fibrosis. End-stage liver disease with cirrhosis was an exclusion criterion for LILT. All patients with liver fibrosis showed a normalization of liver enzymes when they were weaned from parenteral nutrition. But patients with higher grade liver fibrosis tend to develop more complications perioperatively. After LILT, all patients with liver fibrosis who could be weaned from parenteral nutrition showed a normalization of liver enzymes. Preoperative liver biopsy is mandatory in order to differentiate reversible liver fibrosis from end-stage liver disease. A higher grade of liver fibrosis and elevated INR has been shown to be a sensitive parameter for peri- and postoperative complications.

Evaluation of intestinal absorption after longitudinal intestinal lengthening for short bowel syndrome

Purpose The aim of this study was to answer if the longitudinal intestinal lengthening and tailoring (LILT) by Bianchi, modified by Aigrain, can allow the child to be weaned from parenteral nutrition (PN) and if the length of the bowel after the procedure can influence the results of the absorption test such as Schilling or d-xylose test. Patients and Methods We reviewed the files of 7 children who have had LILT from 1980 to 2003. We performed to explore 2 intestinal function tests: the d-xylose and the Schilling tests. Both were performed early (during the first year after the procedure) and late (during the second year) after the LILT. We used the χ 2 and Bartlett's correlation tests for statistical analysis. Results There were 6 boys and 1 girl. The surgical indication was short bowel syndrome with parenteral nutrition owing to multiple intestinal atresia (2 cases), severe necrotizing enterocolitis with volvulus (1 case), necrotizing enterocolitis (1 case), intestinal atresia with gastroschisis (2 cases), and volvulus owing to malrotation (1 case). The length of the bowel was significantly different before and after LILT ( P < .0001). After LILT, the length of the bowel was significantly correlated with the percentage of PN on energy at 6 months ( P = .02) and at 12 months ( P = .001). Moreover, the length of the bowel after the procedure was significantly correlated with the results of the d-xylose test during the first year ( P = .002) but not with the results after the second year. The length after lengthening influenced neither the results of the Schilling test during the first nor those of the second year after. Four patients were weaned from the PN 21 months in average after the LILT (57%); 1 was not because we had only a 2-month follow-up. The average follow-up was 111 (5 months; range, 4- 206). Conclusion Longitudinal intestinal lengthening and tailoring for short bowel syndrome is a good option to allow children to be weaned from the PN. The length of the bowel after the procedure can influence the absorption test such as d-xylose during the first postoperative year but not during the second and does not influence the Schilling test. We think it is not necessary to perform these tests during the follow-up of these patients.

Longitudinal intestinal lengthening and tailoring: results in 20 children.

Longitudinal intestinal lengthening and tailoring (LILT) is increasingly favoured as a treatment for short-bowel syndrome. In a personal series, 20 children underwent LILT between 1962 and 1997. There was negligible morbidity (hemiloop anastomotic stenosis in 2) and no operative mortality. At a mean follow-up of 6.4 years overall survival was 45%, and certain features were clearly related to outcome. Survivors had > 40 cm of residual small bowel (commonly jejunum) and had little hepatic dysfunction despite parenteral nutrition of similar time and nature as non-survivors. Children who did not survive had < 40 cm of residual small bowel and developed early lethal hepatic dysfunction of unclear aetiology. Outcome did not seem to be influenced by the presence of the ileocaecal valve or the length of residual colon. Children born with short-bowel should be offered LILT at an early stage when still in good physical condition, so as to avoid liver-damaging intraluminal stasis and bacterial translocation and to enhance intestinal adaptation and hepatoprotective factors.