Longitudinal Intestinal Lengthening 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.

Engineering Auxetic Cylinders and Intestine to Improve Longitudinal Intestinal Lengthening and Tailoring Procedure.

Auxetic materials can be exploited for coupling different types of tissues. Herein, we designed a material where the microorganism metabolic activity yields the formation of buckled/collapsed bubbles within gelling silicone cylinders thus providing auxetic properties. The finite element model of such hollow auxetic cylinders demonstrated the tubular structure to promote worm-like peristalsis. In this scenario, the described hybrid auxetic structures may be applied to the longitudinal intestinal lengthening and tailoring procedure to promote enteral autonomy in short bowel syndrome. The presented material and analytical design synergistic approach offer a pioneering step for the clinical translation of hybrid auxetic materials.

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

Surgical Treatment of Short Bowel Syndrome-The Past, the Present and the Future, a Descriptive Review of the Literature.

Short bowel syndrome (SBS) is a devastating disorder with both short- and long-term implications for patients. Unfortunately, the prevalence of SBS has doubled over the past 40 years. Broadly speaking, the etiology of SBS can be categorized as congenital or secondary, the latter typically due to extensive small bowel resection following diseases of the small intestine, e.g., necrotizing enterocolitis, Hirschsprung’s disease or intestinal atresia. As of yet, no cure exists, thus, conservative treatment, primarily parenteral nutrition (PN), is the first-line therapy. In some cases, weaning from PN is not possible and operative therapy is required. The invention of the longitudinal intestinal lengthening and tailoring (LILT or Bianchi) procedure in 1980 was a major step forward in patient care and spawned further techniques that continue to improve lives for patients with severe SBS (e.g., double barrel enteroplasty, serial transverse enteroplasty, etc.). With this review, we aim to provide an overview of the clinical implications of SBS, common conservative therapies and the development of operative techniques over the past six decades. We also provide a short outlook on the future of operative techniques, specifically with respect to regenerative medicine.

Autologous Intestinal Reconstruction Surgery in Short Bowel Syndrome: Which, When, and Why.

Short bowel syndrome (SBS), secondary to any natural loss or after any extensive bowel resection for congenital malformations or acquired disease, is the most common cause of intestinal failure in children. Extensive introduction of parenteral nutrition (PN) has dramatically changed the outcome of these patients, allowing for long-term survival. The main goal in children with SBS remains to be increasing enteral tolerance and weaning from PN support. Post resection intestinal adaptation allows for achievement of enteral autonomy in a subset of these patients, but the inability to progress in enteral tolerance exposes others to long-term complications of PN. Autologous intestinal reconstruction surgery (AIRS) can facilitate the fulfilment of enteral autonomy, maximizing the absorptive potential of the remaining gut. All the different intestinal reconstruction techniques, from simple procedures like tapering, reversed segments, and colon interposition, to more complex lengthening procedures (LILT: longitudinal intestinal lengthening and tailoring, STEP: serial transverse enteroplasty, and SILT: spiral intestinal lengthening and tailoring) and techniques designed for peculiar problems like controlled intestinal tissue expansion or duodenal lengthening are presented. AIRS indications, clinical applications, and results reported in the literature are reviewed.

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.

Modified Antimesenteric Tapering Enteroplasty: An Alternative Technique for the Treatment of Dysfunctional Anastomosis in Patients With Short Bowel.

Several techniques have been described to taper the dilated small bowel to improve intestinal motility and decrease complications related to overdilated small bowel, including longitudinal intestinal lengthening and tapering, serial transverse enteroplasty, and spiral intestinal lengthening and tailoring. We propose an alternative technique designed to optimize bowel function and minimize the effects of recurrent small-bowel bacterial overgrowth in patients with short or ultra-short gut syndrome and dysfunctional anastomosis with maintenance of the actual absorptive surface. The dilated side-to-side anastomosis is identified, and the mesentery leaves from both the proximal and distal small-bowel loops are separated by using blunt dissection. The previous anastomosis is divided longitudinally with a GI stapler. Once the small-bowel transection is completed, 2 separate blind loops of intestine are created, each one with half the circumference of the dilated side-to-side anastomosis. The antimesenteric stapled line is then reinforced with an outer layer of running suture. The blind loops of the tapered small bowel are then trimmed and anastomosed in an end-to-end isoperistaltic fashion in 2 layers. There were no postoperative complications. The length of the tapering ranged from 10 to 23 cm, corresponding to approximately 16% (range, 13%-20%) of the remaining small-bowel length. Three of 4 patients presented significant improvement of their symptoms and were able to have their parenteral support discontinued. Modified antimesenteric tapering enteroplasty is an alternative technique to improve intestinal motility and treat patients with short-bowel syndrome and dysfunctional side-to-side anastomosis without the need for further small-bowel resection. This bowel-sparing technique represents a valuable option in the armamentarium of the surgeon who manages patients with intestinal failure.

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.

Long-term results of surgery for bowel lengthening: how many transplants are avoided, for which patients?

One of the biggest successes of intestinal rehabilitation programs is that more patients achieve enteral autonomy without transplantation. Many factors are responsible of this accomplishment including new parenteral formulas, better catheter management, surgical management, and the experience of the teams. The purpose of this review is to analyze recent published papers regarding intestinal lengthening procedures trying to find out how many transplantations are avoided and for which patients. A trend towards performing less intestinal transplants has been identified in the last years. The general improvement of intestinal rehabilitation accounts for this step forward. However, the role of intestinal lengthening has not been clarified. Surgical techniques for autologous reconstructive surgery are not limited to bowel lengthening. Longitudinal intestinal lengthening and tailoring and serial transverse enteroplasty offered good results in terms of intestinal adaptation, long-term survival, and subsequent need of intestinal transplantation. In recent series, less than one quarter of patients who underwent intestinal lengthening required salvage intestinal transplantation.

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.

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.

PO-0116 Results Of A Single Centre Register For Paediatric Intestinal Failure

Aim Intestinal failure (IF) is a rare condition requiring long term parenteral nutrition (PN). The complex management of IF needs a multidisciplinary approach by expert team of paediatricians, surgeons, gastroenterologists, nurses and dieticians. The risks of medical, surgical and nutritional complications must be prevented or managed appropriately. The primary aims of the register were to list the patients, the aetiology of IF, the therapeutic approach of the disease and the follow-up. Methods We collected retrospectively the data of 21 patients followed in our centre for IF between 1999 and 2013. Results Twenty-one patients were included, their age ranged between 2 mo and 15 yrs (mean 7.4 yrs). The causes of IF were short bowel syndrome (16), malabsorption (1) and intestinal motility disorders (4). Sex ratio was 0.29 (F/M). All children required home PN. The duration of PN was (43.1 ± 46.9 mo). 10/21 (47.6%) patients could be totally weaned from PN after 25.4 ± 33.5 months, 5/21 (24%-after 109 ± 74.9 months) were off PN after longitudinal intestinal lengthening tapering (4LILT) or 1 after combined serial tapering enteroplasty (STEP) and (LILT). PN partially weaned in the 6/21 (23.8%) remaining patients. Conclusions Results of this single centre experience demonstrate that with a proactive multidisciplinary IF management, including surgical rehabilitation procedures, the majority of patients have obtained partial or full enteral autonomy. Extension of this register to other centre in Belgium or Europe would be interesting and probably enhance the quality of patient follow-up.

Update on surgical therapies for intestinal failure.

Parenteral nutrition enables long-term survival in patients with intestinal failure; however, it is associated with life-threatening complications necessitating alternative techniques of management to enable weaning parenteral nutrition and minimizing parenteral nutrition-associated comorbidities. This review aims at discussing the indications and techniques most commonly utilized for surgical management in a short gut. Surgical therapy for short gut has evolved and undergone refinement in the past 30-40 years with the older procedures being largely abandoned and replaced by newer and refined techniques. Lengthening surgeries (longitudinal intestinal lengthening and tapering and serial transverse enteroplasty) are the most commonly applied procedures in a dilated bowel with intestinal transplantation option reserved as a last resort for recurrent life-threatening central venous line infections, progressive loss of central venous access, and development of progressive liver disease. Management of intestinal failure requires a multidisciplinary approach to optimize intestinal rehabilitation and overall patient outcome. Although intestinal transplantation remains an excellent option for patients with severe life-threatening complications, autologous intestinal reconstruction appears to remain the better overall option.

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.

Autologous Intestinal Reconstructive Surgery to Reduce Bowel Dilatation Improves Intestinal Adaptation in SBS

To the Editor: Almond et al (1) reported 8 of 26 patients referred with intestinal failure (IF) from short bowel syndrome with >40 cm of dilated residual bowel. The study aimed to establish whether surgery to reduce dilatation could improve outcome; timing of surgery varied from approximately 3 months to 3 years (median approximately 10 months). Strikingly, 7 of 8 patients developed severe IF-associated liver disease (IFALD) (2) (mean peak bilirubin 250 μmol/L) preoperatively, yet liver function tests normalised after surgery, and 7 of 8 patients were weaned from parenteral nutrition. These interesting observations should be interpreted with caution. This seems to be an extremely unusual group of patients because, as the authors stressed, all were totally dependent on parenteral nutrition for nutrient requirements. It appears that despite “hepatosparing” strategies, severe IFALD (bilirubin >100 μmol/L) (2) developed in all but 1. Underlying condition and interventions were heterogeneous, including serial transverse enteroplasty procedure, longitudinal intestinal lengthening, and tapering enteroplasty. Although the authors considered small intestinal bacterial overgrowth to be a key factor in failure of adaptation, no diagnostic tests were performed (3), and no information was given about empirical preoperative treatment with antibiotics or prokinetics. This article brings into focus a difficult group of patients with bowel dilatation, but raises questions about the optimal nature and timing for surgery, difficulty in reliably diagnosing small intestinal bacterial overgrowth, and whether aggressive medical management of early IFALD, including prokinetics and antibiotics, may have facilitated enteral feeding and gut adaptation, while preventing the progression of liver disease. Robust research will be needed to provide definitive answers to these questions.

Autologous Intestinal Reconstructive Surgery to Reduce Bowel Dilatation Improves Intestinal Adaptation in Children With Short Bowel Syndrome

Intestinal failure (IF) is a common consequence of neonatal small bowel pathology. In our experience, bowel dilatation is often responsible for the IF state in patients who fail to adapt despite adequate residual bowel length. The aim of the present study was to investigate the role of surgery to reduce bowel dilatation, and thus favour PN independence, for these children. Data were collected prospectively for all of the patients referred to our unit for a 7-year period (2004-2011). Eight patients (2 congenital atresia, 2 gastroschisis with atresia, 1 simple gastroschisis, 3 necrotising enterocolitis) with gut dilatation who failed adaptation despite a bowel length >40 cm were identified. Preoperatively, all patients were totally dependent on parenteral nutrition (PN). Patients were managed by longitudinal intestinal lengthening and tailoring (n = 3), serial transverse enteroplasty (n = 2), or tapering enteroplasty (n = 3). Median age at time of surgery was 273 days (103-1059). Mean gut length increased from 51 (35-75) to 73 cm (45-120) following surgery (P = 0.02). Incidence of sepsis (P = 0.01) and peak serum bilirubin levels (P = 0.005) were reduced postoperatively. PN was discontinued after a median of 110 days (35-537) for 7 patients; 1 patient remains on PN 497 days after surgery. These data indicate that reconstructive surgery to reduce bowel diameter may be an effective technique for treating IF in patients with short bowel syndrome, without sacrificing intestinal length. We suggest that this technique may reduce the need for bowel transplantation in this group of patients.