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Objectives: To review recent evaluations of pediatric patients with intestinal failure (IF) for intestinal transplantation (ITx), waiting list decisions, and outcomes of patients listed and not listed for ITx at our center., Methods: Retrospective chart review of 97 patients evaluated for ITx from January 2014 to December 2021 including data from referring institutions and protocol laboratory testing, body imaging, endoscopy, and liver biopsy in selected cases. Survival analysis used Kaplan-Meier estimates and Cox proportional hazards regression., Results: Patients were referred almost entirely from outside institutions, one-third because of intestinal failure-associated liver disease (IFALD), two-thirds because of repeated infective and non-IFALD complications under minimally successful intestinal rehabilitation, and a single patient because of lost central vein access. The majority had short bowel syndrome (SBS). Waiting list placement was offered to 67 (69%) patients, 40 of whom for IFALD. The IFALD group was generally younger and more likely to have SBS, have received more parenteral nutrition, have demonstrated more evidence of chronic inflammation and have inferior kidney function compared to those offered ITx for non-IFALD complications and those not listed. ITx was performed in 53 patients. Superior postevaluation survival was independently associated with higher serum creatinine (hazard ratio [HR] 15.410, p = 014), whereas inferior postevaluation survival was associated with ITx (HR 0.515, p = 0.035) and higher serum fibrinogen (HR 0.994, p = 0.005)., Conclusions: Despite recent improvements in IF management, IFALD remains a prominent reason for ITx referral. Complications of IF inherent to ITx candidacy influence postevaluation and post-ITx survival., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: Anastomotic ulceration (AU) is a rare but life-threatening complication of pediatric short bowel syndrome (SBS). AUs may be challenging to detect and refractory to treatment. This study aimed to identify features associated with symptomatic bleeding AUs in children with SBS and factors that may impact resolution of bleeding. The relationship between dietary changes and symptomatic anastomotic hemorrhage was also explored., Methods: We conducted a retrospective chart review of 381 patients cared for in the Intestinal Rehabilitation Program at our center from 2013 to 2022. Patients with symptomatic AUs were identified based on at least 1 endoscopic procedure showing AUs and evidence of clinically significant gastrointestinal bleeding. We collected patient demographics, clinical characteristics, dietary history, radiologic imaging, and histopathology. We used descriptive statistics to identify patterns of presentation., Results: AUs were identified in 22 patients who were followed for a median duration of 2.9 years after anastomotic ulcer identification. AUs uniformly evolved years after the initial anastomosis (median 3.2 years). Characteristics included bowel stricture (4/22), small bowel-colon anastomosis (19/22), partial colectomy (17/22), and an increase in whole foods fraction (12/18). Bleeding resolved with operative intervention in the majority with anastomotic stricture (3/4). Recurrent bleeding was common in those without stricture (13/18). In a subset of patients without stricture, whole food reduction was associated with improvement or resolution of bleeding (5/6)., Conclusions: We observed a higher proportion of patients with AUs who responded to surgical intervention in the subset of children with definitive anastomotic strictures versus those without, suggesting that careful characterization of intestinal anatomy may be critical to predicting response to therapy. We also observed that bleeding from AU typically first manifested within 1 year of a shift from elemental or hydrolyzed enteral formula to a whole food-based diet (including commercial blenderized feeds), which may indicate that components of the enteral diet play a role in the pathogenesis of AU. Further studies are needed to validate these hypotheses., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in children. The mainstay of treatment for IF is parenteral nutrition (PN). The aim of this position paper is to review the available evidence on managing SBS and to provide practical guidance to clinicians dealing with this condition. All members of the Nutrition Committee of the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) contributed to this position paper. Some renowned experts in the field joined the team to guide with their experience. A systematic literature search was performed from 2005 to May 2021 using PubMed, MEDLINE, and Cochrane Database of Systematic Reviews. In the absence of evidence, recommendations reflect the expert opinion of the authors. Literature on SBS mainly consists of retrospective single-center experience, thus most of the current papers and recommendations are based on expert opinion. All recommendations were voted on by the expert panel and reached >90% agreement. The first part of this position paper focuses on the physiological mechanism of intestinal adaptation after surgical resection. It subsequently provides some clinical practice recommendations for the primary management of children with SBS from surgical resection until discharged home on PN., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: Autologous intestinal reconstructive (AIR) surgery is frequently utilized in the management of pediatric short bowel syndrome (SBS). However, little is known about the long-term sequela of these procedures., Methods: We undertook a retrospective follow-up study addressing parenteral nutrition (PN) dependence, nutritional status, intestinal morbidity, and related complications in SBS patients having undergone AIR surgery (SBS-AIR, n = 19). We compared results with conservatively treated control SBS patients (SBS-C, n = 45). Eligible patients were identified from our institutional intestinal failure registry during 1985-2019., Results: After median 11.4 follow-up years, 42% of SBS-AIR patients received PN in relation to 36% in SBS-C group ( P = 0.6210), and overall PN duration was significantly longer (35.4 vs 10 months, P = 0.0004) in SBS-AIR group. Although symptoms of intestinal dysfunction improved in majority (62%) of patients after AIR surgery, their symptoms remained more frequent and severe at latest follow-up compared to SBS-C group (39% vs 5%, P = 0.0015). Although bacterial overgrowth was more frequent in SBS-AIR group (53% vs 24%, P = 0.0416), latest endoscopy findings and fecal calprotectin levels as well as occurrence of anastomotic/staple line ulcerations were comparable between groups. Histological liver steatosis (50% vs 18%, P = 0.042) and impaired bone health (26% vs 6.7%, P = 0.042) were more frequent in SBS-AIR patients., Conclusions: While AIR surgery improved gastrointestinal symptoms and transition to enteral autonomy in majority of patients, a noteworthy proportion of them continued to suffer from clinically significant intestinal dysfunction and related complications. Close long-term follow-up of pediatric AIR surgery patients is mandatory., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: Total colonic aganglionosis involving the small bowel is a rare form of Hirschsprung disease. We aim to analyse the long-term outcomes, digestive autonomy, and complications, to suggest recommendations for prevention and treatment., Methods: All patients born between 2000 and 2015 followed in our centre were retrospectively included. We analysed the length of aganglionosis, surgical procedures, growth, duration of parenteral nutrition (PN), enterocolitis, liver disease, intestinal transplantation., Results: Twenty-five patients were followed for a median of 10.9 years. Fifteen patients had less than 80 cm of ganglionic small bowel (SB) with a median of 20 cm. Ten patients had more than 80 cm of ganglionic sB with a median of 115 cm. The median PN duration was significantly shorter for patients with more than 80 cm: 0.9 versus 7.5 years in those with less than 80 cm (P  < 0.001). No patient with less than 80 cm was weaned off PN, except 1 who underwent intestinal transplantation. Ten patients with less than 80 cm develop enterocolitis on the excluded segment, leading to emergency entero-colectomy in 5. Liver disease was more frequent in patients with less than 80 cm (11 vs 0). Three patients required combined liver-intestine transplantation; 2 underwent an isolated intestinal transplantation., Conclusions: Digestive autonomy was possible in most patients with more than 80 cm of ganglionic SB. The more severe complication was enterocolitis. Liver disease compromised long-term survival without transplantation. Both complications should be prevented by early diversion and enterectomy of the whole aganglionic segment. Follow-up in or together with a multidisciplinary intestinal rehabilitation centre is suggested., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objective: Management of short bowel syndrome (SBS) has significantly evolved recently. We present our single-center, 25-year experience focusing on the implementation of a refined multidisciplinary SBS care protocol., Methods: This is a retrospective review of the patients with SBS treated at our tertiary center from 1988 to 2014, with either <25% short bowel remaining or duration of parenteral nutrition (PN) >3 months. Patients with primary intestinal motility disorders were excluded. Clinical characteristics, including intestinal anatomy, markers of cholestasis, and catheter-related infections (CRIs), were analyzed. The implementation of a refined modern uniform management protocol in 2009 divided the cohort into 2 subgroups, whose outcomes are compared., Results: Forty-eight patients with SBS were identified (median gestational age 33 weeks). Of them, 22 were born between 2009 and 2014. The main causes of SBS were necrotizing enterocolitis (46%) and midgut volvulus (23%). Median remaining small bowel length was 36 cm. The overall survival was 23 of 26 (88%) before 2009 and 21 of 22 (95%) thereafter, whereas none were transplanted. Duration of PN shortened from a median of 15 to 6 months (P = 0.0015) in the latter cohort, whereas frequency of autologous intestinal reconstruction surgery (31%) remained unchanged. Frequency of neonatal cholestasis was similar in both groups (75%), but cleared in all after 2009. Before 2009, 2 patients died of progressive cholestatic liver failure. The CRI rates decreased from 1.7 to 0.7 per 1000 catheter-days between 2000-2008 and 2009-2014, respectively (P = 0.0178)., Conclusions: Uniform refined multidisciplinary approach decreased the duration of PN and CRI rates with high transplant-free survival and avoidance of liver failure, although the frequency of transient neonatal cholestasis remained unchanged.

Objectives: Intestinal atresia is a common cause of intestinal obstruction in neonates. The predominant cause of late complications in these children is prolonged dependence on parenteral nutrition (PN). Our aims were to analyze the outcomes for patients with intestinal atresia at our institution, and to see how these changed with the implementation of an intestinal rehabilitation program (IRP)., Methods: This is a retrospective cohort study. The patient population is all children with intestinal atresias (118 patients) treated at our institution from July 2000 to June 2010, 20 of whom became PN dependent., Results: Survival to hospital discharge was 95% for all patients, and 100% for those who had isolated atresia. Twenty of 118 patients (17%) were PN dependent beyond initial hospital discharge. At discharge, their median intestinal length was 22.5 cm, and they required PN for a mean of 88.5% of energy needs. Of these 20 patients, 2 died, 2 received transplants, and 2 transferred away for transplantation. The remaining 14 joined the IRP. Their mean energy requirement from PN is presently 10%, down from 87% at IRP enrollment, and 10 patients fully weaned off PN. Eleven of the 14 children had hyperbilirubinemia, with mean direct bilirubin of 7.5 mg/dL. All resolved their cholestasis during an average of 12 weeks., Conclusions: These results compare favorably with those reported in earlier periods. With programs such as the IRP, patients with short bowel secondary to intestinal atresia can show improvement in liver function and nutritional parameters, and discontinue PN, avoiding the need for transplantation.

Objectives: 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., Methods: 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)., Results: 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., Conclusions: 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.

Objectives: The aim of the present study was to investigate the segment- and time-related changes in rat short bowel syndrome and construct a 4-dimensional (4D) geometrical model of intestinal adaptation., Methods: Sprague-Dawley rats were divided into 3 groups: 2-day, 7-day, and 15-day postresection groups in which 75% of the jejunoileum was removed. Histological and morphometrical parameters in the remaining proximal to distal intestinal segments, from the jejunum to the distal colon, were comparatively evaluated in the groups. The data were used to construct a 4D geometric model in which villi were considered as cylinders, and their surface area was expressed as cylinder lateral area., Results: Major adaptive changes were observed in the ileum consisting of an increase in both the diameter of base and the height of villi. A parallel reduction in their number/mm was observed. The resulting ileal architecture was characterized by a limited number of large villi. An opposite pattern was observed in the jejunum whose postresection structure consisted of an increased number of villi. No changes were observed in the colon. Postresection restructuring was early and faster in the ileum than in the jejunum resulting in an increase in absorptive area of 81.5% and 22.5% in the ileum and jejunum, respectively., Conclusions: Postresection adaptation is intestinal segment-specific because all of the major changes occur in the ileum rather than in the jejunum. Sparing ileal segments during resection may improve the outcome of patients undergoing extensive intestinal resection. Our 4D model can be used to test interventions aimed at optimizing postresection intestinal adaptation.

Objectives: In the present study, we evaluated the effect of transforming growth factor-beta 2 (TGF-β2)-enriched diet on enterocyte turnover and correlated it with TGF-β2 receptor expression along the villus-crypt axis in a rat model of short bowel syndrome (SBS)., Methods: CaCo-2 cells were incubated with increasing concentrations of TGF-β2. Alamar Blue reduction test was used for investigation of cell viability and evaluation of cell apoptosis was assessed by flow cytometry. Male rats were divided into 4 groups: Sham rats underwent bowel transection, Sham TGF-β rats were treated with diet enriched with TGF-β2, SBS rats underwent a 75% bowel resection, and SBS TGF-β rats were fed a diet enriched with TGF-β2 after bowel resection. Parameters of intestinal adaptation, enterocyte proliferation and apoptosis were determined at sacrifice. TGF-β2r expression in villus tips, lateral villi and crypts was assessed by immunohistochemistry. The effect of TGF-β2 on enterocyte turnover for each compartment was evaluated in correlation with TGF-β2r expression., Results: Incubation of CaCo-2 cells with TGF-β2 resulted in a significant decrease in cell viability and increased cell apoptosis. TGF-β2r expression in crypts increased in SBS rats (vs sham) and was accompanied by decreased cell proliferation and increased cell apoptosis following TGF-β2 administration. A significant decrease in TGF-β2r expression at villous tips in SBS rats was accompanied by a decreased cell apoptosis in this compartment following exposure to TGF-β2-enriched diet., Conclusions: In a rat model of SBS, the inhibiting effect of TGF-β2 on enterocyte turnover correlates with TGF-β2 receptor expression along the villus-crypt axis.

Background and Aim: Short bowel syndrome poses a great challenge to pediatric teams. Several innovative techniques in the management of total parenteral nutrition (TPN) and bowel reconstructive surgery have improved the outcomes of these children. The authors present their experience during the last decade as a specialist unit using improved techniques and multidisciplinary approaches in the management of this condition., Methods: All of the children presenting with short bowel syndrome between 2000 and 2009 were identified. Diagnosis, length of residual gut, age at definitive surgery, length of gut prelengthening, length of gut postlengthening, TPN status, and survival were recorded. Median values were calculated., Results: Twenty-seven children were identified (14 boys, 13 girls). Overall survival was 92%. Two children died. Nineteen children required bowel lengthening and 8 children had simple bowel reconstruction while on our protocol. Overall median age at definitive surgery was 12 months. Overall median residual gut length for these was 35.5 cm, whereas the median residual gut length for patients undergoing bowel lengthening was 25 cm. Postbowel lengthening, the median gut length was 90 cm. TPN data were unavailable for 2 patients. Overall, excluding the 2 patients who died and the 2 we have no TPN data on, of 23 patients, 21 (91%) are now off TPN., Conclusions: Our series shows improved results not only with survival but also in the number of patients that are off TPN. Multidisciplinary approach consisting of both medical and surgical expertise is necessary in the management of these patients. The authors advocate centralisation of short gut services to experienced centers with multidisciplinary expertise.

Background and Aims: Short bowel syndrome (SBS) describes the malabsorptive state seen following extensive bowel resection. Management aims to optimise nutritional intake, promote intestinal adaptation, and prevent the development of complications. Surgical options such as autologous gastrointestinal reconstruction (AGIR) attempt to restore enteral autonomy to the patient. Although the literature focuses on the objective measurements of success following AGIR techniques, subjective assessment of its effect on the quality of life (QoL) should also be sought. Because children with SBS are reliant on caregivers, caregivers' satisfaction with surgery and their perception of the effect of AGIR on their daily lives should be assessed. This is the first report of caregiver satisfaction following AGIR in children., Methods: All children with SBS who had AGIR between January 1999 and June 2010 were identified. A questionnaire was developed. Caregivers were asked to complete the questionnaire to rate their satisfaction with surgery and the pre- and postoperative daily care, medical needs, and subjective interpretation of QoL for their child. Data were analysed using SPSS version 18, using the Friedman nonparametric test and 2-way analysis of variance by rank. Statistical significance was set at ≤0.05., Results: Thirty-two children were identified. Seven assessments were done outside our centre (5 outside the UK) and were not included in the study. One patient moved to another city and was not contacted. Two children died. Twenty patients were contactable and were included in the present study. Parents were satisfied with the AGIR and saw improvements in their child's physical condition, bowel habit, and subjective interpretation of QoL postoperatively. Parents perceived significant improvements in the ease of caring and in aspects of their relationship with their child, including the quality of time spent, their enjoyment, and the level of their frustration with them., Conclusions: The present study found that AGIR improved the physical well-being of the child and gave the impression of improvements on specific QoL aspects for both the child and the parents.

Objectives: Short bowel syndrome occurring after surgery for acquired or congenital intestinal abnormalities causes considerable neonatal morbidity and mortality. Animal models are a valuable research tool for this problem; however, few successful neonatal models have been developed and most do not include distal intestinal resection as seen commonly in human babies. We report novel piglet models addressing these gaps., Subjects and Methods: Neonatal piglets (1-6 days) underwent venous and gastric catheter insertion and 75% intestinal resection. Group 1 (n = 6) had midintestinal resection with jejunoileal anastomosis; group 2 (n = 5) had distal intestinal resection with jejunocolic anastomosis; group 3 (n = 5) were sham controls; and group 4 (n = 5) were sow reared. Postoperatively, groups 1 to 3 piglets commenced parenteral nutrition (PN), and enteral nutrition was introduced and advanced using a standard regimen. Data collection included days on PN, weight gain, fat absorption, small intestine lengthening, and bowel/liver histology., Results: Group 2 piglets had more days on PN (P = 0.008), less weight gain (P = 0.027), and greater malabsorption (P = 0.012). They did not show small intestine lengthening and had more cholestatic liver disease. Group 1 piglets had histological evident intestinal adaptation and 1.5-fold intestinal lengthening (P = 0.001)., Conclusions: These novel piglet models of short bowel syndrome are the first to represent the full clinical spectrum of intestinal failure as observed in human neonates. By considering the impact of different short bowel anatomy on potential for adaptation and growth, these animal models are a significant advance. They permit evaluation of new therapies to promote intestinal adaptation and reduce complications, such as cholestasis.

Objective: Few infant animal models have been used to study infantile short bowel syndrome (SBS). Most SBS models involve removal of the proximal small bowel followed by jejunoileal anastomosis, which has unclear clinical relevance to human infantile SBS that often results from surgical treatment for necrotizing enterocolitis and involves removal of the ileum, ileocecal valve, and part of or the entire colon. Our objective was to develop a more appropriate SBS model in developing rats., Materials and Methods: Twenty-day-old weanling rats were divided into 2 surgery groups, ileocecal resection (ICR) and sham groups, and a control group that did not undergo surgery. All were fed a liquid diet ad libitum for 7 days after surgery or for 7 days in the controls, and body weight, food intake, and stool changes were recorded daily. The rats were then euthanized and intestinal lengths and weights were recorded. Samples of intestine from the distal jejunum and proximal colon were collected for histology. Mucosal samples from the middle, distal jejunum, and colon were collected for measurements of mucosal weights, DNA, RNA, and protein levels. Maltase activity was determined in the small intestine., Results: Eighty-five percent of rats survived the ICR with subsequent development of diarrhea, hyperphagia, and poor growth. Adaptive responses to ICR, as compared with sham, were evidenced by increased intestinal and mucosal weights, DNA, RNA, and protein levels, increased maltase activity and villous thickness in distal jejunum, and increased mucosal thickness in the colon., Conclusions: This ICR model in weanling rats is appropriate for studying human infantile SBS.

Colon is a crucial partner for small intestinal adaptation and function in patients who underwent extensive small intestinal resection or transplantation. This short review deals with the different properties and roles of the colon in these settings, involving fluid and electrolytes absorption, absorption of medium-chain triglycerides, and production of short-chain fatty acids for malabsorbed energy salvage. The colon may adapt after small intestinal resection, whereas it hosts the most important part of the intestinal microbiota, which plays a crucial role in intestinal function and health. Also, colon may be responsible for D-lactic acidosis as well, as it can be injured by noninfectious colitis. Finally, the relevance of a simultaneous colon grafting is discussed as it is occasionally considered in specific patients requiring intestinal transplantation.

Objective: Selected infants with short bowel syndrome (SBS) and progressive intestinal failure associated liver disease (IFALD) may benefit from isolated liver transplantation (iLTx). The aim of the study is to identify risk factors for unfavourable outcome in iLTx., Patients and Methods: A retrospective review of medical records from 1998 to 2005 was undertaken. Risk factors were assessed by comparing long-term survivors with those who died after iLTx., Results: Fifteen iLTx were performed in 14 infants with IFALD. All were parenteral nutrition (PN) dependent, but had tolerated enterally 54% (38-100) of energy intake before iLTx. Median residual bowel was 60 cm (30-200). Eight out of 14 had intact ileocaecal valve (ICV). Median bilirubin was 298 micromol/L (87-715) and all had portal hypertension. Eight out of 9 survivors were weaned from PN after median 15 months. In 4 out of 9 children, nontransplant surgery after iLTx facilitated intestinal adaptation. Growth velocity had improved at 3 years after iLTx (P=0.001). Five children who died had poor enteral tolerance following iLTx (P<0.002), which correlated with pretransplant dysmotility seen in 4 out of 5 children shown by contrast studies (P=0.02)and increased frequency of line infections before (>6/year P<0.04) and after (P<0.001) iLTx., Conclusions: Isolated liver transplantation is a lifesaving option for selected children with SBS and IFALD. Revised criteria are proposed: progressive IFALD; 50 cm functional bowel in absence of ICV or 30 cm with ICV; 50% daily energy intake tolerated enterally for 4 weeks with satisfactory growth; and children with dysmotile bowel should be assessed for combined liver/bowel transplant unless the dysmotility is resolved and associated with minimal line infections.

Objectives: 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., Patients and Methods: 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)., Results: 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)., Conclusions: 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.

Objective: The objective of the present study was to examine the effect of glucagon-like peptide-2 (GLP-2) administration in a piglet, juvenile model of short bowel syndrome., Materials and Methods: Four-week-old piglets underwent either a sham operation or 75% small bowel resection. Postoperatively, piglets received either polymeric infant formula diet or the diet and subcutaneous human recombinant GLP-2 (1600 microg/day for 7 days, 800 microg/day thereafter). Food intake was monitored throughout the experiment, and stool and serum samples obtained fortnightly. After the piglets were killed, tissues were obtained from the duodenum, jejunum, ileum, and terminal ileum, and used for morphological and functional analysis., Results: Treatment with GLP-2 resulted in significantly increased numbers of proliferating and apoptotic cells in the ileum of sham and small bowel resection piglets (P < 0.05). GLP-2 administration resulted in decreased weight gain, serum albumin, and disaccharidases in both sham and small bowel resection piglets (P < 0.001 compared with polymeric infant formula diet alone)., Conclusions: This is the first study to our knowledge to examine the effect of GLP-2 administration in a juvenile short bowel syndrome model. Contrary to adult rodent studies, administration of GLP-2 resulted in adverse outcomes including reduced ability to gain weight; decreased serum albumin, tissue maltase, and sucrase; and villous atrophy. We anticipate this information will have important implications for future paediatric clinical trials.

Background: Short bowel syndrome (SBS) develops after massive small bowel resections. Patients with less than 12 cm of jejunoileum have a slim possibility of being weaned from parenteral nutrition (PN)., Patients and Methods: In a retrospective review of records of consecutive patients with SBS, 8 patients were evaluated for treatment by adaptation and weaning from PN. These included 4 patients with class I SBS (0-10 cm of small bowel), one with class II SBS (>10-25 cm), one with class III SBS (>25-50 cm), and 2 with class IV SBS (>50-75 cm). Adaptation was assessed by measuring growth in the small bowel and the ability to be weaned from PN., Results: Adaptation was achieved primarily by extending the length of jejunoileum by approximately 450% over the first 2.5 years after resection and by increasing the degree of colonic fermentation and absorption of nutrients. As of July 1, 2005, all of the patients were off PN, with the exception of 2 patients with class I-A SBS: patient 3 had a remaining jejunoileum of only 2.5 cm and patient 4 had a remaining jejunoileum of 9 cm but developed eosinophilic enterocolitis. These 2 patients continued with PN on alternate months., Conclusions: Bowel growth after massive small bowel resection provides an objective parameter of adaptation and a means of predicting ability to be weaned from PN. Aggressive nutritional support makes even patients with class I SBS, whose disease was previously considered hopeless, likely candidates to achieve freedom from PN.

Objective: To analyze outcomes in children with intestinal failure treated by our Intestinal Rehabilitation Program (IRP) in a 4-year period., Patients and Methods: A total of 51 parenteral nutrition (PN)-dependent patients (20 male) were enrolled in the IRP. Median age was 1.7 years, with the primary diagnoses being gastroschisis, necrotizing enterocolitis, volvulus, and congenital atresia. Median small bowel intestinal length was 35 cm, with the majority of patients having only jejunum as remaining bowel anatomy. Thirty-six of the 51 patients had liver disease characterized by cirrhosis, advance bridging fibrosis, and portal and periportal fibrosis. Height, weight z score, platelet count, albumin, and bilirubin levels were measured at the beginning and end of the study., Results: Of the 51 patients, 29 had 46 different surgical intestinal repairs. Twenty-nine of the 36 patients with hyperbilirubinemia had normalized serum bilirubin with treatment. Ten patients required transplantation. Five patients died of sepsis, influenza, or complications after intestinal transplantation. Of the remaining 37 patients in the IRP, 31 were weaned from parenteral nutrition (5 with cirrhosis); 6 patients are in the process of weaning. Survival rate of the patients in the IRP was 90%. Growth has continued along the same curve, and some patients have exhibited significant catch-up., Conclusions: With an aggressive medical/surgical approach, even patients with intestinal failure and advanced liver disease can avoid transplantation. Patients in the IRP showed improved liver function and nutritional parameters with the ability to discontinue PN while maintaining growth. Early referral of these patients to specialized centers before the development of advanced liver disease is recommended.

A systematic review of literature was performed on the practice of mucous fistula refeeding in neonates with short bowel syndrome. No randomised controlled studies were identified. Case series reports of 30 infants showed improved weight gain (from -2.36 +/- 1.24 to 21.06 +/- 3.02 g/d) and reduced need for total parenteral nutrition. Randomised controlled trials are required to establish the benefits of mucous fistula refeeding in this condition.

Background: Premature neonates with short bowel syndrome often have diverting enterostomies and distal mucous fistulae. The authors reviewed their experience in 12 premature neonates in whom proximal bowel contents were re-fed into the mucous fistula., Methods: We reviewed the records of 12 premature neonates who presented with acute abdomen and who underwent intestinal resection with formation of diverting enterostomy and mucous fistula between July 1999 and December 2002. All received parenteral nutrition. Refeeding of enterostomy contents into the distal mucous fistula was commenced after patency of the distal intestine was confirmed by radiologic examination. Demographic data, body weight and clinical outcomes were recorded., Results: Median gestational age was 31 weeks and mean birth weight was 1.59 kg. Diagnoses included necrotizing enterocolitis (n = 6), meconium ileus-like conditions (n = 2), ileal atresia (n = 2), malrotation with volvulus (n = 1) and focal intestinal perforation (n = 1). Refeeding was successfully established in all patients with no complications. The mean duration of refeeding was 63.5 days. All patients achieved good weight gain after refeeding (18.9 +/- 2.9 g/d) with a reduction of parenteral nutrition requirements. All enterostomies were subsequently closed. Four patients died of unrelated causes after reanastomosis and the remaining eight were discharged., Conclusions: Mucous fistula refeeding is safe in premature neonates with enterostomies. It can prevent disuse atrophy in the distal loop and facilitate subsequent reanastomosis. Furthermore, the increased absorptive function provided by the small bowel incorporated in the mucous fistula can reduce the requirement for total parenteral nutrition.

Intestinal failure (IF) can be defined as the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children. In developed countries, IF mainly includes individuals with the congenital or early onset of conditions requiring protracted or indefinite parenteral nutrition (PN). Short bowel syndrome was the first commonly recognized cause of protracted IF. The normal physiologic process of intestinal adaptation after extensive resection usually allows for recovery of sufficient intestinal function within weeks to months. During this time, patients can be sustained on parenteral nutrition. Only a few children have permanent intestinal insufficiency and life-long dependency on PN. Non-transplant surgery including small bowel tapering and lengthening may allow weaning from PN in some cases. Hormonal therapy with recombinant human growth hormone has produced poor results while therapy with glucagon-like peptide-2 holds promise. Congenital diseases of enterocyte development such as microvillus inclusion disease or intestinal epithelial dysplasia cause permanent IF for which no curative medical treatment is currently available. Severe and extensive motility disorders such as total or subtotal intestinal aganglionosis (long segment Hirschsprung disease) or chronic intestinal pseudo-obstruction syndrome may also cause permanent IF. PN and home-PN remain are the mainstays of therapy regardless of the cause of IF. Some patients develop complications while receiving long-term PN for IF especially catheter related complications (thrombosis, sepsis) and liver disease. These patients may be candidates for intestinal transplantation. This review discusses the causes of irreversible IF and emphasizes the specific medico-surgical strategies for prevention and treatment of these conditions at several stages of IF.

Background: The primary goal of surgical therapy for short bowel syndrome is to increase intestinal absorptive capacity. Many surgical procedures have been described for this purpose. One of these is ileal reverse-segment procedure. This procedure after massive small-bowel resection is an alternative way to treat short bowel syndrome, but how it affects intestinal morphology in short bowel syndrome has not been investigated. The aim of this study is to investigate macroscopic and microscopic effects of reverse-segment procedure on the short bowel., Methods: Twenty rats underwent resection of 80% of the small bowel. The rats were separated into two groups (n = 10). In the first group (reverse group), a reverse segment was formed by twisting a 2-cm ileal segment 180 degrees, without damaging its vascularity. In the second group (control group), a 2-cm ileal segment was resected, preserving its mesentery, and end-to-end anastomosis was performed to maintain the intestinal passage. The segment was not twisted 180 degrees. The 2-cm proximal (jejunal) and distal (ileal) segments of the resected bowel were reserved for histologic investigation. Two months later, the rats were killed and the jejunal and ileal segments were evaluated morphologically., Results: In the reverse group, body weight and total intestinal length significantly increased (14% more than in the control group). The diameter of both proximal (jejunal) and distal (ileal) segments in the reverse group also increased 53.8% and 22.8%, respectively ( P < 0.05). Histologically, crypt depth and villus height of the ileal segment in the reverse group increased 15.2% and 18.2% more than in the control group ( P < 0.05). No histologic change was observed at the jejunal level except for intestinal muscle thickness., Conclusions: Ileal reverse-segment procedure in rats with short bowel syndrome 1) does not cause intestinal obstruction, 2) increases total bowel length and body weight, 3) increases the diameter of both jejunal and ileal segments, and 4) increases villus height and crypt depth only at the ileal level. For this reason, reverse-segment procedure positively affects intestinal adaptation.

Background: Sugar absorption tests are an effective, noninvasive way to assess intestinal permeability. The role of intestinal barrier integrity in complications and outcome of short-bowel syndrome is not known. The purpose of the study was to evaluate whether such tests provide information on the status of intestinal mucosa of these patients., Methods: Six children with short-bowel syndrome--median age, 12 months, and median small bowel length at birth, 30 cm--had a sugar test with 3-o-methyl-D-glucose, D-xylose, D-rhamnose, and melibiose approximately 2 months after operation. The melibiose/L-rhamnose ratio was used as an index of permeability, and percentages of 3-o-methyl-D-glucose and D-xylose absorbed were used as indices of absorption. Parenteral nutrition requirement, bowel length, liver disease, recent sepsis, and bacterial overgrowth were recorded., Results: Three patients had increased permeability, and all of them had had a recent episode of sepsis and severe liver disease. All subjects had malabsorption of 3-o-methyl-D-glucose, and five of six had malabsorption of D-xylose and L-rhamnose. The absorption of 3-o-methyl-D-glucose correlated with bowel length (r2 = 0.78; P = 0.04), whereas the absorption of D-xylose correlated with parenteral requirement (r2 = 0.66; P = 0.04) at that time., Conclusions: Increased permeability was observed in three of six patients with short-bowel syndrome associated with a recent episode of sepsis and severe liver disease. Other indices of malabsorption correlated significantly with different clinical features of the disease. A prospective larger scale study in a homogeneous population is indicated to assess at multiple points during the disease course whether the test can be helpful in the management of these patients.

Background: With the higher survival rate of premature neonates as a result of improved neonatal intensive care, the incidence of necrotizing enterocolitis, and thus the incidence of short-bowel syndrome, is increasing. An appropriate animal model resembling the (premature) neonate with short-bowel syndrome suitable for clinically relevant neonatal bowel adaptation and intervention studies, is not available at present. The purpose of this study was the development of a short-bowel syndrome model that mimics the clinical state of the affected neonatal patient., Methods: Sixteen 7-day-old piglets received either a small bowel transection (group A) or a 75% resection (group B). The piglets were fed 125 kcal/kg body weight per day, including additional electrolytes. The animals were weighed daily and were killed 28 days after surgery. Bowel samples were obtained at both time points., Results: Mortality rates in groups A and B were 0% and 8%, respectively. Body weight gain was significantly higher in group A than in group B (156% vs. 93%; P = 0.01). Jejunal villus length was higher in group B than in group A (74% vs. -2%; P = 0.006), and crypt depth was higher in group B in both jejunum (201% vs. 67%; P = 0.001) and ileum, (197% vs. 20%; P = 0.001), than in group A., Conclusions: In 7-day-old piglets 75% small bowel resection leads to a clinical short-bowel syndrome, demonstrated by reduced weight gain and typical changes in bowel adaptation parameters. The excellent survival of the animals provides a possibility for the study of bowel adaptation in a neonatal model as well as in intervention studies.

Management of extreme short bowel syndrome (SBS) has changed dramatically over the last 20 years with notable improvements in survival and quality of life in patients with this syndrome. A review of our institution's medical records over a 12-year period (1980-1992) revealed 32 patients with < 100 cm (range, 14-94; median, 40) of functional small bowel after intestinal resection. The causes of intestinal loss included necrotizing enterocolitis (11 cases), atresias (8 cases), long-segment Hirschsprung's disease (5 cases), midgut volvulus (5 cases) and gastroschisis (3 cases). The mean follow-up period was 4.2 years, and four deaths were recorded (12.5%). Survival of eight of nine (88.9%) patients without an ileocecal valve (ICV) and with < 40 cm of small bowel was noted. The absence of an ICV, however, was associated with significantly prolonged total parenteral nutrition. Follow-up surgical procedures, including intestinal lengthening, tapering enteroplasty. Martin's procedure, longitudinal myectomy-myotomy, and ostomy take-down, were performed in 20 of the patients (64%). Prolonged survival and normal development can be expected for the patient with severe SBS who is given meticulous nutritional support and treated with carefully planned secondary surgical intervention. These results are also seen in patients with extreme SBS (< 40 cm residual small bowel length) and no ICV.

With utilization of the techniques discussed in this review, patients with short bowel syndrome can be expected to live prolonged lives, even after fairly massive resection. Advances in parenteral and home parenteral nutrition and changes in the long-term management of chronic complications have altered the lifestyles of patients with short bowel syndrome. Intestinal transplantation may become an effective mode of therapy within a short period of time. Many surgeons faced with the possibility of leaving a child with only 10-15 cm of bowel in the past have closed the abdomen with the dead bowel and let the child die. These recent advances raise interesting ethical dilemmas. Research into the process of intestinal adaptation as well as greater experience with intestinal transplantation and other modes of therapy may further improve prognosis.

To determine the frequency of gastric acid hypersecretion in infants with chronic malabsorption due to short bowel syndrome, acid secretory function was determined in 23 infants with malabsorption 2-22 months following small bowel resection and in a control group of 14 chronically ill, age- and weight-matched infants who did not undergo bowel resection. The prevalence of basal acid hypersecretion (defined as acid output 2 SD above the mean for the control group) was 17% (4 of 23). Basal gastric acid hypersecretion was associated with two factors: massive small bowel resection and initiation of enteral feeding. Basal acid hypersecretion was present on the initial study in 3 of 7 infants with less than one-third of the small bowel remaining, but in only 1 of 16 with more than one-third intact (p less than 0.05). Hypergastrinemia was present in 3 of 6 infants following massive bowel resection, but in only 1 of 15 with more than one-third intact (p less than 0.05), but hypergastrinemia was not consistently associated with hypersecretion. In each of six previously unfed infants, a trial of enteral feeding resulted in increased basal and maximal acid output. Three infants developed basal acid hypersecretion during initiation of enteral feeding. There was no evidence of pentagastrin-stimulated maximal acid hypersecretion in any of the infants.

Intestinal tapering and lengthening were performed in a child with short bowel syndrome to prevent the need for home parenteral nutrition. The intestinal lengthening was not successful, presumably because the vascular anatomy was not favorable. Significant clinical improvement and avoidance of home parenteral nutrition resulted from the tapering procedure. These procedures have a role in the treatment of selected patients with short bowel syndrome.