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This study evaluated the quality of life (QoL) in patients who have recovered from surgical necrotizing enterocolitis (NEC). This is a cross-sectional study conducted in a tertiary centre and patients who have received surgery for NEC between 2000 and 2014 were invited to participate. The Pediatric Quality of Life Inventory Generic (PedsQL™) Core Scale Version 4.0 was used as the assessment tool. Values were reported as median (interquartile rage) and compared with age-matched controls. During the study period, 90 patients were eligible for the study and 29 patients have completed the survey. There was no gender difference and the median age was 10 years (9–13 years). Nine patients have suffered from short bowel syndrome (SBS) as a result of the surgery. For the QoL assessment, 17 participants have completed both parent proxy and child-rated survey; 11 completed the parent-rated survey and 1 completed child-rated survey only. The scores for parent-rated survey were - overall: 86.4 (70.7–92.7); physical: 95.3 (83.6–100) and psychosocial: 82.5 (66.3–90.4). The scores for child-rated survey were – overall: 82.1 (73.4–96.2); physical: 96.9 (90.6–99.2) and psychosocial: 81.7 (64.2–95.8). Regarding the impact of previous SBS on the QoL, there were no significant difference in the overall score for both parent proxy and child-rated survey (SBS-ve vs + ve) (parent-rated: 87.5 vs 85.3, p = 0.849; child-rated: 81.0 vs 88.0, p = 0.503). There were also no differences in physical and psychosocial assessments (parent-rated: [physical] 95.3 vs 95.3, p = 0.267; [psychosocial] 84.2 vs 80.0, p = 0.274; child-rated: [physical] 95.3 vs 96.9, p = 0.395; [psychosocial] 79.2 vs 87.5, p = 0.611). The QoL in long-term survivors of surgical NEC without major medical illnesses is comparable to normal population. However, they may have a lower psychosocial well-being that should be addressed. Previous history of SBS does not have a significant impact on the future QoL. III. [ABSTRACT FROM AUTHOR]

Background/purposeIntraluminal springs have recently been shown to lengthen segments of intestine in a process known as distraction enterogenesis. We hypothesized that biocompatible springs could be used to lengthen defunctionalized murine small intestine and would lead to identifiable intestinal adaptations at the molecular level.MethodsAge and weight matched C57BL/6 mice underwent surgical insertion of nitinol spring-loaded capsules into a Roux limb of jejunum. Segment lengths were measured at initial spring placement and at euthanasia after 14 and 21 days. Histology and gene expression of the Roux limb were evaluated at scarification and compared to untreated control segments.ResultsIntestinal segments loaded with compressed springs lengthened an average of 240%, which was significantly longer than control segments loaded with either empty capsules or uncompressed springs. Muscularis thickening was greater in spring-treated mice compared to controls without springs. Crypt depth and Lgr5+ expression was greater in mice that received compressed spring treatments when compared to control groups.ConclusionsInsertion of a compressed nitinol spring into a Roux limb results in significant intestinal lengthening, smooth muscle thickening, and Lgr5+ expression in a mouse model. The ability to increase small bowel length in a defunctionalized murine model may be used to understand the mechanism of distraction enterogenesis.

• ICG-FA is used to determine anastomotic perfusion rates, thereby reducing surgical complications and aiding in intraoperative decision-making in adults, but not yet extensively described in pediatric surgery. • This systematic review analyzes the current knowledge on usefulness of ICG for intestinal perfusion assessment in pediatric gastrointestinal surgery and its safety in neonates. • ICG might be useful for intraoperative intestinal perfusion assessment, perhaps even more than conventional clinical assessment. • The safety profile of ICG looks promising in neonates. Larger prospective studies are necessary to confirm these assumptions and seem warranted given the safety profile. Although ICG-FA may be valuable in assessing anastomotic perfusion, reliable data on its use in pediatric gastrointestinal surgery is lacking. This systematic review analyzes whether ICG is useful for intestinal perfusion assessment in pediatric gastrointestinal surgery and safe to use in neonates. Systematic searches of PubMed, EMBASE & MEDLINE and CENTRAL were performed (last conducted December 6, 2021). The main inclusion criteria were (1) use of ICG for intestinal perfusion assessment and (2) use of ICG in young infants. Exclusion criteria were lack of an English or Dutch full-text and MINORS quality score <60%. Data was presented in overview tables. The usefulness in pediatric gastrointestinal surgery was assessed by surgical outcome. Safety of ICG in neonates was assessed by complication or adverse event occurrence. Regarding intestinal perfusion assessment, four studies were included, reporting 45 patients (median age 1.5 years). ICG was considered useful for anastomotic blood flow evaluation and intraoperative determination of resection length. Regarding ICG safety in neonates, eight studies were included, reporting 46 infants (median age 24.9 days), of which 18 neonates. All but one studies reported the absence of complications or adverse events. Two studies reported subcutaneous dye retention, which fully disappeared within two weeks. Although the number of available studies is small, ICG might be useful for intraoperative intestinal perfusion assessment, perhaps even more than conventional clinical assessment. Furthermore, its safety profile looks promising in neonates. Larger prospective studies are necessary to confirm these assumptions and seem warranted given the safety profile. Since this is a systematic review, a Level of Evidence for clinical studies cannot be determined for this manuscript. [ABSTRACT FROM AUTHOR]

It is well known that small bowel length is a dominant prognostic indicator in patients with short bowel syndrome (SBS). The relative importance of jejunum, ileum, and colon is less well defined in children with SBS. Here we review the outcome of children with SBS with respect to the type of remnant intestine. A retrospective review of 51 children with SBS was conducted at a single institution. The duration of parenteral nutrition use was the main outcome variable. The length of the remaining intestine as well as the type of intestine were recorded for each patient. Kaplan–Meier analyses were conducted to compare the subgroups. Children with greater than 10% expected small bowel length or more than 30 cm of small bowel achieved enteral autonomy faster than those with less. The presence of ileocecal valve enhanced the ability to wean from parenteral nutrition. The presence of ileum significantly enhanced the ability to wean from parenteral nutrition. Patients with the entire colon also achieved enteral autonomy sooner than those with partial colon. The preservation of ileum and colon is important in patients with SBS. Approaches to preserve or lengthen ileum and colon may be beneficial for these patients. IV. [ABSTRACT FROM AUTHOR]

Massive small bowel resection (SBR) is associated with liver injury and fibrosis. Efforts to elucidate the driving force behind hepatic injury have identified multiple factors, including the generation of toxic bile acid metabolites. Sham, 50% proximal, and 50% distal SBR were carried out in C57BL/6 mice to determine the effect of jejunal (proximal SBR) versus ileocecal resection (distal SBR) on bile acid metabolism and liver injury. Tissues were harvested at 2 and 10-week postoperative timepoints. When compared with 50% proximal SBR, mice that underwent distal SBR exhibited less hepatic oxidative stress as verified by decreased mRNA expression of tumor necrosis factor-α (TNFα , p ≤ 0.0001), nicotinamide adenine dinucleotide phosphate oxidase (NOX , p ≤ 0.0001), and glutathione synthetase (GSS , p ≤ 0.05). Distal SBR mice also exhibited a more hydrophilic bile acid profile with reduced abundance of insoluble bile acids (cholic acid (CA), taurodeoxycholic acid (TCA), and taurolithocholic acid (TLCA)), and increased abundance of soluble bile acids (tauroursodeoxycholic acid (TUDCA)). In contrast with proximal SBR, ileocecal resection alters enterohepatic circulation leading to reduced oxidative stress and promotes physiological bile acid metabolism. These findings challenge the notion that preservation of the ileocecal region is beneficial in patients with short bowel syndrome. Administration of selected bile acids may present potential therapy to mitigate resection-associated liver injury. III—Case-Control Study. • DDistal SBR and TUDCA supplementation decreases oxidative stress and SBR-associated hepatic injury. • Distal SBR and TUDCA supplementation have healthier bile acid profiles compared to proximal SBR. • Enterohepatic circulation has critical involvement in liver injury prevention, but distal SBR and TUDCA supplementation likely differ in the mechanism utilized to confer hepatoprotection. [ABSTRACT FROM AUTHOR]

Resection-associated liver steatosis, injury, and fibrosis is a devastating complication associated with massive small bowel resection (SBR). Peroxisome proliferator-activated receptor-alpha (PPARα) is a key regulator of intestinal lipid transport and metabolism whose expression is selectively increased after SBR. Here we asked if attenuating intestinal PPARα signaling would prevent steatosis and liver injury after SBR. Pparα was deleted selectively in adult mouse intestine using a tamoxifen-inducible Cre-LoxP breeding schema. Mice underwent 50% SBR. At 10 weeks post-operatively, metabolic phenotyping, body composition analysis, in vivo assessment of lipid absorption and intestinal permeability, and assessment of adaptation and liver injury was completed. Pparα intestinal knockout and littermate control mice were phenotypically similar in terms of weight trends and body composition after SBR. All mice demonstrated intestinal adaptation with increased villus height and crypt depth; however, Pparα intestinal knockout mice exhibited decreased villus growth at 10 weeks compared to littermate controls. Liver injury and fibrosis were similar between groups as assessed by serum AST and ALT levels, Sirius Red staining, and hepatic expression of Col1a1 and Acta2. Inducible intestinal deletion of Pparα influences structural adaptation but does not mitigate liver injury after SBR. These findings suggest that enterocyte PPARα signaling in adult mice is dispensable for resection-induced liver injury. The results are critical for understanding the contribution of intestinal lipid metabolic signaling pathways to the pathogenesis of hepatic injury associated with short bowel syndrome. • What is known? Resection-associated liver steatosis, injury, and fibrosis is a devastating complication associated with massive small bowel resection. Peroxisome proliferator-activated receptor-alpha is a key regulator of lipid transport and metabolism whose expression is selectively increased in the intestine after SBR. • What is new? Inducible intestinal deletion of PPARα, a major regulator of intestinal lipid metabolism, influences structural adaptation but does not mitigate liver injury after SBR. These findings suggest that enterocyte PPARα signaling in adult mice is dispensable for liver injury following SBR. [ABSTRACT FROM AUTHOR]

Prior to the late 1960s, pediatric short bowel syndrome was a frequently fatal disease. Currently, pediatric interdisciplinary bowel rehabilitation centers report very high survival rates. The mortality trends, up-to-date definitions, incidence, causes, and clinical manifestations of short bowel syndrome are reviewed. Emphasis is placed upon the nutritional, medical, and surgical advances that have contributed to the dramatic improvement in outcomes for pediatric short bowel syndrome patients. Recent findings and remaining challenges are highlighted. [ABSTRACT FROM AUTHOR]

• 44% of patients achieved enteral autonomy (EA) following autologous gastrointestinal reconstructive (AGIR) surgery. • Percentage of expected of small bowel length (≥15%) before AGIR is a strong predictor of achievement of EA. • The AGIR technique employed did not influence weaning from parenteral nutrition. • Aetiology of short bowel syndrome (gastroschisis) can affect outcomes and a tailored, multi-disciplinary approach is recommended. The 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. Retrospective review of all patients undergoing AGIR surgery in two tertiary paediatric surgical units, between 2010 and 2021. Continuous data is presented as median (range). Twenty-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%. AGIR 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. Retrospective Case-Series [ABSTRACT FROM AUTHOR]

The purpose of the study is to examine the long-term safety of an endoluminal bowel lengthening device prior to its use in the first human trial. In addition, device performance and natural passage will be evaluated. Endoluminal lengthening springs were surgically placed into the jejunum of Yucatan minipigs using the Eclipse XL1 device. A matching internal control segment of jejunum was marked at the time of operation. Weekly weights and fluoroscopic studies were obtained to evaluate spring deployment and position until devices passed. Animals were euthanized at 28, 60, 90, and 180 days. At necropsy, length measurements were recorded, and histopathologic analysis was performed. There were no bowel obstructions or overt perforations attributable to the device. All surviving animals gained weight and were clinically thriving. All devices passed out of the rectum by 180 days. Bowel lengthening was seen in all experimental segments, and minimal fibrosis was observed by 180 days. Jejunal lengthening persisted after device had passed through the intestinal tract after 180 days. Early histopathologic changes of the jejunum during distraction enterogenesis normalized over time. [ABSTRACT FROM AUTHOR]

• In the field of pediatric intestinal failure, there are limited data on the health-related quality of life (HRQOL) from the perspective of the child. • In this prospective study, children reported better HRQOL scores compared to parent-proxy data. Child-reported HRQOL scores increased with patient age. • Child-reported HRQOL scores in intestinal failure were lower than scores from reference populations of healthy children and children with chronic illness. Recent studies have focused on parent-reported health-related quality of life (HRQOL) in children with intestinal failure (IF). However, there is a paucity of data on HRQOL from the perspective of the child with IF. A prospective study of child self-reported HRQOL was performed in a regional intestinal rehabilitation program from 2015 to 2019. The PedsQL 4.0 Generic Core Scales were administered annually to children with IF ages five years and older along with their parents. Survey data was stratified by age and compared with parent-proxy scores and reference populations of healthy and chronically ill children. Linear mixed-effect models were constructed to identify associations with child self-reported HRQOL. A total of 140 surveys were administered to 69 children and their parents. Median child age at survey was 8 (IQR 6–10) years. Child self-reported HRQOL scores increased with each increasing age range. Children reported higher HRQOL scores compared to parent-proxy data in all age groups. Children with IF had lower HRQOL scores compared to healthy children in all survey dimensions (p < 0.001) and to children with chronic illness in the school and social functioning dimensions (p < 0.05). In adjusted analysis, longer remnant bowel length was independently associated with decreased HRQOL scores in children (p < 0.05). Children with IF reported better HRQOL compared to parent-proxy data. While these HRQOL scores improved with age, they remain significantly lower than healthy and chronically ill peers. The association between bowel length and child-reported HRQOL deserves further investigation. Level II [ABSTRACT FROM AUTHOR]

Background: Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD., Methods: The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD., Results: This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations., Conclusions: A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group's pre- and postoperative management., Level of Evidence: V., Competing Interests: Conflict of interest The authors report no conflict of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Short bowel syndrome (SBS) patients require total parenteral nutrition (TPN) following massive small bowel resection (SBR), which may cause intestinal failure-associated liver disease (IFALD), a life-threatening complication. Hepatocyte growth factor (HGF) acts as a potent hepatocyte mitogen with anti inflammatory and antioxidant actions. The present study evaluated the effect of recombinant human HGF (rh-HGF) on SBR and subsequent IFALD using a parentally fed rat model of SBS. Rats underwent jugular vein catheterization for continuous TPN and 90% SBR. They were divided into 2 groups: TPN alone (SBS/TPN group: n = 7) or TPN plus the intravenous administration of rh-HGF (0.3 mg/kg/day) (SBS/TPN+HGF group: n = 7). On day 7, their tissues and stool were harvested to evaluate the effects of HGF. Regarding the histological findings, based on the nonalcoholic fatty liver disease (NAFLD) activity score, the SBS/TPN+HGF group showed significantly less hepatic steatosis and inflammatory cell infiltration than the SBS/TPN group (NAFLD activity score, 4.00 ± 1.83 vs. 1.00 ± 0.82; p < 0.01). The SBS/TPN+HGF group showed a higher expression of Farnesoid X receptor in the liver and lower expression of Toll-like receptor 4 in the ileum than the SBS/TPN group. Regarding the composition of the bacterial gut microbiota, Actinobacteria, Bacteroidetes and Proteobacteria were decreased in the SBS/TPN+HGF group compared with the SBS/TPN group. In our SBS with TPN rat model, rh-HGF administration had a preventive effect against hepatic steatosis and dysbiosis. rh-HGF may therefore be a potentially effective therapeutic agent for SBS and subsequent IFALD. Experimental research. [ABSTRACT FROM AUTHOR]

Pediatric intestinal failure (PIF) affects nutrition, metabolism, and endocrine development, but its downstream impact on puberty is unknown. A retrospective review was performed of patients age >8 years with PIF managed at an intestinal rehabilitation program. Outcomes of interest were peak height velocity (PHV), age at PHV, and age at pubertal onset (Tanner stage 2). Outcomes were stratified by sex and compared to established norms. Of 110 patients with PIF, 54.5% were male. Compared to the CDC 50th percentile, PHV in PIF patients was similar for females (8.09±2.36 vs. 7.37 cm/yr; p = 0.23) but significantly higher for males (9.27±2.56 vs. 7.91 cm/yr; p = 0.038). Age at PHV in PIF patients was significantly younger for both males (12.31±2.14 vs. 13.38 years; p = 0.049) and females (10.70±1.06 vs. 11.71 years; p = 0.001). PIF patients reached pubertal onset earlier than published norms; this was significant for males (12.41±1.80 vs. 13.44 years; p = 0.014), but not for females (10.45±1.81 vs. -11.15 years; p = 0.13). The mean height-for-age Z-score was -1.2, with 20% of patients having a Z-score less than -2. Pubertal onset and growth are neither delayed nor diminished in patients with PIF. The high incidence of short stature, however, highlights the importance of optimizing prepubertal linear growth to attain full height potential. Type of Study: Prognosis study (Retrospective cohort study) [ABSTRACT FROM AUTHOR]

• Clarifies differences in the definition of NEC totalis. • Defines key ethical issues that arise during the care of patients with NEC totalis. • Provides ethical framework for addressing family and provider dilemmas. Necrotizing enterocolitis (NEC) totalis is a devastating disease of the newborn intestine. A precise clinical definition of the extent of gastrointestinal involvement is lacking in the existing literature, and the clinical outcomes are typically viewed as grim. Herein, we present a series of clinical case examples of patients with varying degrees of NEC totalis and other co-morbid conditions, with possible anticipated outcomes based on current data. We define the key ethical issues and provide a framework and discussion of the ethical issues involved in the care of patients with NEC totalis and recommendations of how to approach discussions with the family of these patients We discuss the ethical considerations for both the providers caring for these patients, and the patient's family members. The management of patients with NEC totalis is complex and ethically challenging. V [ABSTRACT FROM AUTHOR]

Background: Short bowel syndrome (SBS) is a rare but serious form of organ failure, and patients with SBS depend on total parenteral nutrition (PN) to maintain growth and development. The present study aimed to evaluate the experiences and outcomes of children with SBS managed by a multidisciplinary intestinal rehabilitation programme in a tertiary paediatric centre., Methods: A retrospective single-centre analysis of all paediatric patients with a clinical diagnosis of SBS between 2001 and 2022 was performed. Clinical outcomes and their predictors were extracted and analysed., Results: Of the 64 children included in the study, 43 (67%) had extensive necrotising enterocolitis. The median bowel length was 45 cm (interquartile range (IQR) = 18-65) and 18.9% (IQR = 10-28.5) of the expected length based on age. Over a mean follow-up period of 8.9 years, 57 patients (89%) survived, and 50 (78%) weaned off PN. The presence of intestinal failure-associated liver disease (IFALD) (OR = 6.375, p = 0.02) and patients managed before the introduction of fish oil-based PN in 2007 (OR = 5.895, p = 0.001) were significant predictors of mortality. There was an overall improvement in survival over time (p = 0.003). Ultrashort bowel length was not associated with significantly higher mortality (OR = 1.1, p = 0.65) but was a poor prognostic factor for weaning off PN (OR = 3.57, p = 0.004). Among all patients who weaned off PN, two had bowel lengthening procedures and one received a glucagon-like peptide 2 (GLP-2) analogue., Conclusions: A multidisciplinary intestinal rehabilitation programme offers a comprehensive approach for patients with SBS and has been shown to be effective with favourable outcomes. Improvements in the choice of PN and the development of new treatment strategies potentially improved the survival and enteral autonomy of SBS patients., Level of Evidence: III., (Copyright © 2024 Elsevier Inc. All rights reserved.)

short bowel syndrome is marked by inadequate intestinal surface area to absorb nutrients. Current treatments are focused on medical management and surgical reconfiguration of the dilated intestine. We propose the use of spring-mediated distraction enterogenesis as a novel intervention to increase intestinal length. Given our previous success lengthening intestinal segments using springs with spring constant ~7 N/m that exerts 0.46 N or higher, we sought to determine the minimal force needed to lengthen porcine small intestinal segments, and to explore effects on intestine over time. Juvenile Yucatan pigs underwent laparotomy with enterotomy to introduce nitinol springs intraluminally (n = 21 springs). Bowel segments (control, spring-distracted) were retrieved on post-operative day (POD) 7 and 14, and lengths measured. Thickness of cross-sectional intestinal layers were measured using H&E, and submucosal collagen fiber orientation measured using trichrome stained sections. all pigs survived to POD7 and 14. Spring constants of at least 2 N/m exerting a minimum force of 0.10 N significantly lengthened intestinal segments (p <0.0001). The stronger the spring force, the greater the induced thickness of various intestinal layers at POD7 and 14. Collagen fiber orientation was also more disordered because of stronger springs. a spring constant of approximately 2 N/m exerting 0.10 N and greater significantly lengthens intestinal segments and stimulates intestinal structural changes at POD7 and 14. This suggests a decreased force is capable of inducing spring-mediated distraction enterogenesis. [ABSTRACT FROM AUTHOR]

Following a serial transverse enteroplasty (STEP) procedure some children develop redilation of the small intestine leading to impaired enteral tolerance and inability to wean parenteral nutrition (PN). The benefit of a second STEP procedure (2STEP) has been controversial. We performed a retrospective review of our experience (2008–2018) performing 2STEP, with comparative analysis of nutritional outcomes pre- and postsurgery. During this period 2STEP was performed in 23 patients (13 F:10 M) at a median (25%–75%) age of 2.2 (1.2–3.6) years. Median intestinal length was 68 (40–105) cm before and 85 (40–128) cm after 2STEP. Leading up to 2STEP, PN provided almost 75% of estimated calorie needs. By 24 weeks following 2STEP drops in mean PN percent approached statistical significance (p = 0.07) and at most recent follow up the mean PN percentage was statistically better than at the time of operation or 4 weeks prior to 2STEP, and was nearly significant compared with 12 weeks (p = 0.07) and 24 weeks (p = 0.06) prior. Thirteen children were completely off parenteral support. When small intestine redilation occurs following a STEP procedure and where PN cannot otherwise be weaned we believe these data support performing a 2STEP. We cannot predict preoperatively which children will ultimately benefit. 3 (retrospective comparative study). [ABSTRACT FROM AUTHOR]

Increasingly, for pediatric patients with short bowel syndrome (SBS), intestinal lengthening procedures such as serial transverse enteroplasty (STEP) are being offered with the hope of improving patients' chances for achieving enteral autonomy. However, it remains unclear to what extent STEP reduces the long-term need for intestinal transplant or improves survival. Based on existing literature, a decision analytic Markov state transition model was created to simulate the life of 1,000 pediatric SBS patients. Two simulations were modeled: 1) No STEP: patients were listed for transplant once medical management failed and 2) STEP: patients underwent STEP therapy and subsequent transplant listing if enteral autonomy was not achieved. Sensitivity analysis of small bowel length and anatomy was completed. Base case patients were defined as neonates with a small bowel length of 30cm. For base case patients with an ostomy and a NEC SBS etiology, STEP was associated with increased rates of enteral autonomy after 10 years for patients with an ICV (53.9% [STEP] vs. 51.1% [No STEP]) and without an ICV (43.4% [STEP] vs. 36.3% [No STEP]). Transplantation rates were also reduced following STEP therapy for both ICV (17.5% [STEP] vs. 18.2% [No STEP]) and non-ICV patients (20.2% [STEP] vs. 22.1% [No STEP]). 10-year survival was the highest in the (+) STEP and (+) ICV group (85.4%) and lowest in the (-) STEP and (-) ICV group (83.3%). For SBS patients, according to our model, STEP increases rates of enteral autonomy, reduces need for intestinal transplantation, and improves long-term survival. Economic/Decision Analysis or Modeling Studies Level III [ABSTRACT FROM AUTHOR]

Studies about differences in microbial communities between the small intestine and colon in infants with short bowel syndrome (SBS) are rare. We aimed to characterize the bacterial diversity of small bowel stoma effluents and feces of SBS infants. Seven SBS infants were enrolled in this study and provided two samples (one from the stoma and the other from the anus) each. Eleven age-matched healthy controls were recruited to provide one fecal sample each. 16S rRNA gene MiSeq sequencing was conducted to characterize the microbiota diversity and composition. The bacterial diversity of the stoma effluents was significantly higher than that in the feces of SBS infants. Proteobacteria dominated in both the stoma effluents and colonic. Acinetobacter (P = 0.004), Klebsiella (P = 0.015), Citrobacter (P = 0.019), and Lactobacillus (P = 0.030) were more abundant in stoma effluents compared to feces of SBS patients, while Bacteroidetes, Bifidobacterium and Veillonella were less abundant in stoma effluents. Significantly higher levels of Proteobacteria, Enterococcus and lower levels of Blautia , Collinsella , Faecalibacterium , Veillonella were present in the fecal samples of SBS patients than those in the healthy controls. Kyoto Encyclopedia of Genes and Genomes pathways related to metabolism and membrane function were depleted in SBS patients. The predominant intestinal bacterial groups were different in SBS children before and after the fistula closure. Fecal samples of SBS patients featured overabundant Proteobacteria and less SCFA producing bacteria. Depleted functional profiles of the microbiome were found in fecal samples of SBS patients. III. [ABSTRACT FROM AUTHOR]

The optimal regimen for enteral nutritional support in the management of children with short bowel syndrome (SBS) is not well characterized. A high fat, enteral diet is theoretically beneficial due to increased caloric density and enhanced structural adaptation. We therefore sought to determine the long-term effects of a high fat diet (HFD) on liver injury, a common complication of SBS, compared to a standard chow (SC) diet. Using a parenteral nutrition-independent model of resection-associated liver injury, C57BL/6 mice underwent a sham operation or a 50% or 75% proximal small bowel resection (SBR). Mice in each group were then fed either a HFD (35% kcal fat) or SC (13% kcal fat). At post-operative week 15, markers of liver injury were quantified. Liver triglyceride levels were increased from 7- to 19-fold in mice on the HFD compared to mice fed SC in the sham, 50%, and 75% resection groups. Serum ALT (2.2-fold increase in 75% resected mice compared to sham controls) and AST (2.0- and 2.7-fold increases in 50% and 75% resected mice, respectively) levels as well as fibrotic liver staining were elevated only in resected mice fed a HFD. Long-term enteral feeding of HFD in our murine SBS model is associated with hepatic steatosis and liver injury. Our observation that liver steatosis and injury occur independent of parenteral nutrition suggests that enteral feeding composition and magnitude of intestinal loss may make a significant contribution to intestinal failure-associated liver disease. [ABSTRACT FROM AUTHOR]

Babies with short bowel syndrome (SBS) have small intestinal microbial disturbances that impact gut function. Characterizing the small bowel microbiota is challenging, and the utility of sampling stool is unclear. This study compares the microbiota from fecal samples and the small bowel. Stool samples were collected (2016–2017) from infants with SBS and colon in continuity (COLON) or SBS with small bowel ostomy (sbSTOMA). The abundance and quantity of major bacterial genera was compared between groups and to healthy controls using 16S rRNA sequencing and qPCR. Kruskall-Wallis test was used for analysis with P values < 0.05 considered significant. Samples (n = 41) were collected from 15 SBS infants (< 2 years) (9 sbSTOMA, 6 COLON) and 3 healthy infants. Demographics and small intestinal length did not differ between sbSTOMA and COLON infants. The microbiota of SBS groups differed significantly from healthy controls. Fecal samples contained higher quantities of bacteria, but there were no significant differences between sbSTOMA and COLON groups in the abundance of facultative or obligate anaerobes, anti-inflammatory Clostridia, Enterobacteriaceae, or Bifidobacterium. Infants with SBS have disturbances to their intestinal microbiota. Sampling small intestinal effluent is challenging. Stool samples may provide a window into the more proximal microbial community. Diagnostic. Level II. [ABSTRACT FROM AUTHOR]

We sought to describe long-term outcomes of infants with severe surgical necrotizing enterocolitis (NEC). Data were collected on infants with surgical NEC (2009–2018). Severe surgical NEC was defined by extensive bowel loss with residual bowel length < 30 cm, and "NEC totalis" was identified per operative report. Post-operative management and long-term outcomes were assessed, including enteral autonomy, severe neurodevelopmental disability, and educational attainment. Of 268 infants with surgical NEC, 41 (15%) had severe surgical NEC, and 14/41 were identified as "NEC totalis". Zero severe NEC vs. 8 (57%) "NEC totalis" patients were placed on comfort measures following initial surgery (p < 0.001). Twenty-five patients (93%) with severe NEC survived vs 3/6 with "NEC totalis" (p < 0.001). The 28 survivors (68%) were followed for a median (IQR) duration of 8(4,10) years. Nine (32%) with severe NEC were weaned from parenteral nutrition. Eight (29%) had ≥ 1 marker for severe neurodevelopmental disability, and 11/16(69%) (7–16 years) were attending school at last follow-up. Long-term survival is excellent following initial discharge, and achievement of enteral autonomy is feasible in patients with severe surgical NEC. The majority of patients who survive do not have severe neurodevelopmental disability and participate in school. Given current survivals and outcomes, focus on measured residual bowel length may be more appropriate than the subjective term "NEC-totalis." Prognosis Study. Level II. [ABSTRACT FROM AUTHOR]

Spring-mediated intestinal lengthening has been studied in numerous animal models to effectively achieve up to a 3-fold increase in length. In this study we are interested in optimizing this method of spring lengthening. Juvenile mini-Yucatan pigs underwent laparotomy for spring implantation. Springs were secured by plicating the intestine around the springs. In one set of experiments, varying degrees of plication were compared to determine the necessary narrowing needed to confine the spring. In another set of experiments, dissolvable sutures were used for the plication to allow for spontaneous spring passage postoperatively. Intestinal segments were retrieved and evaluated for lengthening and histological changes. Pigs tolerated their diet advancement to a regular diet postoperatively. 10% plication resulted in a 1.3-fold increase in length, while 50% plication resulted in a 2.7-fold increase in length (p < 0.05). At two months postoperatively, the majority of springs had safely passed out of the intestine. All lengthened intestine showed significant growth histologically. A 50% reduction in lumen diameter achieves optimal spring-mediated intestinal lengthening. Springs can safely pass out of the intestine, thus avoiding a second operation for spring removal. These results may be important in developing future therapies for short bowel syndrome. Level I experimental study. [ABSTRACT FROM AUTHOR]

The study aims to describe long-term outcomes and disease burden of neonatal onset short bowel syndrome (SBS). Utilizing the WHO criteria for adolescence, patients 10–19 years of age with neonatal onset SBS requiring parenteral nutrition (PN) for > 90 days and followed by our multidisciplinary intestinal rehabilitation center between 2009 and 2018 were included for analysis. Seventy adolescents with SBS were studied. Median (IQR) age at last follow up in our center was 15 (11, 17) years. There was 0% mortality in the cohort, and 94% remained transplant free. Fifty-three patients (76%) achieved enteral autonomy. Three patients were weaned from PN without transplantation after six years of follow-up and another four after ten years of care at our multidisciplinary center. Disease burden remained higher in adolescents receiving PN, including inpatient hospitalizations (p < 0.01), procedures (p = 0.01), clinic visits (p < 0.01), and number of prescribed medications (p < 0.01). Survival for adolescents with neonatal onset SBS is excellent. Of the cohort studied, there was no mortality, and more than 75% achieved enteral autonomy. Disease burden remains high for adolescents who remain dependent on PN. However, achievement of enteral autonomy is feasible with long-term multidisciplinary rehabilitation. Prognosis study. Level II. [ABSTRACT FROM AUTHOR]

Short bowel syndrome is associated with intestinal mucosal inflammation and microbial dysbiosis, leading to intractable complications. Partially hydrolyzed guar gum (PHGG) has trophic and anti-inflammatory effects on the intestine. We investigated whether PHGG ameliorates small intestinal mucosal damage and alters the intestinal microbiota using a rat small bowel resection (SBR) model. Sprague Dawley rats were divided into sham operation (Sham), Sham/PHGG, SBR, and SBR/PHGG groups. On day 21, all rats were euthanized. To assess small intestinal mucosal damage, the degeneration rate was morphometrically evaluated and immunohistochemically examined using anti-CD45 antibodies. Analyses of fecal microbiota using 16S rRNA and short-chain fatty acid production were also performed. The mucosal degeneration rate was significantly higher in the SBR group than in the Sham or SBR/PHGG groups. The number of CD45-positive cells was significantly higher in the SBR group than in the Sham, Sham/PHGG, or SBR/PHGG groups. The relative abundance of family Lachnospiraceae was significantly higher in the SBR/PHGG group than in the SBR group. PHGG administration alleviated small intestinal mucosal damage which could be associated with modulation of the intestinal microbiota. [ABSTRACT FROM AUTHOR]

The purpose of the study was to determine the accuracy of acoustic radiation force impulse (ARFI) ultrasound elastography in assessing the degree of liver disease in children with short bowel syndrome (SBS). A prospective observational cohort study of patients with SBS who underwent a liver biopsy and ARFI elastography was performed. Mean shear wave speed (SWS) and stage of fibrosis was evaluated using t-tests. Receiver operating characteristic curves (ROC) were generated and the area under the curves (AUC) estimated in order to assess the accuracy of SWS measurements to discriminate between stages of fibrosis. Thirty-seven paired elastography and biopsy samples from 31 patients were included. The median age was 0.6 years, and 61% were male. There was a significant positive correlation between stage of fibrosis and mean SWS (β = 0.16 m/s increase per stage, p =<0.001). ROC analysis revealed that mean SWS had good accuracy for discriminating between mild liver fibrosis (F0-F1) and moderate to severe fibrosis (F2-F4) (AUC = 0.80, 95% CI 0.65-0.95). In addition, ROC analysis demonstrated that mean SWS can also accurately discriminate between mild to moderate fibrosis (F0-F2) and more severe fibrosis (F3-F4) (AUC = 0.84, 95% CI 0.71-0.96). ARFI elastography is an accurate, non-invasive method to monitor liver disease in children with SBS. Retrospective Cohort Study II [ABSTRACT FROM AUTHOR]

The serial transverse enteroplasty (STEP) tapers and lengthens the gut to improve adaptation. Mortality has decreased with multidisciplinary intestinal rehabilitation programs (IRP) allowing more time to reach adaptive potential. We reviewed our STEP experience to compare surgical outcomes between early and late eras of our IRP. A retrospective cohort study of all STEP patients managed by our IRP (Jan 2003–Dec 2016; era 1 2003–2005, era 2 2006–2016) was completed. Patient demographics, operative data, complications, and outcome data were collected. Univariate analysis between eras with nonparametric statistics was performed. Thirty-six patients received STEP (Era 1 = 12; Era 2 = 24) [median age 5mo; males 22/36 (61.1%)]. In Era 2 a higher proportion had gastroschisis (8.3% vs 58.3%); p = 0.004) and shorter pre-STEP small bowel remnant (48 vs 111 cm, p = 0.001). The median increase in bowel length post-STEP was 52.9%. Overall, 42% of patients reached enteral autonomy (Era 1 7/12 (58%) vs Era 2 8/24 (33%); p = 0.15). Median time to PN discontinuation was shorter in Era 1 (259 vs 968 days, p =.208). Staple line complications were higher in Era 1 (16.7% vs 0%; p = 0.040). Presently, STEP is reserved for a specific subset of IRP patients, allowing 42% to wean off PN. STEP's use should be under the umbrella of a multidisciplinary IRP. Retrospective cohort study. III [ABSTRACT FROM AUTHOR]

Abstract Purpose Quantify short-term outcomes associated with initial surgery [laparotomy (LAP) vs. peritoneal drain (PD)] for necrotizing enterocolitis (NEC) in extremely-low-birth-weight (ELBW) infants. Methods Using the Children's Hospitals Neonatal Database, we identified ELBW infants < 32 weeks' gestation with surgical NEC (sNEC). Unadjusted and multivariable regression analyses were used to estimate the associations between LAP (or PD) and death/short bowel syndrome (SBS) and length of stay (LOS). Results LAP was the more common initial procedure for sNEC (n = 359/528, 68%). Infants receiving LAP were older and heavier. Initial procedure was unrelated to death/SBS in both bivariate (LAP: 43% vs PD: 46%, p = 0.573) and multivariable analyses (OR = 0.89, 95% CI = 0.57, 1.38, p = 0.6). LAP was inversely related to mortality (29% vs. 41%, p < 0.007) in bivariate analysis, but not significant in multivariable analysis accounting for markers of preoperative illness severity. However, the association between LAP and SBS (14% vs. 5%, p = 0.012) remained significant in multivariable analyses (adjusted OR = 2.25, p = 0.039). LOS among survivors was unrelated to the first surgical procedure in multivariable analysis. Conclusion ELBW infants who undergo LAP as the initial operative procedure for sNEC may be at higher risk for SBS without a clear in-hospital survival advantage or shorter hospitalization. Level of evidence Level II. [ABSTRACT FROM AUTHOR]

Abstract Background Short bowel syndrome is a debilitating condition with few effective treatments. Spring-mediated distraction enterogenesis can be used to lengthen intestine. The purpose of this study is to determine whether multiple springs in series can safely increase the total amount of lengthening. Methods Juvenile mini-Yucatan pigs each received three nitinol springs placed within their jejunum. Plication was used to narrow the intestine around each spring to secure them. Compressed springs were used in the experimental group, while uncompressed springs were used in the control group. The intestine was examined 3 weeks later for lengthening and histologic changes. Results All pigs tolerated diets postoperatively with continued weight gain, and no dilation or obstruction of the intestine was observed. Segments of intestine that contained compressed springs had a significant increase in length from 2.5 cm to 3.9 ± 0.2 cm per spring, compared to segments containing control springs that showed no change (p < 0.001). Conclusions Intestinal plication can be safely used to secure multiple springs in series to achieve intestinal lengthening without compromising intestinal function. Using several springs at once allows for a greater amount of total lengthening. This is a promising model that has potential in the treatment of short bowel syndrome. [ABSTRACT FROM AUTHOR]

Abstract Aims and objectives Fish oil (FO) lipid emulsion and a new lipid emulsion (SMOF) are important treatments for intestinal failure-associated liver disease. We evaluated the efficacy of FO and SMOF lipid emulsion on intestinal mucosal adaptation using a total parenteral nutrition (TPN)-supported rat model of short bowel syndrome. Material & methods Sprague–Dawley rats underwent jugular vein catheterization and 90% small bowel resection and were divided into three groups: TPN with soy bean oil lipid emulsion (SO group), FO lipid emulsion (FO group), or SMOF (SMOF group). On day 13, the rats were euthanized, and the small intestine was harvested. The microscopic morphology and crypt cell proliferation rate (CCPR) were then evaluated. Results The villus height of the ileum in the SMOF group was significantly higher than in the SO group. The crypt depth of the intestine in the SMOF group was significantly lower than in the SO group. The CCPRs of the intestine in the FO and SMOF groups were both higher than in the SO group. Conclusions Lipid emulsion affected the bowel morphology, such as the mucosa as well as the intestinal smooth muscle. Further studies are needed to clarify the mechanisms. [ABSTRACT FROM AUTHOR]

Abstract Introduction Despite medical and surgical management, a subset of children with short bowel syndrome (SBS) who have discrepancy between proximal small bowel and distal colon have persistent feeding intolerance. We propose the use of an Ostomy in Continuity (OIC) (Bishop-Koop or Santulli) as a salvage procedure to decompress the proximal bowel while still maintaining maximal intestinal length, in these patients. Methods A retrospective chart review of 104 SBS patients identified sixteen patients who underwent an OIC. Measures of reliance on parenteral nutrition (PN), growth, intestinal failure associated liver disease, the rate of central venous catheter infections and enterocolitis were collected. These parameters were compared before and after the placement of OIC in the same patients at a median follow-up period of 24 months (range 3–52 months). Outcome measures include intestinal autonomy and survival without intestinal and liver transplant. Results All 16 patients showed significant improvement in their enteral tolerance after OIC. The mean PN caloric requirement decreased from 95% to 21% (p = 0.0001). The median weight Z score improved from − 1.18 to 0.20 (p = 0.0006) and the median height Z score improved from − 2.74 to − 1 (p = 0.0001). The mean conjugated bilirubin decreased from 10.3 mg/dl to 0.3 mg/dl (p = 0.0001) in nine patients with hyperbilirubinemia. There was no decrease in central venous catheter infections (CVCI) but there was a decrease in the rate of enterocolitis. None of the patients required intestinal or liver transplant. There was one minor skin excoriation complication in one patient with a Bishop-Koop stoma. Conclusions The application of an ostomy in continuity within a comprehensive intestinal rehabilitation program is a novel approach in the treatment of refractory short bowel syndrome that improves intestinal autonomy and decreases the rate of enterocolitis. Type of Study Case Series. Level of evidence IV (Cohort Study). [ABSTRACT FROM AUTHOR]

Background Spring-mediated distraction enterogenesis has been shown to increase the length of an intestinal segment. The goal of this study is to use suture plication to confine a spring within an intestinal segment while maintaining luminal patency to the rest of the intestine. Methods Juvenile mini-Yucatan pigs underwent placement of nitinol springs within a defunctionalized Roux limb of jejunum. A 20 French catheter was passed temporarily, and sutures were used to plicate the intestinal wall around the catheter at both ends of the encapsulated spring. Uncompressed springs placed in plicated segments and springs placed in nonplicated segments served as controls. The intestine was examined approximately 3 weeks after spring placement. Results In the absence of plication, springs passed through the intestine within a week. Double plication allowed the spring to stay within the Roux limb for 3 weeks. Compared to uncompressed springs that showed no change in the length of plicated segments, compressed springs caused a significant 1.7-fold increase in the length of plicated segments. Conclusions Intestinal plication is an effective method to confine endoluminal springs. The confined springs could lengthen intestine that maintains luminal patency. This approach may be useful to lengthen intestine in patients with short bowel syndrome. Level of Evidence Level I Experimental Study. [ABSTRACT FROM AUTHOR]

Background The advent of regional multidisciplinary intestinal rehabilitation programs has been associated with improved survival in pediatric intestinal failure. Yet, the optimal timing of referral for intestinal rehabilitation remains unknown. We hypothesized that the degree of intestinal failure-associated liver disease (IFALD) at initiation of intestinal rehabilitation would be associated with overall outcome. Methods The multicenter, retrospective Pediatric Intestinal Failure Consortium (PIFCon) database was used to identify all subjects with baseline bilirubin data. Conjugated bilirubin (CBili) was used as a marker for IFALD, and we stratified baseline bilirubin values as CBili < 2 mg/dL, CBili 2–4 mg/dL, and CBili > 4 mg/dL. The association between baseline CBili and mortality was examined using Cox proportional hazards regression. Results Of 272 subjects in the database, 191 (70%) children had baseline bilirubin data collected. 38% and 28% of patients had CBili > 4 mg/dL and CBili < 2 mg/dL, respectively, at baseline. All-cause mortality was 23%. On univariate analysis, mortality was associated with CBili 2–4 mg/dL, CBili > 4 mg/dL, prematurity, race, and small bowel atresia. On regression analysis controlling for age, prematurity, and diagnosis, the risk of mortality was increased by 3-fold for baseline CBili 2–4 mg/dL (HR 3.25 [1.07–9.92], p = 0.04) and 4-fold for baseline CBili > 4 mg/dL (HR 4.24 [1.51–11.92], p = 0.006). On secondary analysis, CBili > 4 mg/dL at baseline was associated with a lower chance of attaining enteral autonomy. Conclusion In children with intestinal failure treated at intestinal rehabilitation programs, more advanced IFALD at referral is associated with increased mortality and decreased prospect of attaining enteral autonomy. Early referral of children with intestinal failure to intestinal rehabilitation programs should be strongly encouraged. Level of evidence Treatment Study, Level III. [ABSTRACT FROM AUTHOR]

Background Short bowel syndrome (SBS) is the most common cause of intestinal failure in children. Many factors have been investigated in an attempt to define which parameters influence most survival and ability to wean off parenteral nutrition (PN). The aim of this study was to investigate if aetiology of SBS affects the outcomes in paediatric patients treated with autologous gastrointestinal reconstructive surgery. Methods All children with SBS who underwent autologous gastrointestinal reconstructive surgery between 2002 and 2012 were retrospectively reviewed and outcome measures were recorded. Results Forty-three patients were divided into 4 groups according to aetiology (gastroschisis, volvulus, necrotizing enterocolitis (NEC), intestinal atresia). No significant differences were found among groups regarding survival and median age at surgery. The volvulus group had a lower pre-operative bowel length in comparison with gastroschisis and intestinal atresia and the lowest percentage of patients off PN (30%). Gastroschisis had the lowest rate of preserved ileocaecal valve (10%), while intestinal atresia had the highest (66%). For children who weaned off PN, intestinal atresia had also the longest time to achieve enteral autonomy (14.5 months), while NEC had the shortest (3.5 months), followed by gastroschisis (8.5 months). None of the patients needed transplant. Conclusions In our experience it does not appear that diagnosis is significantly related to outcome and this is consistent with the conclusions of other reviews. However, it should be noted that in our series patients with volvulus had the worse outcome in terms of weaning off PN when compared with intestinal atresia. Type of Study Retrospective Study. Level of Evidence II [ABSTRACT FROM AUTHOR]

Background Short bowel syndrome occurs following massive small bowel resection (SBR) and is one of the most lethal diseases of childhood. We have previously demonstrated hepatic steatosis, altered gut microbiome, and increased fat deposition in our murine model of SBR. These novel findings prompted us to investigate potential alterations in glucose metabolism and systemic inflammation following intestinal resection. Methods Male C57BL6 mice underwent 50% proximal SBR or sham operation. Body weight and composition were measured. Fasting blood glucose (FBG), glucose, and insulin tolerance testing were performed. Small bowel, pancreas, and serum were collected at sacrifice and analyzed. Results SBR mice gained less weight than shams after 10 weeks. Despite this, FBG in resected mice was significantly higher than sham animals. After SBR, mice demonstrated perturbed body composition, higher blood glucose, increased pancreatic islet area, and increased systemic inflammation compared with sham mice. Despite these changes, we found no alteration in insulin tolerance after resection. Conclusions After massive SBR, we present evidence for abnormal body composition, glucose metabolism, and systemic inflammation. These findings, coupled with resection-associated hepatic steatosis, suggest that massive SBR (independent of parenteral nutrition) results in metabolic consequences not previously described and provides further evidence to support the presence of a novel resection-associated metabolic syndrome. [ABSTRACT FROM AUTHOR]

Purpose The purpose of this study was to investigate whether anticoagulation (AC) results in thrombus resolution and increased line longevity in children with intestinal failure (IF) and catheter-associated central venous thrombosis (CVT). Methods A retrospective, single institution review was performed of children with IF who were dependent on parenteral nutrition with known CVT between 2006 and 2017. Frequency of catheter-related complications including infection, occlusion, and breakage were compared 18 months prior to and after starting AC. Thrombus resolution during anticoagulation was also determined. Data were analyzed using Poisson regression. p-Values < 0.05 were considered significant. Results Eighteen children had ≥ 1 CVT, with the subclavian vein most commonly thrombosed (44%). All children were treated with low molecular weight heparin, and 6 patients (33%) had clot resolution on re-imaging while receiving AC. Bloodstream infections decreased from 7.9 to 4.4 per 1000 catheter days during AC (p = 0.01), and the number of infections requiring catheter replacement decreased from 3.0 to 1.0 per 1000 catheter days (p = 0.01). There were no significant differences in line occlusions or breakages. Conclusion Anticoagulation for children with intestinal failure and central venous thrombosis may prevent thrombus propagation, and decrease blood stream infections and line replacements. Further research is needed to determine optimal dosing and duration of therapy. Level of Evidence III; Retrospective Comparative Study. [ABSTRACT FROM AUTHOR]

Purpose Small bowel length is the most reliable predictor of enteral independence in pediatric short bowel syndrome. Retrospectively measured bowel lengths on upper GI with small bowel follow-through (UGI/SBFT) were compared to operative measurements. Methods A pediatric radiologist and surgical trainees blinded to operative measurements retrospectively analyzed UGI/SBFT studies using the digital radiography curved measurement tool. Children with SBS and severe intestinal failure (parenteral nutrition > 90 days) at a multidisciplinary intestinal failure program 2002–2015 were included. Data were expressed as median (Q1, Q3). Results Thirty-six children aged 0.8 (0.4, 3.7) years were analyzed. Fifty-six percent had intestinal malrotation, and 58% had prior serial transverse enteroplasty. Studies were conducted within 10 (7, 20) days of surgery. Intraoperative bowel length was 90 cm (45, 142), while UGI/SBFT measurement by radiologist was 45 cm (28, 63), with a mean difference of 47 cm (SD 58 cm, p < 0.001) and a mean percent error of 50%. Radiographic assessment underestimated intestinal length in 83% of patients. Conclusion Bowel length measured retrospectively from upper GI with small bowel follow-through studies usually underestimated intraoperative bowel length. The limits of agreement were too wide for this technique to be clinically useful. Operative measurement remains necessary to assess intestinal length and rehabilitation potential. Type of study Study of Diagnostic Test. Level of evidence Level III. [ABSTRACT FROM AUTHOR]

Background The gut microbiota plays a vital role in modulating the metabolic and immune functions of the intestines. We aimed to analyze the dysbiosis of microbiota in infants with short bowel syndrome (SBS) with different complications. Procedure We included 26 fecal samples from 18 infants with SBS during parenteral nutrition. The samples were categorized into three groups: asymptomatic, parenteral nutrition-associated liver disease (PNALD), and central line-associated bloodstream infection (CLABSI). Seven healthy infants were enrolled as controls. Fecal microbiota, secretory IgA, calprotectin, bile acids, and short chain fatty acids were detected. Results The bacterial diversity of the Asymptomatic and Control Groups was significantly higher than that in the PNALD and CLABSI Groups. Proteobacteria was the most pronounced phylum in the PNALD and CLABSI Groups. Decreased acetate was observed in all SBS samples; however, fecal secretory IgA and calprotectin and the proportion of primary and secondary bile acids did not differ from those in healthy controls. Conclusions Marked alterations of the intestinal microbiota with decreased level of acetate were shown in SBS patients compared with healthy controls. Over-abundance of Proteobacteria (especially Enterobacteriaceae ) was found in the samples from the PNALD and CLABSI Groups. Level of evidence Prognosis Study, Level I. [ABSTRACT FROM AUTHOR]

Background In remains unclear why in some short bowel syndrome (SBS) patients, the remaining small bowel (SB) dilates excessively leading to requirement of tapering surgery. Methods Among SBS children, we retrospectively analyzed risk factors for tapering surgery with logistic regression and compared the outcome of operated patients ( n = 16) to those managed conservatively ( n = 44) with Cox proportional hazards regression. Results SBS was caused by necrotizing enterocolitis (NEC) ( n = 31), SB atresia (SBA) ( n = 13), midgut volvulus ( n = 12), or gastroschisis ( n = 4). Patients with spontaneous symptomatic SB dilatation unable to wean parenteral nutrition (PN) underwent tapering surgery at median age of 1.04 (interquartile range 0.70–3.27) years. Missing ICV was related to an 8-fold ( p = 0.003) increased risk while SBA diagnosis was related to a 13-fold risk of tapering surgery ( p < 0.001). Increasing SB length and NEC diagnosis were protective of tapering ( p = 0.027–0.004). Of operated patients, 75% reached enteral autonomy during follow-up and their postoperative adjusted PN weaning rate was similar to nonoperated children ( p = 0.842). Conclusion SBS children with short remaining SB, missing ICV, and SBA etiology are more likely while NEC patients are less likely than others to necessitate tapering surgery. Postoperative PN weaning rates were comparable to patients who initially had more favorable intestinal anatomy and adapted without surgery. [ABSTRACT FROM AUTHOR]

Background Our objective was to evaluate the accuracy of acoustic radiation force impulse (ARFI) elastography in determining extent of liver fibrosis in pediatric patients with short bowel syndrome (SBS). Methods This prospective cohort study included pediatric patients with SBS who underwent ultrasound with ARFI measurements of shear wave speed (SWS) and liver biopsy within 30 days of each other between 12/2014–9/2015. The mean and median SWS were compared to the stage of fibrosis. Receiver operating characteristic curves (ROC) were also generated to assess the classification accuracy of SWS measurements for mild (F0-F1) versus moderate/severe fibrosis (F2-F4). Results Twelve patients were included with median age 1.4 years and median weight 11.3 kg. The median direct bilirubin level was 0.3 mg/dL at the time of biopsy. On liver biopsy, 6 patients had mild fibrosis (F1), 3 had septal fibrosis (F3), and 3 had cirrhosis (F4). The area under the ROC curve for elastography to differentiate moderate/severe liver fibrosis from mild disease was 0.83 (95CI 0.58–1.00) and 0.86 (95CI 0.63–1.00) for the median SWS and mean SWS, respectively. Conclusions These early results suggest that ARFI ultrasound elastography may be a reliable non-invasive method to monitor liver fibrosis in pediatric patients with SBS. Type of study Prospective observational cohort. Level of evidence 3. [ABSTRACT FROM AUTHOR]

Background A significant number of children with short bowel syndrome experience intestinal failure-associated liver disease. We recently demonstrated accelerated hepatic steatosis after 50% small bowel resection (SBR) in mice. Since SBR is associated with alterations in the gut microbiome, the purpose of this study was to determine whether TLR4 signaling is critical to the development of resection-associated hepatic steatosis. Methods Male C57BL6 (control) and TLR4-knockout (KO) mice underwent 50% proximal SBR. Liver sections were analyzed to obtain the percent lipid content, and Ileal sections were assessed for morphological adaptation. Intestinal TLR4 mRNA expression was measured at 7 days and 10 weeks. Results Compared to controls, TLR4 KO mice demonstrated similar weight gain and morphological adaptation after SBR. Hepatic steatosis was decreased 32-fold in the absence of TLR4. Intestinal TLR4 mRNA expression was significantly elevated 7 days after SBR. We also found that TLR4 expression in the intestine was 20-fold higher in whole bowel sections compared with isolated enterocytes. Conclusions TLR4 signaling is critical for the development of resection-associated steatosis, but not involved in intestinal adaptation after massive SBR. Further studies are needed to delineate the mechanism for TLR4 signaling in the genesis of resection-associated liver injury. Level of evidence Animal study, not clinical. [ABSTRACT FROM AUTHOR]

Purpose Children with short bowel syndrome (SBS) often require numerous operations to optimize intestinal function. Postoperative intestinal strictures are a complication that inhibits enteral feeding advancement and prolongs parenteral nutrition dependency, often requiring reoperation. Our objective was to review our experience with fluoroscopic balloon dilatation to treat intestinal strictures. Methods A retrospective cohort study of intestinal failure patients with SBS was completed. Patients who had radiologically diagnosed intestinal strictures and treated with fluoroscopic guided balloon dilatation were included [n = 6]. Data related to demographics, anatomy, surgical procedures, and dilatation procedures were collected. Descriptive summary statistics were employed. Results 98 intestinal failure patients were recruited between 2011 and 2015. Five of 98 patients (5.1%) [2 males; median age 4.4 months] underwent fluoroscopy guided balloon dilatation of 6 strictures. Balloon dilatation was successful in 4/6 (67%). The median number of dilatations was 2 per patient (range = 1–3). Median time to feed initiation postdilatation was 3 days. One patient developed an anastomotic leak after dilatation that required antibiotics, but no reoperation. Conclusion Four of six (67%) postoperative bowel strictures in 5 patients with SBS were successfully treated with fluoroscopically guided balloon dilatation. Balloon dilatation is less invasive than reoperation, preserves bowel length and reduces time to reinitiation of enteral feeding. Level of evidence 3. [ABSTRACT FROM AUTHOR]

Purpose Short bowel syndrome (SBS) is a highly morbid condition primarily because of parenteral nutrition (PN)–associated complications. Bowel lengthening via serial transverse enteroplasty (STEP) has become standard of care. While initial STEPs have resulted in weaning from PN, outcomes of repeated STEPs (ReSTEPs) are not well described. We investigated outcomes of initial STEP compared to ReSTEP procedures. Methods This retrospective review of STEPs included 17 children and a total of 24 procedures. Demographics, complications, hospital readmission rates, postoperative costs, and PN weaning were analyzed. Results Neither patient-specific data nor the etiology of SBS was predictive of requiring a ReSTEP. PN weaning was more likely in the year following a first STEP (18% wean rate vs. 0% for ReSTEP, p > .05). No ReSTEP patients reached enteral autonomy. Enteral nutrition (%EN) increases were greater after first STEP compared to ReSTEP (26.0% vs. 4.7%, p = 0.03). This trend was true for bowel length as well, where first STEPs resulted in a 51% increase in bowel length compared to a 20% increase after in ReSTEP ( p = 0.02). Conclusions ReSTEPs failed to result in significant PN weaning, with no ReSTEP patients achieving enteral autonomy during follow-up. Given its higher costs, smaller bowel length gains, and limited ability to produce enteral autonomy, surgeons should carefully consider performing ReSTEP procedures. Level of evidence Level III. [ABSTRACT FROM AUTHOR]

Introduction Techniques of distraction enterogenesis have been explored to provide increased intestinal length to treat short bowel syndrome (SBS). Self-expanding, polycaprolactone (PCL) springs have been shown to lengthen bowel in small animal models. Their feasibility in larger animal models is a critical step before clinical use. Methods Juvenile mini-Yucatan pigs underwent jejunal isolation or blind ending Roux-en-y jejunojejunostomy with insertion of either a PCL spring or a sham PCL tube. Extrapolated from our spring characteristics in rodents, proportional increases in spring constant and size were made for porcine intestine. Results Jejunal segments with 7 mm springs with k between 9 and 15 N/m demonstrated significantly increased lengthening in isolated segment and Roux-en-y models. Complications were noted in only two animals, both using high spring constant k > 17 N/m. Histologically, lengthened segments in the isolated and Roux models demonstrated significantly increased muscularis thickness and crypt depth. Restoration of lengthened, isolated segments back into continuity was technically feasible after 6 weeks. Conclusion Self-expanding, endoluminal PCL springs, which exert up to 0.6 N force, safely achieve significant intestinal lengthening in a translatable, large-animal model. These spring characteristics may provide a scalable model for the treatment of SBS in children. [ABSTRACT FROM AUTHOR]