Short Bowel Syndrome Children Research Articles

Cutoffs and Characteristics of Abnormal Bowel Dilatation in Pediatric Short Bowel Syndrome.

Although excessive intestinal dilatation associates with worsened outcomes in pediatric short bowel syndrome (SBS), little is known about the natural history and definition of pathological dilatation. We addressed fore-, mid-, and hind-gut dilatation in children with SBS, who had not undergone autologous intestinal reconstructive (AIR) surgery, in relation to controls. SBS children without history of AIR surgery (n = 59) and age-matched controls without any disclosed intestinal pathology (n = 140) were included. Maximum diameter of duodenum, small bowel (SB), and colon were measured in each intestinal contrast series during 2002 to 2020 and expressed as diameter ratio (DR) to L5 vertebrae height. Predictive ability of DR for weaning off parenteral nutrition (PN) was analyzed with Cox proportional hazards regression models using multiple cutoffs. Duodenum (DDR), SB (SBDR), and colon (CDR) DR were 53%, 183%, and 23% higher in SBS patients compared to controls ( P < 0.01 for all). The maximal DDR and SBDR measured during follow-up is associated with current PN dependence and young age. DDR correlated with SBDR ( r = 0.586, P < 0.01). Patients with maximal DDR less than 1.5, which was also the 99th percentile for controls, were 2.5-fold more likely to wean off PN ( P = 0.005), whereas SBDR and CDR were not predictive for PN duration. All segments of remaining bowel, especially SB, dilate above normal levels in children with SBS. In SBS children without AIR surgery, PN dependence and young age is associated with duodenal and small intestinal dilatation, while duodenal dilatation also predicted prolonged PN.

The role of gastrostomy feeding during intestinal rehabilitation for children with short bowel syndrome.

Following extensive bowel resection, many children with short bowel syndrome (SBS) are routinely offered a placement of gastrostomy tube (G-tube) for feeding. This nutritional pathway is aimed to accommodate the gastric and small bowel motor disturbances related to SBS, and to promote weaning off parenteral nutrition (PN) to achieve enteral autonomy (EA). The aim of this study was to investigate the effect of gastrostomy feeding in outcomes of children with SBS. A retrospective cohort of all SBS children managed at our multidisciplinary Intestinal Rehabilitation Center as part of an Intestinal Rehabilitation Program. SBS was defined as PN dependence for more than six weeks following extensive bowel resection. Patients treated with G-tube feeding were compared with patients without G-tube in terms of PN duration, reaching EA, physical development, and surgical parameters. A total of 36 SBS patients diagnosed between 2003 and 2022 were included. The most common etiologies included congenital intestinal atresia (31%) and necrotizing enterocolitis (25%). SBS-G-tube (group A) contained 20 children, and SBS (group B) contained 16 children. A total of 21 children reached EA (58%); ten from group A (50%), and 11 from group B (69%) (p > 0.05). Within EA patients, mean PN duration was 49 ± 44months in group A, and 24 ± 33months in group B (p > 0.05). Patients who reached EA had 22% longer residual small bowel when compared with PN-dependent patients (p = 0.003). However, the outcomes were adjusted for residual small and large bowel length and percentages, a residual ileocecal valve, and a colon in continuity with no differences between the groups. Two-thirds of children from group A reported G-tube related complications (mechanical, bleeding, or infections). We did not find differences in mean height and weight percentiles between the groups (p > 0.05). We did not find significant advantage of gastrostomy feeding in reaching EA. Because there are surgical and mechanical complications related to this procedure, further prospective studies are required to determine G-tube relevance for children with SBS.

Successful experience in the use of teduglutide in children with short bowel syndrome

The article presents an overview of current trends in the approach to the treatment of patients with short bowel syndrome (SBS), including the example of pediatric patients in the Tyumen region. SBC is an extremely rare nosological form in the practice of a clinician and, as a rule, patients with such a diagnosis require a multidisciplinary approach. The most common manifestations of the disease are symptoms of malabsorption: dumping syndrome, progressive weight loss, deficiency of macro- and micronutrients, other manifestations of dyspepsia and abdominal pain. Attention is drawn to the fact that the number and intensity of symptoms varies significantly among patients. The traditional tactic for treating such patients includes the need for lifelong parenteral nutrition or, as a necessary measure, intestinal transplantation. Rehabilitation measures in SBS children should take into account the requirements for correcting impaired absorption and restoring normal trophic status; relief of diarrhea, prevention and treatment of dysbiosis; control and correction of general digestive disorders. We found that the analogue of glucagon-like peptide 2 (GLP-2) - teduglutide - makes it possible to reduce the amount of parenteral nutrition, infusion time, and also achieve a complete transition to enteral autonomy. The method of treatment with Teduglutide is the most promising for patients of this category.

P-29: Rapid Progression of Parenteral Nutrition-associated Liver Disease on Liver Biopsies of Pediatric Patients with Short-gut Syndrome: a Case Series

Introduction: The rate of parenteral nutrition-associated liver disease (PNALD) development in short-bowel syndrome (SBS) children on total parenteral nutrition (TPN) is unknown. We present the first case series of pediatric SBS patients on TPN with rapid progression of their PNALD on liver biopsies (LB) obtained <12 months (m) after TPN initiation. Methods: Charts of 81 children with a history of TPN use at a single institution between 2009-2019 were reviewed for inclusion criteria: 1) SBS diagnosis 2) LB obtained <12 m after TPN initiation and 3) No underlying liver disease. Clinical history, nutritional support, and liver function markers (direct bilirubin [Dbili], aspartate aminotransferase [AST], alanine aminotransferase, international normalized ratio, AST to Platelets Ratio Index, ultrasound elastography and LB) were collected via retrospective chart review approved by our institutional review board. Liver pathology was graded by one pathologist. Results: 10 patients fulfilled criteria; the mean duration on TPN prior to LB was 4m (range 1-10.8m) with half on Intralipid, and the other half on an alternative lipid emulsion. Seventy percent also received enteral feeds. Forty percent patients had elevated Dbili. All LBs showed moderate to marked bile ductular reaction associated with a mixed, portal-predominant inflammatory infiltrate of lymphocytes, neutrophils, and occasional eosinophils of mild to moderate severity. Inflammatory infiltration of interlobular bile ducts was a rare finding in a subset of cases; plasma cells were not a prominent feature. Most samples showed some degree of cholestasis, usually canalicular. Lobular inflammation and hepatocyte necrosis were typically mild and nonzonal/spotty though some showed diffuse ballooning hepatocellular injury. No significant steatosis was observed. Fibrosis on trichrome stain was variable, typically portal-predominant; few cases demonstrated pericellular fibrosis. Based on the LB pathology, 70% had a change in medical care such as changing the lipid emulsion. Conclusion: The progression of PNALD in SBS children on LBs <12 months from TPN initiation was more rapid than previously hypothesized. LBs taken at the time of another surgical procedure may be warranted for SBS pediatric patients on TPN.

Glucocorticoids Improve Enteral Feeding Tolerance in Pediatric Short Bowel Syndrome With Chronic Intestinal Inflammation.

A group of short bowel syndrome (SBS) patients developed chronic intestinal inflammation while struggling weaning off parenteral nutrition (PN). They did not respond to standard management of SBS and food allergy. We treated them with glucocorticoids and described the outcome. Our study is a retrospective descriptive study. We reviewed records from the intestinal rehabilitation program from 2006 to 2017. We identified 15 patients whose lab values, pathology results, and clinic notes were reviewed. We had more patients (n = 10) with diagnosis of gastroschisis, and more female patients (n = 9). Seven patients weaned off PN with median treatment duration of 5 months, 5 of which remained on budesonide for significant period of time (median: 7.5 months). One of these 7 patients relapsed, as the patient resumed glucocorticoids because of recurrence of chronic intestinal inflammation. Six of 15 children had significant eosinophils in their initial biopsy, 5 of these children weaned off PN whereas 1 child's gastrointestinal (GI) bleeding stopped. Four patients were not able to decrease PN calorie. Two of these patients' GI bleeding stopped, the other 2 had normalized histology. For SBS children with histologically confirmed chronic intestinal inflammation, glucocorticoids may help promote enteral feeding tolerance. Glucocorticoids regimen should be chosen individually. Patients are more likely to respond if initial histology has significant eosinophilic infiltration. Patients may need to remain on glucocorticoids for over 6 months.

Compromised duodenal mucosal integrity in children with short bowel syndrome after adaptation to enteral autonomy

BackgroundIntestinal adaptation has been extensively studied experimentally, but very limited data is available on human subjects. In this study we assessed intestinal adaption in humans with short bowel syndrome (SBS). MethodsWe comparatively evaluated mucosal hyperplasia, inflammation, barrier function and nutrient transport using histology, immunohistochemistry and qPCR for selected 52 key genes in duodenal biopsies obtained from children with SBS after weaning off parenteral nutrition (n = 33), and matched controls without intestinal pathology (n = 12). Small bowel dilatation was assessed from contrast small bowel series. ResultsDuodenal mucosa of SBS children showed increased histologic inflammation of lamina propria (p = 0.033) and mucosal mRNA expression of tumor necrosis factor (p = 0.027), transforming growth factor (TGF)-β2 (p = 0.006) and caveolin-1 (CAV1; p = 0.001). Villus height, crypt depth, enterocyte proliferation, apoptosis and expression of proliferation and nutrient transport genes remained unchanged. Pathologic small bowel dilatation reduced crypt depth (p = 0.045) and downregulated mRNA expression of interleukin (IL)-6 by three-fold (p = 0.008), while correlating negatively with IL6 (r = -0.609, p = 0.004). Loss of ileocecal valve (ICV) upregulated mRNA expression of toll-like receptor 4 (TLR4), TGF-β1, CAV1, several apoptosis regulating genes, and mRNA expression of zonulin (p < 0.05 for all). ConclusionsDespite successful adaptation to enteral autonomy, duodenal mucosa of SBS children displayed histologic and molecular signs of abnormal inflammation and regulation of epithelial permeability, whereas no structural or molecular signs of adaptive hyperplasia or enhanced nutrient transport were observed. Excessive dilatation of the remaining small bowel paralleled impaired duodenal crypt homeostasis, while absence of ICV modified regulation of mucosal inflammation, regeneration and permeability. Level of evidenceII

Microbial alteration of small bowel stoma effluents and colonic feces in infants with short bowel syndrome

Background and aimStudies 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. MethodsSeven 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. ResultsThe 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. ConclusionsThe 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. Level of evidenceIII.

P3B.12: Spiral intestinal lengthening and tailoring (SILT) as a rescue procedure for SBS patients with difficult clinical situations.

Introduction: Autologous gastrointestinal reconstructive (AGIR) procedures are essential component of intestinal rehabilitation program for patient with short bowel syndrome (SBS). Unfortunately, several patients develop postoperative bowel re-dilatation with loss of bowel adaptation. Spiral intestinal lengthening and tailoring (SILT) technique promises to regain intestinal physiology by tailoring the bowel even if there is no massive bowel dilatation. The aim of this study was to report our experience using SILT in SBS with difficult clinical situation. Methods: Retrospective review of the single AGIR surgeon was performed from 2012 to date. Patients’ demographics, pre- and post-procedure bowel length, surgical complications, and postoperative parenteral nutrition (PN) requirements were analyzed. Data were compared using independent samples, Mann-Whitney’s U-test. Data were expressed in mean (IQR) Results: 9 children with SBS underwent SILT between 2012 and 2017. Rescue SILT was performed in 4 patients with age at procedure of 58.5 months (45–143.3). 3 patients had previous STEP in history of SBS post gastroschisis while one had mild dilatation after AGIR due to long segment Hirschsprung. Preoperative small bowel length measured 66 cm (49.5–75.7) with a diameter of 7 cm (6–8). SILT allowed a median increase in length of 68.5% (p = 0.34) and a significant tailoring of the dilated segment providing a reduction in diameter of 63% (p = 0.02). No major complications related to SILT were encountered and none of the children required further surgical intervention following a follow-up of 6.5 months (4.5–39.6). Interestingly, a significant reduction of PN requirement at 4 months (p = 0.02) associated with improve of liver function was reported. Conclusion: In this study SILT was reported to have no peri-operative complication and to have good results in complex SBS patients. This procedure offers a safe alternative for SBS children after the failure of conventional AGIR procedures.

P3B.14: Glucocorticoid treatment improves enteral feeding tolerance in pediatric short bowel syndrome patients with chronic intestinal inflammatory changes

Introduction: Studies suggest that chronic local and systemic inflammation are related to short bowel syndrome (SBS). We believe that glucocorticoids may reduce inflammation and improve enteral tolerance in pediatric SBS patients. A group of patients from our intestinal rehabilitation program developed chronic intestinal inflammation under biopsy while struggling weaning off parenteral nutrition (PN). We treated them with oral prednisolone taper, maintenance budesonide and sulfasalazine. We will describe the treatment outcome. Method: Our study included 15 patients whose lab values, pathology results and clinic notes were reviewed, and excluded patients who took steroids for post-transplant immunosuppression or severe food allergy to maintain the homogenous of our patient cohort. Results: The most common reason for SBS was gastroschisis. The small bowel length range was 20–108 cm. Thirteen patients had more than ½ colon remaining. The median age of starting steroid therapy was 3.3 years. Twelve patients had decreased PN calorie count or normalized follow-up histology. Six patients were able to wean off PN completely with median treatment duration of 5 months, five of which remained on maintenance budesonide for significant period of time (median: 7.5 months), the other one had steroids stopped 5 months before being off PN due to clinical improvement and concern for medication noncompliance. Six out of fifteen children had significant eosinophils in their initial biopsy, five were able to wean off PN while one had significant symptom improvement (GI bleeding stopped). Two patients were not on steroids continuously, as they resumed steroids months later due to histological recurrence of the chronic inflammatory process. Three patients were still not able to be cut down PN calorie. Two patients’ intestinal biopsies findings were still consistent with chronic inflammatory process. Conclusion: For SBS children with histologically confirmed chronic intestinal inflammatory changes, steroid therapy may help to promote enteral feeding tolerance and wean off PN. Patients may respond better if their initial histology has chronic intestinal inflammation with significant eosinophilic infiltrate. Patients may need to remain on maintenance steroids for a period of time even if they were able to wean off PN or significantly reduce PN calories as they may experience regression on enteral tolerance or evidence of GI bleeding as weaning off steroids is attempted.

250.5: Early Predictors of Enteral Autonomy in Pediatric Intestinal Failure:Development of a Disease Severity Score

Introduction: Patients with short bowel syndrome (SBS) are dependent on parenteral nutrition (PN) while their bowel attempts to compensate for loss of function. Our objective was to create a SBS disease severity score that would predict the probability of achieving enteral autonomy (EA) using clinical variables available in the early postoperative period. Methods: A retrospective cohort study of SBS children managed by our Intestinal Rehabilitation Program (IRP) was completed. Data abstracted included demographic, anatomic and outcome variables including serum conjugated bilirubin, proportion of enteral nutrition (EN) and episodes of sepsis specifically at 6 months post gut loss. A univariate analysis and Cox proportional hazards (CPH) model was performed. A score predicting EA was created based on weighting of Cox model coefficients. For all analyses, an alpha-value of <0.05 was considered significant. Results: 139 patients were analyzed (61% males). Ninety-five (68%) achieved EA. Those who achieved EA had a longer residual small bowel (75% vs 24%; p<0.0001) and colon (100% vs 75%; p<0.0001) and were less likely to have the ileocecal valve removed (26% vs 57%; p=0.0005). At 6 months, children who achieved EA had higher enteral tolerance (100% vs 30%; p<0.0001), a lower conjugated bilirubin (0 vs 71.5umol/L; p<0.0001) and less septic episodes (1.0 vs 2.0; p=0.0112). Cox proportional hazards modeling found >50% residual small bowel (HR 2.68 [95%CI 1.60–4.49], p<0.001), ICV intact (HR 0.61 [95%CI 0.37–1.02], p<0.06) and >50% enteral tolerance at 6 months (HR 5.70 [95% CI 2.77–11.74] p<0.001) were positively associated with EA. Conjugated bilirubin >34umol/L at 6 months was negatively associated with EA (HR 0.42 [95%CI 0.27–0.66], p<0.001). A severity score was created by weighting CPH parameter estimates [small bowel length >50%, ICV intact, CB<34umol/L and EN>50% for a maximum score of 8. Disease severity strata were developed (severe [0–2; 25.7% EA], moderate [3–5; 52.9% EA] and mild [6–8; 97.1% EA]. Disease severity strata were developed (severe [0–2; 9/35 (25.7%) EA], moderate [3–5; 18/34 (52.9%) EA and mild [6–8; 68/70 (97.1%) EA]. Conclusion: We propose a paediatric intestinal failure disease severity score that predicts probability of EA, stratified into mild, moderate and severe. The score allows prognostication of individual patients, and could assist research by adjusting outcome reporting or stratifying recruitment.

Short bowel mucosal morphology, proliferation and inflammation at first and repeat STEP procedures

BackgroundAlthough serial transverse enteroplasty (STEP) improves function of dilated short bowel, a significant proportion of patients require repeat surgery. To address underlying reasons for unsuccessful STEP, we compared small intestinal mucosal characteristics between initial and repeat STEP procedures in children with short bowel syndrome (SBS). MethodsFifteen SBS children, who underwent 13 first and 7 repeat STEP procedures with full thickness small bowel samples at median age 1.5 years (IQR 0.7–3.7) were included. The specimens were analyzed histologically for mucosal morphology, inflammation and muscular thickness. Mucosal proliferation and apoptosis was analyzed with MIB1 and Tunel immunohistochemistry. ResultsMedian small bowel length increased 42% by initial STEP and 13% by repeat STEP (p=0.05), while enteral caloric intake increased from 6% to 36% (p=0.07) during 14 (12-42) months between the procedures. Abnormal mucosal inflammation was frequently observed both at initial (69%) and additional STEP (86%, p=0.52) surgery. Villus height, crypt depth, enterocyte proliferation and apoptosis as well as muscular thickness were comparable at first and repeat STEP (p>0.05 for all). Patients, who required repeat STEP tended to be younger (p=0.057) with less apoptotic crypt cells (p=0.031) at first STEP. Absence of ileocecal valve associated with increased intraepithelial leukocyte count and reduced crypt cell proliferation index (p<0.05 for both). ConclusionsNo adaptive mucosal hyperplasia or muscular alterations occurred between first and repeat STEP. Persistent inflammation and lacking mucosal growth may contribute to continuing bowel dysfunction in SBS children, who require repeat STEP procedure, especially after removal of the ileocecal valve. Level of evidenceLevel IV, retrospective study.

Impact of Short Bowel Syndrome on Quality of Life and Family: The Patient's Perspective.

Short bowel syndrome (SBS) has an impact on children and their families not only physically, but also emotionally, mentally, and socially. This study aimed to evaluate quality of life and family impact in patients with SBS, using the Pediatric Quality of Life Inventory (PedsQL) measurement model. PedsQL questionnaires were administered to patients with SBS followed at the Pediatric Surgery of Royal Manchester Children's Hospital. The scores were compared between two groups differing in age (children <5 y vs. >5 y) and with known-groups from literature published by Varni et al. RESULTS: Forty-three patients were sent the questionnaires, and 30 (70%, 17 < 5 and 13 > 5) responded. Family Impact Module failed to distinguish between families of children younger and older than 5 years of age. In contrast, it distinguished between families with SBS children and the ones with children in a long-term care facility. Healthcare satisfaction was worse in families of children over 5 years regarding information received, inclusion of the family, and communication. When compared with normal population, SBS children scored worse on Generic Core Scales for all domains except for emotional functioning. Although advances have been made on the treatment of children with SBS, and improvements have been reached on home PN, this condition is still significantly affecting the quality of life of children and their families. The future quality control of medical care must have greater focus on psychosocial and emotional functioning, aiming for the best possible quality of life.

D-lactic acidosis in children with short bowel syndrome who underwent autologous bowel reconstruction: A retrospective study

Introduction: d-lactic acidosis (DLA) is an uncommon complication in children with Short Bowel Syndrome (SBS) with intestinal dilation. This condition is related to excess of production of d-lactate by intestinal bacteria when carbohydrates (CHO) are not completely absorbed in the small bowel. Our aim is to evaluate the DLA incidence in SBS children referred to our unit.

Small Bowel Dilatation Predicts Prolonged Parenteral Nutrition and Decreased Survival in Pediatric Short Bowel Syndrome.

To analyze risk factors and prognostic significance of small bowel (SB) dilatation in children with short bowel syndrome (SBS). In SBS, the remaining SB may dilate as part of intestinal adaptation. The impact of dilatation on parenteral nutrition (PN) dependence and survival has not been studied systematically. SB diameter of SBS children (n = 61) was measured in contrast SB series (n = 169, median age 0.94, range 0.32-2.7 years) during 2002 to 2015, and expressed as millimeters (SB width) and as ratio to L5 vertebra height (SB diameter ratio). Linear regression was used to examine risk factors for dilatation. PN weaning and survival were analyzed with Cox proportional hazards regression. Maximal SB diameter ratio during follow-up was predicted by PN dependence and SB atresia, while maximal absolute SB width by birth weight, age, PN duration, and remaining bowel length. Weaning off PN was 14-fold more likely in patients with maximal SB diameter ratio <2.00 compared with >3.00 (P = 0.005), and 5.4-fold more likely when maximal SB width was <20 mm compared with >30 mm (P = 0.023). After adjustment for age, remaining SB length, and the presence of ileocecal valve, both estimates of maximal SB dilatation remained significant independent predictors for weaning off PN. When all measurements were included, the cumulative survival was worse if SB diameter ratio exceeded 2.00 (P = 0.002-0.042). SB dilatation predicts prolonged PN duration and decreased survival in SBS children. Measurement of maximal SB diameter standardized to L5 vertebra height may be a valuable objective tool for patient follow-up and assessment of prognosis.

Small bowel dilation in children with short bowel syndrome is associated with mucosal damage, bowel-derived bloodstream infections, and hepatic injury

Liver disease occurs frequently in short bowel syndrome. Whether small bowel dilation in short bowel syndrome could influence the risk of liver injury through increased bacterial translocation remains unknown. Our aim was to analyze associations between small bowel dilation, mucosal damage, bloodstream infections, and liver injury in short bowel syndrome patients. Among short bowel syndrome children (n = 50), maximal small bowel diameter was measured in contrast series and expressed as the ratio to the height of the fifth lumbar vertebra (small bowel diameter ratio), and correlated retrospectively to fecal calprotectin and plasma citrulline-respective markers of mucosal inflammation and mass-bloodstream infections, liver biochemistry, and liver histology. Patients with pathologic small bowel diameter ratio >2.17 had increased fecal calprotectin and decreased citrulline (P<.04 each). Of 33 bloodstream infections observed during treatment with parenteral nutrition, 16 were caused by intestinal bacteria, cultured 15 times more frequently when small bowel diameter ratio was >2.17 (P<.001). Intestinal bloodstream infections were predicted by small bowel diameter ratio (odds ratio 1.88, P=.017), and their frequency decreased after operative tapering procedures (P=.041). Plasma bilirubin concentration, gamma-glutamyl transferase activity, and histologic grade of cholestasis correlated with small bowel diameter ratio (0.356-0.534, P<.014 each), and were greater in the presence of intestinal bloodstream infections (P<.001 for all). Bloodstream infections associated with portal inflammation, cholestasis, and fibrosis grades (P<.031 for each). In linear regression, histologic cholestasis was predicted by intestinal bloodstream infections, small bowel diameter ratio, and parenteral nutrition (β=0.36-1.29; P<.014 each), while portal inflammation by intestinal bloodstream infections only (β=0.62; P=.033). In children with short bowel syndrome, small bowel dilation correlates with mucosal damage, bloodstream infections of intestinal origin, and cholestatic liver injury. In addition to parenteral nutrition, small bowel dilation and intestinal bloodstream infections contribute to development of short bowel syndrome-associated liver disease.

Anastomotic ulcers in short bowel syndrome: New suggestions from a multidisciplinary approach

Background and aimsAnastomotic ulceration (AU) is a rare potential life-threatening complication that may occur after intestinal resection. The diagnosis is often delayed after a long-lasting history of refractory anemia. The pathogenesis remains unknown and there are no established therapies. The aim of the study was to analyze the medical history of children with short bowel syndrome (SBS) who were experiencing AU. MethodsRecords of SBS children were retrospectively reviewed. Demographics, baseline characteristics, presentation, diagnosis and treatment of AU cases were analyzed. ResultsEight out of 114 children with SBS were identified as having AU. Mean gestational age was 32.5weeks. Underlying diseases were: 5 necrotising enterocolitis, 2 gastroschisis and 1 multiple intestinal atresia. The mean age at AU diagnosis was 6.5years (diagnosis delay of 35months). All but 2 patients had AU persistency after medical treatment. Endoscopic treatment (2 argon plasma coagulation; 1 platelet-rich fibrin instillation; 2 endoscopic hydrostatic dilations) was effective in 3 out of 5 children. Surgery was required in 3 patients. ConclusionsSevere bowel ischemic injury, especially in preterm infant, could predispose to AU development. Medical treatment showed discouraging results. We firstly described that different endoscopic treatment could be attempted before resorting to further surgery.Level of Evidence: IV.

Small Bowel Dilatation in Pediatric Short Bowel Syndrome Associates with Mucosal Damage, Bowel-Derived Bloodstream Infections, and Liver Injury.

Introduction: Adaptive small bowel (SB) dilatation is common in short bowel syndrome (SBS). Whether SB dilatation predisposes to bloodstream infections (BSI) or associates with mucosal damage or liver injury remains unknown. Methods: Among SBS children (n=50), maximal SB diameter was measured in contrast series (n=179), expressed as ratio to fifth lumbar vertebra height (SB diameter ratio), and related to fecal calprotectin and plasma citrulline; markers of mucosal inflammation and mass, BSIs, liver biochemistry, and liver histology. Results: Median length of remaining SB was 38 (interquartile range 25–60) cm, age at contrast series 1.04 (0.34-3.01) years, and SB diameter ratio 2.14 (1.65-2.71). Patients with pathological SB diameter ratio >2.17 predicting prolonged PN-dependence had increased fecal calprotectin (86 vs. 22, p=0.012), plasma bilirubin (8 vs. 6, p=0.033), gamma-glutamyl transferase (52 vs. 20, p<0.001), and aminotransferase concentrations (45 vs. 30; 59 vs. 42, p<0.001-0.019) while decreased plasma citrulline (15 vs. 26, p=0.033) compared to others. All BSIs occurred during parenteral nutrition (PN) and 48% (16/33) of them were caused by intestinal bacteria, cultured 15 times more frequently when SB diameter ratio exceeded 2.17 (Figure 1). In logistic regression, SB diameter ratio was the only predictor of intestinal BSIs (odds ratio 1.88, p=0.017). Histological cholestasis grade was higher when SB diameter ratio exceeded 2.17 vs. ≤2.17 (1.15 vs. 0.21, p<0.001), while cholestasis, portal inflammation, and fibrosis grades were higher in the presence of intestinal BSIs vs. no BSIs (Figure 2). In linear regression, histological cholestasis was predicted by intestinal BSIs, SB diameter ratio, and PN (β=0.36-1.29, p<0.001-0.014), while portal inflammation by intestinal BSIs only (β=0.61, p=0.021). Conclusion: In pediatric SBS, SB dilatation relates with mucosal damage, BSIs of intestinal origin, and cholestatic liver injury, while BSIs of intestinal origin predict both histological cholestasis and portal inflammation. Results suggest the damaged mucosa of excessively dilated SB predisposes to bacterial translocation, which contributes to liver disease development.FIGURE 1: The occurrence of BSIs of intestinal origin according to small bowel (SB) diameter ratio within 6 months of contrast series (n=179).FIGURE 2: Grading of portal inflammation, cholestasis, and fibrosis (Metavir) according to the presence of BSIs of intestinal origin within 6 months of liver biopsies (n=50).

Short Bowel Syndrome

Objectives and Study: Intestinal failure (IF) is a state of severe intestinal malabsorption requiring parenteral nutrition (PN) for survival. The leading cause of IF is short bowel syndrome (SBS). This study aims to show whether the presence of the colon makes a difference in children with SBS, and to find correlations between intestinal absorption rate, citrulline level, anatomy of the remnant bowel and parenteral nutrition dependency. Methods: Twenty-three SBS children were included in this study and divided into two groups according to the presence or absence of the colon. Bowel length, citrulline plasma levels, intestinal absorption rate [by using three days stool balance analysis] and PN dependency index [by using the index Non Protein Energy Intake (NPEI)/Resting Energy Expenditure (REE) by Schofield formula], were assessed. Regression analysis and student t tests comparing variables were performed. Results: Patients with colon (n=14) had significantly shorter remnant small bowel as compared with those without colon (n=9) (29.25 ± 25.64 vs. 60.88 ± 22.54 cm; P < 0.003) and lower fecal sodium losses (48.5 ± 28.07 vs. 85.88 ± 18.98 mmol/l; P < 0.002). No difference was found between the groups for the percentage energy absorption (63.4 ± 19.0 vs 66.4 ± 15.4% respectively), while citrulline plasma levels showed only a tendency to be higher in SBS without colon (19.1 ± 12.17 vs. 14.4 ± 9.72 μmol/l; P = 0.17). PN dependency was not significantly different between neither groups, nor the total energy intake from oral or enteral nutrition. No correlation was found between the different parameters, although the citrulline level correlates with the small bowel length in the group with colon (r2 = 0.57). Conclusion: By using three days’ stool balance analysis, this study demonstrates for the first time in SBS children, the role of the colon in energy salvage in addition to water- electrolytes absorption. It emphasizes the importance of early restoration of intestinal continuity and preservation of intestinal microbiota.

Severe Gut Microbiota Dysbiosis Is Associated With Poor Growth in Patients With Short Bowel Syndrome.

Children with short bowel syndrome (SBS) can vary significantly in their growth trajectory. Recent data have shown that children with SBS possess a unique gut microbiota signature compared with healthy controls. We hypothesized that children with SBS and poor growth would exhibit more severe gut microbiota dysbiosis compared with those with SBS who are growing adequately, despite similar intestinal anatomy. Stool samples were collected from children with SBS (n = 8) and healthy controls (n = 3) over 3 months. Gut microbiota populations (16S ribosomal RNA sequencing and metagenomic shotgun sequencing) were compared, including a more in-depth analysis of SBS children exhibiting poor and good growth. Statistical analysis was performed using Mann-Whitney, Kruskal-Wallis, and χ2 tests as appropriate. Children with SBS had a significant deficiency of the commensal Firmicutes order Clostridiales ( P = .025, Kruskal-Wallis) compared with healthy children. Furthermore, children with SBS and poor growth were deficient in beneficial bacteria known to produce short-chain fatty acids and had expansion of proinflammatory Enterobacteriaceae ( P = .038, Kruskal-Wallis) compared with children with SBS who were growing adequately. Using metabolic function analyses, SBS/poor growth microbiomes were deficient in genes needed for gluconeogenesis but enriched in branched and aromatic amino acid synthesis and citrate cycle pathway genes. Patients with SBS, particularly those with suboptimal growth, have a marked gut dysbiosis characterized by a paucity of beneficial commensal anaerobes, resulting in a deficiency of key metabolic enzymes found in the gut microbiomes of healthy children.

Outcome of home parenteral nutrition in 251 children over a 14-y period: report of a single center

Parenteral nutrition (PN) is the main treatment for intestinal failure. We aimed to review the indications for home parenteral nutrition (HPN) in children and describe the outcome over a 14-y period from a single center. We conducted a retrospective study that included all children who were referred to our institution and discharged while receiving HPN between 1 January 2000 and 31 December 2013. The indications for HPN were divided into primary digestive diseases (PDDs) and primary nondigestive diseases (PNDDs). We compared our results to a previous study that was performed in our unit from 1980 to 2000 and included 302 patients. A total of 251 patients were included: 217 (86%) had a PDD. The mean ± SD age at HPN onset was 0.7 ± 0.3 y, with a mean duration of 1.9 ± 0.4 y. The indications for HPN were short bowel syndrome (SBS) (59%), PNDD (14%), congenital enteropathies (10%), chronic intestinal pseudo-obstruction syndromes (9%), inflammatory bowel diseases (5%), and other digestive diseases (3%). By 31 December 2013, 52% of children were weaned off of HPN, 9% of the PDD subgroup had intestinal transplantation, and 10% died mostly because of immune deficiency. The major complications of HPN were catheter-related bloodstream infections (CRBSIs) (1.7/1000 d of PN) and intestinal failure-associated liver disease (IFALD) (51 children; 20% of cohort). An increased rate of CRBSIs was observed compared with our previous study, but we saw a decreasing trend since 2012. No noteworthy deceleration of growth was observed in SBS children 6 mo after weaning off HPN. SBS was the major indication for HPN in our cohort. IFALD and CRBSIs were potentially life-threatening problems. Nevertheless, complication rates were low, and deaths resulted mostly from the underlying disease.