Bowel Length Research Articles

Combined procedures for surgical short bowel syndrome: experience from two European centres.

Autologous gastrointestinal reconstructive surgery (AGIR) has become a key component of intestinal rehabilitation programs. However, the best surgical option for short bowel syndrome (SBS) remains unknown. This paper presents our experience using combined procedures as primary treatment. We collected data on SBS patients who underwent surgery from 2008 to 2021 in two tertiary European Centres. Combined procedures were defined as more than one technique used on the same patient. Charts were reviewed for demographics, type of procedures, complications, and outcomes. Data are presented as median and IQR. Wilcoxon signed rank was used for all paired analyses. Twenty-one children (12 females) underwent combined procedures. Preoperative median small bowel length was 20 cm (IQR: 15-35 cm); after lengthening, it was 35.5cm (IQR: 30.75-50.50 cm) (P< 0.001). Combined procedures were simultaneous in 15 patients and sequential in 6. At a median of 9.2 years (IQR: 7.55-9.78 years) follow-up, complications were three bowel obstructions after strictures of anastomosis and two wound infections. Two patients achieved enteral autonomy, and others followed a weaning home parenteral nutrition regimen with a median of 4 nights off (IQR: 3-4 nights) starting with a median of 7 nights (IQR: 7-7 nights). Combined AGIR techniques are practical and safe in SBS treatment when tailored to meet patients' needs, combining lengthening, tailoring, and reducing transit time procedures. Therefore, combined AGIR may be considered a resource in intestinal rehabilitation units' armamentarium.

BS P05 Laparoscopic repair of internal hernia post Roux-en-Y Gastric Bypass: Our tricks and technique

Abstract Background The incidence of internal herniation (IH) post Roux-en-Y gastric bypass (RYGB) is variable across literature (0.5–11%). This may be partially related to the difference in the operative techniques of RYGB. Routine closure of mesenteric and Petersen's defects has shown noticeable decline in the incidence of IH over last few years. Nevertheless, it didn't prevent the development of IH at long-term follow up. Methods We describe a video of a case with a large non-obstructing mesenteric internal hernia post RYGB. A 33 years old female patient presented to our bariatric clinic with recurrent attacks of post-prandial abdominal pain over 6 months. She had RYGB 2.5 years before this presentation, with EWL of 65%. Of note, the mesenteric and Petersen's defects were closed in the original RYGB operation. CT scan showed suspicious findings of internal herniation without obstructing signs. Patient had a laparoscopic exploration which showed a large internal herniation through mesenteric defect containing more than two thirds of bowel length. Closure of the defect was done by a purse string technique using non-absorbable prolene 2/0 stitch after reduction of the bowel. Patient had an uneventful postoperative recovery. She denied any further attacks of abdominal pain at her follow up till 6 months postoperatively. Conclusions Diagnosis of non-obstructing internal herniation post RYGB is very challenging. Low threshold for laparoscopic exploration in suspicious cases of remittent abdominal pain is highly recommended. CT scan can't rule out IH completely due to its intermittent nature. Laparoscopic repair remains the optimal approach, even in the large IH, with favourable outcomes. Appropriate identification of the bowel configuration is the key step in such procedure.

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.

Mortality and parenteral nutrition weaning in patients with chronic intestinal failure on home parenteral nutrition: A 30-year retrospective cohort study

BackgroundHome parenteral nutrition (HPN) is the standard treatment for patients with chronic intestinal failure (CIF). Mortality and weaning rates of these patients differ widely among cohorts; however, these outcomes were often considered independent—rather than competing—events, leading to an upward bias of the retrieved estimates. ObjectivesThe aim of this retrospective cohort study was to evaluate, evaluating through a competing risk analysis, the rates and predictors of mortality and weaning in CIF patients from an Italian referral center. MethodsAll adult patients with CIF receiving > 3 mo HPN from 1985 until 2016 were enrolled. Clinical information was collected from the database of the Intestinal Failure Unit of Torino, Italy. Patients were stratified according to the presence or not of short bowel syndrome (SBS). ResultsThe cumulative incidences of death and weaning were 27.3% and 32.3% and 39.0% and 33.7% at 5 and 10 y from HPN initiation, respectively. At multivariable competing risk analyses, mortality was predicted by age (sub-distribution hazard ratio [SHR] = 1.65 per 10-y increase; 95% CI, 1.35–2.01), type 3 SBS (SHR = 0.38; 0.15–0.94), small bowel length ≥ 100 cm (SHR = 0.42; 0.22–0.83), and reconstructive surgery (SHR = 0.11; 0.02–0.64) in SBS patients, and by age (SHR = 1.38 per 10-y increase; 1.16–1.64) and presence of stoma (SHR = 0.30; 0.12–0.78) in non-SBS patients. In the same model, weaning was predicted by type 3 SBS (SHR = 6.86; 3.10–15.16), small bowel length ≥ 100 cm (SHR = 3.54; 1.99–6.30), and reconstructive surgery (SHR = 2.86; 1.44–5.71) in SBS patients, and by age (SHR = 0.79 per 10-y increase; 0.66–0.94) and presence of stoma (SHR = 2.64; 1.38–5.07) in non-SBS patients. ConclusionsSurgical procedures strongly affected mortality and weaning risk in CIF patients.

How accurate is the visual estimation of bowel length by endoscopic surgeons?

Measurement of small bowel length is an essential step in performing bariatric surgery. Surgeons need to measure bowel length in order to create alimentary and biliopancreatic limbs. Inaccurate bowel measurement may affect the outcome of surgery. However, it is not clear how accurate the measurement of bowel length is by surgeons. Two image quizzes marking certain lengths of jejunum were sent to participants. They were asked to estimate the length of marked bowels in maze quizzes. The Error of estimation, prevalence of significant error (error greater than 30 percent of actual length), and the relationship between different participant characteristics was investigated. A total of 86 participants answered the questionnaire. The mean error of estimation was 4.62 cm (27%). Twenty-eight participants (33%) had significant errors in estimation of bowel length. While there are surgeons that can estimate bowel length with decent accuracy, significant errors in estimation of bowel length are not uncommon among surgeons. Surgeons should consider adopting techniques for accurate measurement of the small intestine.

Roux-en-Y gastric bypass with a long versus a short biliopancreatic limb improves weight loss and glycemic control in obese mice.

Roux-en-Y gastric bypass (RYGB) results in long-term weight loss and reduced obesity related co-morbidities. However, little is known about how the lengths of the biliopancreatic limb (BPL), the alimentary limb (AL), and the common limb (CL) affect weight loss and glucose metabolism. Our aim was to establish a RYGB obese mouse model with defined proportions of the AL and BPL and a constant CL to assess the effects on weight loss,glucose metabolism, and obesity-related co-morbidities. In vivo mouse study. Six-week-old male C57BL/6J mice fed with a high-fat diet (HFD) underwent bariatric surgery with defined BPL lengths: a very long, long, and short BPL (35%, 25%, and 15% of total bowel length), or sham surgery. The length of the AL was adjusted to achieve the same CL length. Mice were analyzed for weight loss, glycemic control, and obesity-related co-morbidities. Mice undergoing RYGB surgery with a very long BPL had excessive weight loss and mortality and were therefore not further analyzed. Mice with a long BPL showed a significantly increased total weight loss when compared with mice with a short BPL. In addition, a long BPL improved glucose tolerance, particularly early after surgery. A long BPL was also associated with lower triglyceride levels. Resolution of hepatic steatosis and adipose tissue inflammation was, however, not statistically significant. Of note, bariatric surgery dramatically changed gut microbiota, regardless of limb length. In obese mice, a long BPL results in enhanced weight loss and improved glucose tolerance. These findings could potentially be translated to humans by tailoring the BPL length according to body weight, obesity-related co-morbidities, and total bowel length of an individual patient.

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

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

Efficient achievement of enteral autonomy facilitates resolution of liver injury in necrotizing enterocolitis induced short bowel syndrome

Children with short bowel syndrome (SBS) are at high risk for intestinal failure associated liver disease (IFALD). The aim of this retrospective follow-up study was to compare weaning off parenteral nutrition (PN) and IFALD between necrotizing enterocolitis (NEC) and non-NEC induced SBS. Altogether, 77 patients with neonatal SBS treated by our multidisciplinary intestinal failure unit (NEC n = 38, non-NEC SBS n = 39) were included and followed-up at least for 2 years until median age of 10 years (interquartile range, 6.0–16). Occurrence and characteristics of IFALD was assessed with liver biopsies obtained at median age of 3.2 (1.0–6.7) years (n = 62) and serum liver biochemistry. Overall, NEC patients had less end-jejunostomies and autologous intestinal reconstruction surgeries performed compared to non-NEC patients (< 0.05), while remaining small bowel anatomy was comparable between groups. Cumulative weaning off PN was more frequent and duration of PN shorter among NEC patients (P < 0.05). Overall cumulative probability of histological IFALD was lower among NEC patients during whole follow-up period (P = 0.052) and at 10 years (P = 0.024). NEC patients had lower ALT and GGT levels at last follow-up (P < 0.05 for all). In univariate Cox regression analysis, absence of end-jejunostomy, NEC diagnosis, longer remaining small bowel length, multidisciplinary treatment and prematurity were predictive for weaning off PN, while NEC diagnosis and lower birth weight in addition to multidisciplinary care protected from histological IFALD. Neonates with NEC induced SBS reached enteral autonomy earlier than those with non-NEC SBS, which associated with more efficient resolution of histological IFALD among long-term survivors.

Association of neutropenia at disease onset with severe surgical necrotizing enterocolitis and higher mortality: A retrospective study.

BackgroundNeutrophils are among the earliest immune cells recruited to the site of an intestinal injury, but their predictive role in the progression of necrotizing enterocolitis (NEC) has not been fully elucidated. This study aimed to evaluate if a reduction in neutrophils at the onset of NEC is associated with severe surgical NEC and/or NEC-associated deaths.MethodsThis is a retrospective cohort study in which neonates underwent surgery due to NEC during 2015–2020. The data on absolute neutrophil count (ANC), before and at the onset of NEC, were collected from the complete blood count results. The primary exposure was the difference in absolute neutrophil count (ΔANC) at NEC onset. The primary outcome was severe surgical NEC, defined as the residual small bowel length after intestinal resection of <30 cm.ResultsA total of 157 neonates were included in this study, of which 53 were diagnosed with severe surgical NEC. A decrease in ANC at the onset of NEC was associated with an increased probability of severe surgical NEC (crude odds ratio [OR] 1.248, 95% CI 1.107–1.407; P = 0.000). ΔANC (area under the curve [AUC] 0.729, 95% CI 0.653–0.797; P < 0.001] was a good predictor for severe surgical NEC. The addition of platelets to ΔANC at NEC onset (AUC 0.738, 95% CI 0.662–0.808; P < 0.001) resulted in a higher AUC and specificity for severe surgical NEC prediction than ΔANC alone. A reduction in the neutrophil count at NEC onset (ΔANC > 0) was associated with adverse outcomes (hazard ratio [HR] 3.48, 95% CI 1.64–7.36) and a lower survival probability (χ2 10.63; P < 0.001).ConclusionA reduction in the ANC at the onset of NEC was associated with severe surgical NEC and higher mortality. The addition of platelets to ΔANC at NEC onset resulted in a higher predictive value of severe surgical NEC. This study may provide a new insight into the bedside evaluation of NEC by analyzing data from the day of NEC onset.

613. DOUBLE LUMEN JEJUNAL FREE FLAP RECONSTRUCTION AFTER PROXIMAL ESOPHAGEAL RESECTION FOR MALIGNANCY

Abstract Malignancies located at the level of the upper esophageal sphincter pose a challenge due to the proximity to pharyngeal, laryngeal, and upper esophageal anatomic structures. Poorly differentiated tumors in this area can pose both diagnostic and therapeutic dilemmas, and patients with prior neck radiation may require surgery. In cases that are treated with laryngectomy, pharyngectomy, and proximal esophagectomy, jejunal free flap reconstruction can restore gastrointestinal continuity and function. A single institution early experience with multidisciplinary complex head and neck reconstruction requiring pharyngectomy and proximal esophagectomy was reviewed. Charts were reviewed for demographic details, operative details, and 30 day perioperative occurrences. Radiographic, clinical, and endoscopic records were reviewed. Method and approach for jejunal harvest and abdominal complications were obtained. Location of microvascular anastomoses were recorded for implantation of the jejunum. Length of small bowel utilized was recorded along with details of operative technique and methods for both proximal and distal anastomoses. Pre-operative and post-operative pathology reports were reviewed and compared. The initial 4 cases of pharyngeal and esophageal resection requiring jejunal free flap reconstruction were reviewed. Mean age was 65.3 (50-74), mean BMI was 31.7 (21.7-49.8), and 75% of patients were female. Median length of stay was 13.5 days (8-14) after the index operation. Flap survival rate was 100%, and there was 0% leak rate. Three patients had previous neck surgery, 75% survived to 30 days with 1 patient opting for palliative care. All flaps were harvested using a laparoscopic approach. There were no abdominal donor site related complications. Pathology 50% SCC, mucoid epidermoid carcinoma, and anaplastic thyroid carcinoma. Reconstruction of complex pharyngeal and proximal esophageal defects after oncologic resection for malignancy involving the proximal esophagus abutting the upper esophageal sphincter poses a complex challenge. These proximal esophageal defects are managed effectively with jejunal free flap reconstruction utilizing microvascular anastomoses. A double lumen jejunal free flap should be considered to restore pharygo-esophageal continuity, provides good pharyngeal size match, and can also be safely performed in patients with a re-operative field or prior neck radiation.

Impact of negative pressure wound therapy on enteroatmospheric fistulation in the septic open abdomen.

The effect of negative pressure wound therapy (NPWT) on the pathogenesis and outcome of enteroatmospheric fistulation (EAF) in the septic open abdomen (OA) is unclear. This study compares the development and outcome of EAF following NPWT with that occurring in the absence of NPWT. Consecutive patients admitted with EAF following abdominal sepsis at a National Reference Centre for intestinal failure between 01 January 2005 and 31 December 2015 were included in this study. Patients were divided into two groups based on those that had been treated with NPWT and those that had not (non-NPWT) and characteristics of their fistulas compared. Clinical outcomes concerning nutritional autonomy at 4 years and time to fistula development, size of abdominal wall defect and complete fistula closure were compared between groups. A total of 160 patients were admitted with EAF following a septic abdomen (31-NPWT and 129-non-NPWT). Median (range) time taken to fistulation after OA was longer with NPWT (18 [5-113] vs. 8 [2-60] days, p = 0.004); these patients developed a greater number of fistulas (3 [2-21] vs. 2 [1-10], p=0.01), involving a greater length of small bowel (42.5 [15-100] cm vs. 30 [3.5-170] cm, p=0.04) than those who did not receive NPWT. Following reconstructive surgery, nutritional autonomy was similar in both groups (77% vs. 72%) and a comparable number of patients were also fistula-free (100% vs. 97%). Negative pressure wound therapy appears to be associated with more complex and delayed intestinal fistulation, involving a greater length of small intestine in the septic OA. This did not, however, appear to adversely affect the overall outcome of intestinal and abdominal wall reconstruction in this study.

Motility disorders in children with intestinal failure: a national tertiary referral center experience.

Intestinal dysmotility (ID) problems are common in patients with pediatric-onset intestinal failure (IF) and short bowel syndrome (SBS), leading to significant morbidity and delays in the advancement of enteral nutrition (EN). We aimed to investigate the clinical features and complications of ID in children with IF and SBS. Retrospective chart review of all children with IF and/or SBS who required parenteral nutrition (PN) > 6weeks or small-intestinal resection ≥ 50%. Patients were divided into SBS and non-SBS groups. SBS group was divided into two subgroups: with and without ID. Patients with ID were identified (clinically, radiologically and functionally) and analyzed with regard to demographics, intestinal anatomy, complications and outcomes (short and long term). A total of 42 children with IF were treated in our institution during 2003-2022. In non-SBS group (n = 10), ID was the most common cause of IF (80%). SBS-group included 32 children; 18 children (56%) developed ID. The clinical profile of SBS-ID patients (vs SBS) was: female gender (56%), remaining small bowel length ≤ 55cm, estimated residual small bowel ≤ 28% (p = 0.045) and absence of ICV (56%). Common symptoms of the SBS-ID group were: food intolerance (61%), abdominal distension (50%), vomiting (44%), malabsorption and severe constipation. Complications included FTT (67%) (p = 0.003), bacterial overgrowth with subsequent bloodstream infection (33%) (p = 0.75), and lactic acidosis (11%). Lengthening procedure (STEP) was performed in 11 SBS-ID patients (61%) (p = 0.002). In all patients, STEP operation "rescued" their dysfunctional intestine. Eight of these patients (73%) were weaned from TPN. Survival rate was 100%; however, one SBS-ID patient is a candidate for combined intestinal and liver transplantation. ID is the most common complication of SBS and is the most common cause of IF in non-SBS patients. ID has a high morbidity rate and various clinical manifestations. Successful treatment of these infants may be achieved with the use of tapering enteroplasty.

Transluminal stenting: An intraoperative adjunct for preserving bowel length in the surgical management of necrotizing enterocolitis

Surgical management of necrotizing enterocolitis (NEC) presents a morbid dilemma: it requires maximal preservation of the small bowel length to prevent complications such as short bowel syndrome while ensuring the resection of all necrotic and non-viable bowel segments. We performed a retrospective review of four single institution cases that underwent multiple small bowel resections and anastomoses for NEC. Transluminal stenting was used prior to restoring continuity to preserve small bowel length. All four infants had >40% of predicted bowel length after their procedure, and none of the infants in the first three cases required repeat surgery after the stent removal and returned and restored small bowel continuity. The stent in the fourth case is the most recent and will remain in place for six weeks. The mean time to stent removal was 58 days for the first three cases. All four infants are alive with only mild developmental delays and are tolerating oral feeds without any parental nutrition dependency. Our experience shows that transluminal stenting is an adequate bowel lengthening procedure to lessen the risk of short bowel syndrome without increasing mortality.

213.7: A Data-driven Predictive Analytics Framework for the Analysis of Prognostic Factors and Health Outcomes in Paediatric Intestinal Failure Patients

Introduction: Machine learning (ML) involves computational methods and learning mechanisms that can help generate new knowledge from large databases. These techniques are uniquely suited to analyses of paediatric intestinal failure (IF) where classification, prognostication, identification of risk factors, and knowledge extraction can be challenging. Heterogeneous patient populations, small sample sizes, and variations in treatments may complicate our ability to manually identify trends in data. Factors that predict enteral autonomy (EA) and patient management (optimal intravenous lipid emulsion [ILE] solutions; central venous catheter [CVC] complications) have been described, yet a systematic approach that may be prospectively applied to large datasets has never been performed in this patient population. Our study aims to identify predictors of EA through machine learning (ML) and assess their impact on TPN duration. For patients on TPN, assessments of lipid emulsion (ILE) and central venous catheter (CVC) type were also performed. Methods: Demographics, procedures, and outcomes for patients (N=17) from McMaster Children’s Hospital’s (MCH) Intestinal Rehabilitation Program (1999-2021) were collected for a retrospective chart review. Predictors of EA status were obtained using a decision tree ML model. Performance was evaluated as area under the receiver operator characteristics (AUC) and precision-recall (PR) curves. These predictors were assessed for ability to impact TPN duration with Kaplan-Meier (KM) estimates and Cox proportional-hazards (CPH) models. ILE treatments were compared using the Mann-Whitney U test, and CVC types were compared with KM, CPH, and analysis of variance (ANOVA). Results: The model retrieved 10 predictors of EA status. AUC and PR curves were 91% and 71% respectively. Of these 10 predictors: sex, serial transverse enteroplasty (STEP) procedure, ileocecal valve (ICV) presence, and remnant small bowel length were significant for predicting EA status under KM, but not CPH. For ILE treatment, patients on SMOF Lipid® had lower total bilirubin values compared to Intralipid® at six months (4.0 versus 10.0 uMol/L). Longevity of peripherally inserted central catheters (PICC) was 847 days versus 268 days for tunnelled CVCs, and 181 days for ports (95% CI: 0-2044, 93-443, 144-218). Types of complications were not significantly different across CVC. Conclusions: Our study supports the role for ML frameworks in advanced pattern recognition, and promoting adaptive clinical management. Validation of this model using our single-centre dataset supports similar conclusions derived from larger, multi-center registries. Ongoing use of our ML framework offers a novel contribution to intestinal failure analyses by helping identify unique risk factors and protective factors for EA, and approaches to clinical management in the paediatric IF population.

Determining the Correct Resection Level in Patients with Hirschsprung Disease Using Contrast Enema and Full Thickness Biopsies: Can the Diagnostic Accuracy be Improved by Examining Submucosal Nerve Fiber Thickness?

BackgroundIntraoperative resection level in patients with Hirschsprung disease (HD) is determined by contrast enema, surgeon's intraoperative judgement and full thickness biopsy (FTB) identifying ganglia. This study aims to evaluate diagnostic accuracy of contrast enema and FTB in determination of resection level and whether this can be improved by measuring submucosal nerve fiber diameter. MethodsWe retrospectively analyzed contrast enema and intraoperative FTBs obtained in our center, determining diagnostic accuracy for level of resection. Gold standard was pathological examination of resection specimen. Secondly, we matched transition zone pull-through (TZPT) patients with non-TZPT patients, based on age and length of resected bowel, to blindly compare nerve fibers diameters between two groups using group comparison. ResultsFrom 2000–2021, 209 patients underwent HD surgery of whom 180 patients (138 males; median age at surgery: 13 weeks) with 18 TZPTs (10%) were included. Positive predictive value of contrast enema was 65.1%. No caliber change was found in patients with total colon aganglionosis (TCA). Negative predictive value of surgeon's intraoperative judgement and FTB in determining resection level was 79.0% and 90.0% (91.2% single-stage, 84.4% two-stage surgery) respectively. Mean nerve fiber diameter in TZPT was 25.01 µm (SD= 5.63) and in non-TZPT 24.35 µm (SD= 6.75) (p = 0.813). ConclusionDetermination of resection level with combination of contrast enema, surgeon's intraoperative judgement and FTB results in sufficient diagnostic accuracy in patients with HD. If no caliber change is seen with contrast enema, TCA should be considered. Resection level or transition zone cannot be determined by assessment of submucosal nerve fiber diameter in FTB. Type of Studyclinical research paper.

Generation of Porcine Ileum Through Spring-Mediated Mechanical Distraction

IntroductionShort bowel syndrome is a devastating gastrointestinal disorder in which decreased bowel length results in inadequate absorption causing nutritional deficiencies. Current treatment options are accompanied by significant morbidity. We have proposed spring-mediated distraction enterogenesis as a method to lengthen bowel with success seen in porcine jejunum. We hypothesize that spring-mediated distraction enterogenesis can be demonstrated in porcine ileum with preservation of ileal structure and function. Materials and methodsLaparotomy was performed on juvenile female mini-Yucatan pigs and a gelatin-encapsulated compressed nitinol spring was inserted into the ileal lumen and affixed proximally and distally. A control segment distal to the spring segment was marked with sutures. Postoperatively, pigs were placed on a liquid diet and euthanized on postoperative day 7. Spring and control segments were measured and processed for immunohistochemistry to evaluate for the presence of vitamin B12–intrinsic factor cotransporter, chromogranin A-producing cells, and 5-HT producing cells. ResultsAll seven pigs survived to postoperative day 7 with no adverse effects. On average, pigs gained 84.3 ± 66.4 g/d. Spring segments lengthened 1.5 ± 0.7 cm with a relative lengthening by 128% ± 56%, which was statistically significant when compared to control (P < 0.01). The average density of chromogranin-A cells in control compared to spring segments was not significantly changed (2.9 ± 1.1 cells/mm versus 3.2 ± 1.2 cells/mm, P = 0.17). Both vitamin B12–intrinsic factor cotransporter and 5-HT producing cells were present in both control and lengthened ileum. ConclusionsIntraluminal nitinol springs significantly lengthened porcine ileum. The increase in density of enteroendocrine cells may indicate enhanced endocrine function of the lengthened ileum.

The Variable Response to Teduglutide in Pediatric Short Bowel Syndrome: A Single Country Real-Life Experience

The glucagon-like peptide-2 analog Teduglutide has been shown to enhance intestinal absorption and decrease parenteral nutrition (PN) requirements in short bowel syndrome (SBS). As data in children is limited, we evaluated nationwide real-life experience and treatment outcome in children with SBS. Longitudinal data of children treated with Teduglutide for ≥3 months was collected. Data included demographic and medical background, anthropometrics, laboratory assessments and PN requirements. Treatment response was defined as >20% reduction in PN requirement. The study included 13 patients [54% males, median (interquartile range {IQR}) age of 6 (4.7-7) years]. The most common SBS etiology was necrotizing enterocolitis (38%), and median (IQR) small bowel length was 20 (15-40) cm. Teduglutide treatment ranged between 3 and 51 months [median (IQR) of 18 (12-30) months], with 10 patients (77%) treated >1 year. Response to treatment was observed in 8 patients (62%), with a mean [±standard deviation (SD)] treatment duration of 5.9 (±3.2) months. Among responders, 2 patients were weaned off PN and additional 4 decreased PN needs by >40%. There was a median (IQR) reduction in PN volume/kg of 36% (15%-55%) and in PN energy/kg of 27% (6%-58%). Response was not associated with patients' background, and no correlation was found with bowel length or PN dependency at baseline. Real-life response to Teduglutide is highly variable among children with SBS. While most patients did reach 20% reduction in PN, less achieved further significant reduction or enteral autonomy. No predictive factors of response to treatment were identified, and large multicenter studies are needed to elucidate predictive factors and long-term outcome.

Survival in patients initiating home parenteral support due to nonmalignant short bowel syndrome compared with background population.

Survival has frequently been studied and reported in patients with long term intestinal failure (IF). However, studies comparing the survival and mortality rates with the background population are rare. This study compares the survival in an adult IF, non-malignant, short bowel syndrome (SBS) cohort with a control group and with age- and sex-specific background mortality rates. Patients with SBS, defined by a small bowel length of 200cm or less, due to non-malignant disease, were included and followed until death or censoring on 31 December 2017. Causes of deaths occurring during home parenteral support (HPS) were assessed by review of the charts. Each case was matched with ten controls from the background population according to year of HPS initiation, age, and sex, and their survival was compared. Furthermore, age- and sex-specific mortality rates of the background population were used to calculate the standardized mortality ratio (SMR) and excess mortality. After five years, patients who initiated HPS had a relative survival of 76%. The SMR was 5.0 and the excess mortality was 50 per 1000 years. HPS-related deaths were assessed to account for 11% of deaths during HPS and occurred with an incidence of 10 per 1000 years. The excess mortality was as low as 15 per 1000 years in cases aged less than 40 years. Patients weaning off HPS had a mortality rate closer to that expected in the background population. In adult patients with a non-malignant cause of SBS-IF, the excess mortality was 50 per 1000 years. However, HPS related deaths were rarely registered with an incidence of 10 HPS related deaths per 1000 HPS treatment years.

Is the age at surgery in Crohn’s disease clinically relevant? Differences and peculiarities: a wide single centre experience after long-term follow-up

PurposeThe Montreal classification for Crohn’s disease includes “age at diagnosis” as a parameter but few is reported about the age at surgery. The aim of this study is to evaluate the short- and long-term differences in the postoperative surgical outcome and disease behaviour, according to the age at the first surgery.MethodsPatients consecutively operated for abdominal Crohn’s disease during the period 1986–2012 at our centre were systematically analysed according to their age at first surgery. In our retrospective cohort, the age at first surgery ranged from 13 to 83 years, and patients were arbitrarily divided into four groups: ≤ 19 (G1), 20–39 (G2), 40–59 (G3) and ≥ 60 (G4) years old.ResultsIn total, 1051 patients were included with a median follow-up time of 232 months. The four groups exhibited statistically significant differences in age at diagnosis, smoke habit, time between diagnosis and surgery, disease location and behaviour, history of perianal fistula or abscess, severe malnutrition requiring total parental nutrition before surgery, type of surgery, total length of resected bowel, median duration of hospitalization, incidence of abdominal recurrences and number of surgical recurrences. G1 displays an inverse linear trend with time in the severity of clinical characteristics when compared to G4 groups. On the contrary, the incidence of short-term complications, types of abdominal recurrence and presence of concomitant perianal disease did not vary among groups. In addition, at multivariate analysis, the age at surgery and the disease location were the only independent risk factors for abdominal surgical recurrence.ConclusionDespite first surgery is extremely more frequent between 20 and 59 years, patients from G1 and G4 groups showed clinical differences and peculiarities when compared to the other age groups. The most indolent CD behaviour and occurrence of surgical recurrence was observed in patients having their first abdominal surgery in the elderly, while patients operated before the age of 19 experienced a more aggressive disease course.

Development, Validation, and Evaluation of the Pediatric Inflammatory Crohn’s Magnetic Resonance Enterography Index From the ImageKids Study

Cross-sectional imaging is important in the assessment of transmural inflammation in Crohn's disease (CD). Small bowel involvement is often more extensive in pediatric CD, requiring a panentering measuring tool. We undertook to develop a magnetic resonance enterography (MRE)-based index that would measure inflammation in all segments of the intestine, without rectal contrast. Children with CD underwent ileocolonoscopy and MRE and half were prospectively followed for 18 months when MRE was repeated. Item generation and reduction were performed by a Delphi panel of pediatric radiologists, a systematic literature review, a cross-sectional study of 48 MREs, and a steering committee. Formatting and weighting were performed using multivariate modeling adjusted by a steering committee. MREs were read locally and centrally. Reliability, validity, and responsiveness were determined using several clinimetric and psychometric approaches. Thirty items were initially generated and reduced to 5 using regression analysis on 159 MREs: wall thickness, wall diffusion weighted imaging, ulcerations, mesenteric edema, and comb sign. In the validation cohort of 81 MREs, the weighted global PICMI correlated well with the radiologist global assessment (r= 0.85; P < .001) and with the simple endoscopic score in a subsample with ileocolonic disease (r= 0.63; P < .001). Interobserver and test-retest reliability were high (interclass correlation coefficients, 0.84; 95% confidence interval [CI], 0.79-0.87; and 0.81, 95% CI, 0.65-0.90, respectively; both P < .001). Excellent responsiveness was found at repeated visits (n= 116 MREs; area under the receiver operating characteristic curve 0.96; 95% CI, 0.93-0.99). Transmural healing was defined as PICMI ≤10 and response as a change of >20 points with excellent discriminative validity (area under the receiver operating characteristic curve= 0.96; 95% CI, 0.93-0.99). The PICMI is a valid, reliable, and responsive index for assessing transmural inflammation in pediatric CD. It scores the entire bowel length and does not require intravenous contrast or rectal enema and, therefore, is suitable for use in children. (ClinicalTrials.gov, Number: NCT01881490.).