Ultra-short Bowel Syndrome Research Articles

Segmental Intestinal Reconfiguration (SIR) in Combined Reconstructions for Ultrashort Bowel Syndrome

BackgroundUltrashort bowel syndrome represents the most rare and complex variant of the short bowel syndrome. Autologous intestinal reconstructions for ultrashort bowel syndrome is typically reserved for severe cases characterized by symptomatic dysmotility within the ultrashort dilated segments. Limited surgical techniques are available to substantially increase the length of ultrashort bowels with asymmetric and highly dilated segments.We present a case series detailing the application of a technique for lengthening and tapering significantly dilated ultrashort bowels – segmental intestinal reconfiguration (SIR), based on the Yang-Monti principle. Our analysis delves into the technical features across various anatomical variants and provides mid-term results. MethodsFrom 2021 to 2022, patients experiencing dysmotility in dilated ultrashort bowel segments with a preserved colon underwent sequential combined intestinal reconstructions. Assessments were conducted 18–24 months post-surgery, and statistical calculations employed nonparametric methods to compare pre- and postoperative results. ResultsSix patients, with a median age of 48.5 months, underwent autologous intestinal reconstructions, including SIR. The serial transverse enteroplasty (STEP) was performed on the dilated duodenum in five children, and colon interposition in two. The length of the small bowel exhibited a significant difference before and after surgery (p=0.031). No complications were observed in the postoperative period. The postoperative group demonstrated a lower dependence on parenteral nutrition (NPEI/REE < 80% in 6 (100.0%); p=0.007). Enteral autonomy was restored in two children. ConclusionsThe results of SIR technique in combined reconstructions for ultrashort bowel syndrome in a carefully selected cohort of children demonstrate feasibility and reliability, as well as the restoration of intestinal transport function at midterm evaluation. This comprehensive approach resulted in increased enteral tolerance and reduced parenteral requirements.

Post-operative Ultrashort Bowel Syndrome in a Term Neonate: One-year Follow-up

Ultrashort bowel syndrome occurs when the length of the small bowel is below 10–25 cm, or 10% of that expected for age. It is a rare occurrence in neonates; however, when it occurs, it results in high morbidity and mortality. Surviving newborns face a significant detriment in growth and development due to malabsorption of nutrients. A multidisciplinary approach with aggressive parenteral nutrition is the mainstay of management. Our case presented at 20 days of life, with malrotation and midgut volvulus resulting in post-surgical ultrashort bowel syndrome. In spite of being left with only 20 cm of short bowel, the infant survived on conservative management and is thriving well; the infant has been off parenteral nutrition completely since 11 months of age.

Ileal lengthening through internal distraction: A novel procedure for ultrashort bowel syndrome

PurposeUltrashort bowel syndrome is a rare, but morbid surgical problem without effective treatment. Recent clinical analysis has demonstrated the critical influence of ileal length on ultimate enteral autonomy. Surgical techniques to increase ileal length in nondilated bowel do not exist. We describe a novel technique to lengthen ileum in children with ultrashort bowel syndrome. MethodsBeginning in May 2021 prospective candidate children were identified. Candidacy for ileal tube lengthening included diagnosis of ultrashort bowel syndrome, intact ileocecal valve with remnant ileum, and proximal intestinal stoma or draining gastrostomy. Informed consent was obtained. Following laparoscopic lysis of adhesions, a balloon catheter was inserted through a left flank stab incision and into the lumen of the remnant ileum around a purse string suture. Cecopexy was performed in the right-lower quadrant. Clips were used to mark the cecum and the proximal extent of ileum. The catheter length was fixed externally at the completion of the procedure. Serial x-rays were used to measure distraction effect while increasing tension was applied to the catheter over the subsequent weeks. Ileal tube lengthening was performed until the end of the catheter was reached or the tube was dislodged. A contrast study was performed at the completion of lengthening. Intestinal length at time of restoration of continuity and clinical outcomes were recorded. ResultsFour infants were enrolled from May 2021-July 2023. Diagnoses leading to ultrashort bowel syndrome were mesenteric teratoma, necrotizing enterocolitis, and multiple intestinal atresia. At the time of restoration of intestinal continuity, a median of 1.75 cm (45 %) additional ileal length was achieved at a median of 25.5 days. There were no serious complications following ileal tube lengthening and no additional operative interventions were required. ConclusionsIleal lengthening through internal distraction is a feasible surgical intervention to salvage ileum for infants with ultrashort bowel syndrome. Ileal tube lengthening may result in distraction enterogenesis, providing a novel intervention to increase intestinal length. Level of evidenceIV (Case series without comparison group).

Analysis of risk factors for neonatal short bowel syndrome in two tertiary centers in Brazil

IntroductionShort bowel syndrome (SBS) is defined as a severe reduction in intestinal mucosal absorption secondary to reduced intestinal functional area. The main causative factor for SBS is the length of intestinal resection in the neonatal period. The prognosticating factors are age, length of remnant intestine, preserved ileocecal valve, and venous maintenance. ObjectiveTo evaluate the clinical and surgical aspects affecting survival outcomes in neonates with SBS at two tertiary care centers. Patients & methodsRetrospective cohort study involving two university tertiary care neonatal centers with diagnosed SBS. Neonates were allocated into two groups, SBS (<60 cm) and USBS (ultra-short bowel syndrome) (<25 cm) and demographically compared. ResultsIn total, 53 neonates met the inclusion criteria, with 31 and 22 in the SBS and USBS, respectively; 20 survived and 33 died (37 % survival rate). The remnant bowel length was greater in those with SBS (p < 0.001), with greater ability to acquire full adaptation (p = 0.047). Increased survival rate was associated with use of total parenteral nutrition (TPN) (p = 0.002) and enteral nutrition (p = 0.036), and the ability to acquire full intestinal adaptation (p = 0.047). Risk factors associated with mortality were the inability to tolerate enteral nutrition (p = 0.036) and acquire full intestinal adaptation (p < 0.001); Use of TPN proved to be protective against death (p = 0.002). ConclusionSBS has high mortality rates in neonatal period (57 %). We recommend caution with time taken to full intestinal adaptation, with continued TPN administration during this time, which can improve overall survival outcomes.

Опыт применения аналога глюкагоноподобного пептида-2 у ребенка с синдромом короткой кишки

This article presents a clinical case of ultra-short bowel syndrome. The development of the underlying disease and comorbid disorders are described in dynamics, difficulties and therapeutic options are discussed. This clinical case reflects the real practice of managing a child with a rare disease using current possibilities of pharmacotherapy. Key words: short bowel syndrome, nutritional status, intestinal adaptation, glucagon-like peptide-2 analog

Unenviable decisions: Is it ethically justifiable to withhold parenteral nutrition from infants with ultra-short bowel syndrome?

Infant A was born at term with an antenatal diagnosis of gastroschisis. His parents were well informed about the condition and understood that he would require surgery. However, at delivery, his bowel was found to be severely compromised. Infant A returned from theatre with only four centimeters of small bowel. This is physiologically devastating and easily qualifies as ultrashort bowel syndrome (USBS). Whilst the prognosis from ultrashort bowel syndrome is greatly improving, the condition continues to carry a significant risk of mortality and morbidity, in part attributable to treatment itself. The cornerstone of management of USBS is provision of intravenous parenteral nutrition (PN). This is not a physiologically normal route of nutrition; it is a medical treatment. Infant A’s parents questioned whether continuation of active treatment was appropriate. If a treatment is not in the patient’s best interests, then it can be argued it is not justified to administer it. Decisions about quality of life are intensely personal. Where there is a significant burden of treatment, even when there is a potential for increased survival, whether the course of treatment is in the child’s best interests must be taken with huge emphasis on the parental perspective and their family values. For well-informed, realistic parents who are welcoming of the full picture of information and implications of their decision, I argue that parents are best placed to make the decision for their child. Long-term PN for USBS may well be a medically encouraged treatment. However, it should not be medically mandated.

Bridging Liver Transplantation in the Treatment of Intestinal Failure Associated Liver Disease in Infants-A Bridge Too Far?

Infants with intestinal failure associated liver disease (IFALD) requiring liver and bowel transplant have a high mortality on the transplant waiting list due to the scarcity of the size-matched donor organs. Bridging liver transplantation has been used to allow the children to grow to a reasonable size so that a combined liver and small bowel transplant could be performed in the future. We report on two children with irreversible intestinal failure (ultra-short bowel syndrome secondary to gastroschisis and microvillous inclusion disease) with IFALD who underwent bridging liver transplantation at our institution. Both patients made a good recovery from their initial surgery. One patient died 6 months following surgery from generalized sepsis, and the other patient survived in good condition to undergo a combined liver and small bowel transplant but died a few days post-transplant. In the current era of scarcity of donor organs, this raises an ethical dilemma for the team involved regarding appropriate utilisation of a scarce resource.

Comparing bowel lengthening procedures: which, when, and why?

Purpose of reviewIntestinal failure secondary to short bowel syndrome is still a very serious condition. Treatment consists of parenteral nutrition to provide nutrients and maintain body weight. During the last decades, intestinal lengthening procedures have become more available. The goal of this review is to discuss the results of the literature on the most commonly performed intestinal lengthening procedures.Recent findingsLongitudinal Intestinal Lengthening, Serial Transverse Enteroplasty (STEP), and Spiral Intestinal Lengthening and Tailoring (SILT) are currently the most frequently reported intestinal lengthening procedures. The most recent literature of these procedures is described with respect to indication, technical details, complications, short and long-term outcome, and PN independence.SummaryOn the basis of indication, surgical complexity, complications, and clinical success, we conclude that the STEP procedure is probably the best choice for most centers.

O-10: Outcomes of Children with Intestinal Failure Secondary to Ultrashort Bowel Syndrome

O-10: Outcomes of Children with Intestinal Failure Secondary to Ultrashort Bowel Syndrome

Intestinal rehabilitation with ultrashort bowel syndrome due to necrotizing fungal enteritis

Abstract Introduction Ultra-short bowel syndrome (USBS) was once considered incompatible with life. It can be secondary to multiple cases but most rarely infection or autoimmune disease. Case: We discuss a 13-yr old female with Polyarteritis Nodosa complicated by a necrotizing fungal infection due to C. tropicalis who developed USBS after requiring extensive resection of her bowel, omentum and abdominal wall due to an invasive fungal infection, leaving her with less than 5 cm of jejunum and residual left colon. With intestinal rehabilitation she was able to be weaned down to 70% enteral nutrition and 30% TPN. Conclusion Ultra-short bowel syndrome is increasing in the pediatric population as rates of survival continue to improve. After management of the acute disease, in this case severe infection and vascular disease, long term management with a focus on providing high-quality multidisciplinary intestinal rehabilitation is vital as well as minimizing PN complications.

S3549 Electrolyte Derangement Due to Medication Shortage in an Ultra Short Bowel Syndrome

S3549 Electrolyte Derangement Due to Medication Shortage in an Ultra Short Bowel Syndrome

First Small Intestine Transplant in Western India: An Initial Experience

To report first deceased donor isolated small intestine transplant (DDIT) from western India and discuss feasibility, procedure and outcome of this complex surgery. A 27-year-old male required massive small bowel resection for gangrene following small bowel volvulus. Patient developed ultrashort bowel syndrome with high output stoma. He was started on total parenteral nutrition. Inability to maintain nutrition along with deranged liver functions necessitated early listing for deceased donor small intestinal transplant. DDIT was performed using small intestine from size matched for a 42-year-old female. Inflow was taken from the infrarenal abdominal aorta. The superior mesenteric vein of the graft was drained into the inferior vena cava. Standard immunosuppression and antibiotic prophylaxis were followed. The graft functioned well, and patient was completely weaned off parenteral nutrition and discharged on day 14. However, he had sudden deterioration of health at home 1 month later and succumbed to death before reaching the hospital. Small intestine transplant is a viable option in patients with intestinal failure with complications of parenteral nutrition. Post-operative care and management remains a bigger challenge. There is a need to increase the awareness and develop expertise in successful execution of this complex procedure.

Gastrointestinal Tract Reconstruction in Adults with Ultra-Short Bowel Syndrome: Surgical and Nutritional Outcomes

BackgroundHistorically, adults with ultra short bowel syndrome (USBS) have been considered candidates for lifetime parenteral nutrition (PN) or are referred for visceral transplantation. We examined the surgical and nutritional outcomes of adult patients with USBS managed at a single intestinal rehabilitation center. MethodsWe retrospectively reviewed data on 588 adult patients referred to our center between January 2013 and December 2018. USBS was defined as residual small bowel (SB) length ≤ 50 cm. ResultsForty-five patients (7.6%) with a mean age of 46.7 years (range 17-78) were identified. Indications for enterectomy included mesenteric ischemia (n=17) and internal hernias (n=6), followed by large intraabdominal fibroids, trauma, and allograft enterectomies, with five cases each. Median SB length was 18.0 cm; 20 patients (44.4%) had their entire SB resected. Thirteen patients had an intact colon, of which nine had preservation of the ileocecal valve. Patients who underwent autologous reconstruction of their gastrointestinal (GI) tract required a lower total PN volume (29.0 ± 7.6 vs. 40.8 ± 13.2 ml/Kg/day, p=0.002) and presented better short- and long- term survival (p=0.005). Patients with no gut had higher mortality (p=0.036). Hormonal therapy with the glucagon-like peptide-2 analog teduglutide was used in nine patients (20%) five of whom were weaned off TPN. Excluding patients with no gut (n=20), discontinuation of total PN rate for patients with an end ostomy or tube decompression (n= 6), jejunocolostomy (n= 10), and jejunoileostomy (n=9) were 0%, 40%, and 77.7%, respectively. Eleven patients (44%) with some residual small intestine achieved nutritional autonomy in an average of 20 months after GI reconstruction. Fifteen patients were listed for transplantation (33.3%). Seven patients underwent isolated SB transplantation and achieved nutritional autonomy in an average of three months after transplantation. One-year patient and graft survival were 100%. After a 37-month median follow-up period, 36 of 42 patients followed by our center were still alive (85.7%). ConclusionNutritional autonomy can be achieved in a significant number of patients with USBS in specialized centers with surgical and/or hormonal therapy. The presence of an intact colon and ileocecal valve can significantly increase the adaptation rate. Moreover, restoration of GI tract continuity has a positive impact on medical management and survival.

Ultrashort Bowel Syndrome: long-term survival without ParenteralNutrition

Short Bowel Syndrome (SBS) is a condition in which nutrients are not absorbed (malabsorption) because a large part of the small intestine is missing or has been surgically removed, developing an Intestinal Failure (IF), that is defined as a loss of absorptive capacity secondary to obstruction, dysmotility, surgical resection, congenital defect, or mucosal disease resulting in chronic diarrhea, dehydration, electrolyte abnormalities, micronutrient imbalance, and malnutrition. (1) The long-term survival without parenteral nutrition in patients with the diagnosis of ultrashort bowel syndrome is difficult. This is a case, first reported in the country, of a 63 years old woman with ultrashort bowel syndrome who after three abdominal surgeries was left with only about 35 cm of small intestine with a jejune-transverse anastomosis. During the first surgery the right colon was removed. From the beginning she received Total Parenteral Nutrition (TPN) lasting about three months, at which time she presented a serious reaction after which she refused further TPN treatment. She lived with oral feeding including nutritional supplements for almost five years, and one significant complication,diarrhea, was managed with loperamida and octreotride.

Multidisciplinary Management in Pediatric Ultrashort Bowel Syndrome.

Pediatric intestinal failure (IF) remains a complex and devastating condition resulting in the inability of the gastrointestinal tract to absorb adequate fluids and nutrients to sustain life. The goal in the management of IF is to achieve enteral autonomy and when not possible to avoid and minimize the development of long-term complications. Survival rates for children with IF have continued to improve resulting in an increased population of children with more altered anatomy. While IF remains a rare disease, children with IF secondary to ultrashort bowel syndrome comprise an even smaller patient population. The goal of this article is to review the recent literature related to the impact of multidisciplinary intestinal rehabilitation programs (IRPs) on the management and outcomes of intestinal failure in children with ultrashort bowel syndrome and potential avenues to further improve the long-term outcomes of this patient population.

Protein disulfide isomerase A3: A potential regulatory factor of colon epithelial cells

Aim: This study aimed to investigate the effects of protein disulfide isomerase A3 (PDIA3) on the proliferation and apoptosis of colon epithelial cells, so as to explore its possible role in colon compensation of ultra-short bowel syndrome. Methods: The expression of PDIA3 gene in NCM460 colonic epithelial cells was upregulated and silenced by liposome transient transfection technique. The expression of PDIA3 protein was determined by Western blotting, the proliferation rate of NCM460 cells was detected by CCK8, and the apoptosis rate of NCM460 cells was determined by flow cytometry. Results: DNA sequencing and Western blotting results successfully verified that PDIA3 protein expression in NCM460 cells was upregulated and silenced by liposomal transfection. In the PDIA3 overexpression experiment, the proliferation rate of the experimental group was lower than that of the empty carrier group at 24 h, 48 h, and 72 h, and the apoptosis rate of the experimental group was higher than that of the empty carrier group (P Conclusion: PDIA3 inhibits the proliferation of human colonic epithelial cells (NCM460) and promotes their apoptosis, which may not be a key regulatory protein in colon compensation of ultra-short bowel syndrome.

Torsional Necrosis of an Output Loop Internal Hernia After Roux-en-Y Choledochojejunostomy: A Case Report

Ultra-short bowel syndrome (USBS) refers to a clinical condition characterized mainly by severe diarrhea and nutritional disorders caused by the residual small intestine length of <30 cm (1). After Roux-en-Y choledochojejunostomy, the output loop herniated from the back of the anti-reverse peristalsis loop, resulting in intestinal torsion and necrosis, and leading to this rare case of internal hernia. Patients with torsion often show severe abdominal pain and persistent aggravation, but this symptom lacks specificity, making it easy to be misdiagnosed. Acute intestinal torsion and necrosis often lead to large-scale small bowel resection, resulting in USBS. We report a case of torsional necrosis of an output loop internal hernia after Roux-en-Y choledochojejunostomy, providing certain reference for the diagnosis and management of such cases.

Radiologic Image-Guided Tube Stoma Insertion in Neonatal Short Bowel Syndrome: First Case Report in the Literature.

Background: The management of neonatal short bowel syndrome can be challenging, and it is critical that these babies are managed in a multidisciplinary team setting with specialists who are experienced in the complex management of these children. One of the surgical strategies, initially published by the Bianchi team in Manchester, UK, is controlled tissue expansion program (CTE) which is done via the insertion of catheters into the proximal and distal bowel in the form of tube stomas. The clamping of the proximal tube allows for an increase in length and circumferential diameter of the proximal bowel for a period of time, whilst the distal tube stoma allows for easy refeeding of proximal bowel contents into the distal bowel. Method: CTE is associated with the risk of dislodgement and exposing patients to further surgical procedures with the risk of losing more bowel length. This article describes a new method in the management of such a complication through a less invasive approach of an image-guided procedure by interventional radiologists. Conclusion: Radiologically guided tube stoma reinsertion in a child with ultrashort bowel syndrome is a promising technique and should be considered in a CTE program in the management of short bowel syndrome.

P1.56: Colonic continuity and Incidence of weaning from PN by 5 years of age in children with onset of intestinal failure(IF) related to short bowel syndrome(SBS) by 3 months of age with chronic persistent dependence on parenteral nutrition(PN) at one year of age

Objectives: To review 5-year outcome of children presenting in infancy with chronic intestinal failure (IF) associated with short bowel syndrome (SBS) who were still on parenteral nutrition (PN) treatment at 12 months of age. Method: Medical records of patients with SBS related IF presenting in neonatal period and up to 3 months of age from 2007–2017 who were still on PN at 12 months of age were reviewed. Data obtained included small intestinal length at time of resection, aetiology, presence/absence of ileo-caecal (I-C) valve and/or colon and number of patients still on PN at 5 years. Results: Twenty patients were identified. Eleven were male and 9 female. 14/20 were born prematurely. Diagnoses were necrotising enterocolitis (NEC) in 9, volvulus in 4, long segment Hirschsprung’s disease in 3, gastroschisis in 2, and intestinal atresia in 2. The remaining small intestinal length at time of surgery ranged from 4.5cm - 120cm (median 18cm) in 19 cases (not measured in 1). Four patients had ultra SBS (length <10cm). Eleven patients weaned from PN by 5 years of age and 9 were still on PN. The median age at time of weaning (corrected for gestational age in premature infants) was 2 years 4 months (range 1 year 6 months - 5 years 9 months). Small intestinal length ranged from 7–120 cm (median 30cm) in the weaned group and from 4–90 cm (median 20cm) in the persistent IF group. Seven/11 patients who weaned and 2/9 still on PN were female (p=0.09 not significant). 8/11 who weaned and 2/9 still on PN had NEC (p=0.068, not significant). 7/11 who weaned had an intact I-C valve compared to 3/9 on PN (p=0.6 not significant). All 11 children who weaned had a colon present compared to 5/9 who remained on PN (p=0.026, significant). 11/16 or 68% with colon in continuity weaned. Conclusion: Over 50% (11/20) infants with SBS and PN dependence for >12 months weaned from PN by or at 5 years of age. There was a statistically significant association with presence of colon with 68% with a colon weaning and even with some cases of ultra-SBS. Larger studies are needed to gain other predictors of weaning in this diverse group of patients.

320.3: Ultra-Short Bowel Syndrome (USBS) – Does the lengthening procedures improve the outcomes? Intestinal Rehabilitation Program (IRP) Children’s National Medical Center (CNMC) Washington DC - USA

320.3: Ultra-Short Bowel Syndrome (USBS) – Does the lengthening procedures improve the outcomes? Intestinal Rehabilitation Program (IRP) Children’s National Medical Center (CNMC) Washington DC - USA