Short Bowel Syndrome Research Articles

Prenatal-Postnatal Outcomes and Prognostic Risk Factors of Fetal Volvulus: Analysis of 26 Cases.

The aim of the current study was to reveal ultrasonographic and clinical features, prenatal-postnatal outcomes and prognostic risk factors of fetal volvulus. This retrospective study evaluated all cases of fetal volvulus diagnosed between 2018 and 2024 at the Perinatology center of Zeynep Kamil Women and Children Diseases Training and Research Hospital. In the vast majority of cases, the pediatric surgery team confirmed the conclusive diagnosis of volvulus during the postnatal period. The cohort was divided into two groups, the "survivor" and "deceased", in order to compare the outcome and to evaluate factors determining the outcome. A total of 26 cases of fetal volvulus were followed up in our perinatology center which were confirmed by postnatal pediatric surgery or autopsy. Termination of pregnancy in two cases and intrauterine fetal death in one case were observed. Twenty-three cases reached live birth. Preterm labor, fetal growth restriction, ascites and decreased intestinal peristalsis were significantly more common in the deceased group. Neonatal death in five cases (19.2%), infant death in four cases (15.3%) and short gut syndrome in three cases (11.5%) were long term outcomes. Overall mortality rate may increase when fetal growth restriction, ascites, and decreased intestinal peristalsis are present with volvulus.

Mechanical distraction enterogenesis utilizing springs has equal effectiveness in adult and juvenile pigs.

We aim to apply our spring-mediated intestinal lengthening technique to adults and children with short bowel syndrome. We hypothesized that spring-mediated intestinal lengthening would be achieved in adult and juvenile pigs. Adult and juvenile pigs underwent insertion of a compressed spring into the jejunum. Jejunum was plicated proximal and distal to the capsule for fixation and control jejunum was marked. Pigs were fed a liquid diet for one week. Spring and control segment lengths were compared using Brown-Forsythe and Welch ANOVA test. Three adult pigs weighing 30.4 ± 2.1kg and three juvenile pigs weighing 8.9 ± 1.2kg underwent spring insertion without complications. Springs were compressed from 7.5 to 2cm and had an average spring constant of 9.7 ± 1.2N/m in adults and 5.4 ± 1.5N/m in juveniles. Springs of 1.3cm diameter were implanted in adults and springs of 1.0cm diameter were implanted in juveniles. Spring segments lengthened on average by 86 ± 33% in adults (p = 0.04) and 123% in juveniles (p < 0.01) when compared to the initial length. There was no statistically significant difference in lengthening between juvenile and adult pigs (p = 0.2). Intestinal lengthening was feasible in adult pigs but required a higher force for their larger diameter intestine compared to juvenile pigs.

Continuous glucose monitoring in adults with short bowel syndrome receiving overnight infusions of home parenteral nutrition.

Consumers of home parenteral nutrition (HPN) are susceptible to dysglycemia. The aim was to characterize 24-h glucose profiles of HPN consumers using continuous glucose monitors (CGM) and to identify factors that influence glucose. Glucose profiles of 20 adults with short bowel syndrome (SBS) without diabetes were assessed using the Freestyle Libre Pro CGM. Measures included mean 24-h glucose, coefficient of variation (%), % time in range (TIR 70-140 mg/dL), among others. HPN parameters and lifestyle behaviors were obtained from self-reports and validated surveys. Linear mixed-effects models were used to test associations with glycemic measures adjusted for age, sex, and BMI. Significance was considered at P < 0.05. Participants (77% female, age = 52 years, BMI = 21.4 kg/m², 95% white) had a 24-h mean and CV for glucose of 94.69 (8.96) mg/dL and 20.27%, respectively, and a mean TIR of 87.73%. Among non-daily HPN-dependent patients, the mean glucose and TIR were higher on days receiving HPN. Tapering HPN was associated with -6.882 (95% confidence interval = -12.436, -1.329) % lower CV, and higher HPN dextrose content per gram was associated with 0.039 (95% confidence interval = 0.008, 0.07) % higher CV. Smoking, more depressive symptoms, and higher insomnia severity showed associations with glucose levels and variability. Metabolically stable HPN adult consumers have 24-h glucose measures comparable to healthy adults yet are notable for more time spent below range. The glucose profiles are influenced by HPN parameters such as tapering and dextrose and behaviors including smoking, depressive symptoms, and insomnia.

Home Parenteral Support in Chronic Intestinal Failure-First Results from a Pioneer Portuguese Intestinal Failure Center.

Home parenteral support (HPS) is the core of chronic intestinal failure (IF) treatment. For legal reasons, HPS in Portugal lags behind other European countries, and only a few patients were taken care of at home by nurses. Now, the legislation has changed, allowing patient self-care. The authors report their pioneer experience as the largest Portuguese IF center, evaluating the underlying conditions leading to IF, HPS nutritional impact, HPS-related complications and survival. This is a retrospective study including IF patients who underwent HPS in a Portuguese IF center. The data included demographics, underlying conditions, IF types, HPS duration, BMI at the beginning and end of HPS/follow-up, complications, microbiological agents of infectious complications and current status (deceased or alive with/without HPS). Survival was calculated until death or September 2024. A total of 23 patients (52.2% female, mean age 57.3 years), all with type III IF, were included. Short bowel syndrome (SBS) was the most common cause of IF (69.6%). Of the included patients, 78.3% received home parenteral nutrition; the others received home parenteral hydration. The mean BMI increased significantly, from 19.1 kg/m2 to 22.5 kg/m2 (p < 0.001). Two patients received Teduglutide. The most common complication was catheter-related bloodstream infection (2.5/1000 catheter days). The complications did not increase with patient self-care. At the end of follow-up, 21.7% of patients remained on HPS, 34.8% were alive without HPS, and 43.5% died. The average survival was 43.4 months. One death (4.35%) was attributable to HPS-related complications. The conditions underlying IF varied, with SBS being the most frequent condition. HPS improved the BMI, allowing considerable survival. Despite the complications and one attributable death, HPS was safe, even when relying on patient self-care.

Favorable inhibitory effect of clodronate on hepatic steatosis in short bowel syndrome model rats

PurposeThis study investigated the anti-inflammatory effect of clodronate, a vesicular nucleotide transporter (VNUT) inhibitor, on intestinal-failure-associated liver disease (IFALD) in a rat model of short bowel syndrome (SBS).MethodsThe rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90% small bowel resection. The animals were divided into the following groups: TPN/SBS (Control group), TPN/SBS/intravenous administration of low-dose clodronate (20 mg/kg twice per week; Low group), or TPN/SBS/intravenous administration of high-dose clodronate (60 mg/kg twice per week; High group). On day 7, the rats were euthanized. Hepatic steatosis and hepatocellular injury were also assessed.ResultsHepatic steatosis and lobular inflammation in the liver were observed in all groups. The High group showed histologically reduced hepatic steatosis compared with the Control group. IL-6 and Nlrp3 expression in the High group was significantly suppressed compared to that in the Control group. The expression of other inflammatory cytokines tended to be lower in the High dose group than in the control group. The lipid metabolism gene expression in the liver specimens showed no significant differences among the groups.ConclusionThe high-dose administration of clodronate may, therefore, inhibit hepatic steatosis and inflammation associated with IFALD in patients with SBS.

Clinical significance of determining the level of citrulline in children who have undergone intestinal resection during the neonatal period

Citrulline is an amino acid that is not found in protein and does not enter the body through food, enteral or parenteral nutrition. It is instead synthesized exclusively by enterocytes. Therefore, citrulline concentrations in the blood can reflect the process of biosynthesis in the intestines and are directly related to changes in the function of these cells.Purpose. The aim of the study was to assess citrulline levels in children who had undergone intestinal resection during the neonatal period.Material and methods. The study included 22 children with short bowel syndrome, diagnosed by a doctor (main group; average age 3.7 months). We also included 20 children who had undergone surgery to remove less than 50% of their intestine but did not have short bowel syndrome (comparison group; average age 3 months 1 day). The control group consisted of 23 healthy children who had never had surgery on their gastrointestinal tract and did not have any congenital malformations or clinical signs of enteropathy (average age 5 months 3 days). High performance liquid chromatography-mass spectrometry (HPLC-MS) was used to measure the level of citrulline.Results. The average level of citrulline in the blood of children in the main group was 18 μmol/l (95% CI 10–22.8), in the comparison group – 17.5 μmol/l (95% CI 16–21), in the control group – 26 μmol/l (95% CI 22–3). Citrulline levels in patients who underwent surgery in the neonatal period were significantly different from those of children in the control group There were no differences in the content of citrulline between the main and comparison groups, depending on whether the colon was preserved or not and whether the ileocecal valve was present (p&gt;0.05).Conclusion. An increase in citrulline level in the blood of children receiving therapy with a glucagon-like peptide-2 analog indicates the possibility of using this marker to monitor changes in intestinal absorption associated with physiological or pharmacological adaptation of the intestine.

Features of the diagnostics and surgical treatment of necrotizing enterocolitis

Introduction. Necrotizing enterocolitis (NEC) is one of the common causes for abdominal surgery in newborns and infants over he first months of life with a high mortality rate (23.5%), increased in case of surgery to 67%. Materials and methods. We compared premature (n = 25) and full-term (n = 22) newborns operated for NEC complications (laparotomy, resection of necrotized sections of the intestine, intestinal stoma). Also, we evaluated the results of treatment of infants with intestinal stoma after surgical NEC (n = 110, groups 1, 2 and 3). Results. Full-term cases with congenital heart defects are at risk of surgical NEC (p &lt; 0.05). Subtotal and total forms of NEC are associated with lethal outcomes up to 100% in both premature and full-term infants. It is necessary to preserve the ileocecal angle; length measuring of the remaining intestine along the mesenteric edge helps to predict further risks: 60 cm and less leads to 80–100% of post-resection short bowel syndrome. Conclusion. NEC in full-term newborns is due to its hypoxic nature and impaired mesenteric perfusion (congenital heart defects). Surgical NEC can occur without typical symptoms (“covered perforation”), the discrepancy between the clinical and radiographic picture may indicate to subtotal or total intestinal necrosis with up to 100% of lethal outcomes.

Management Patterns of Teduglutide Use in Short Bowel Syndrome: A Survey of 70 Healthcare Professionals.

This study aimed to gain real-world insights from healthcare professionals (HCPs) regarding the management of adult patients with short bowel syndrome and intestinal failure (SBS-IF) who received teduglutide and achieved parenteral support (PS) independence or PS volume stability for ≥12 months. This cross-sectional survey was conducted in five European countries and Canada via a self-reported questionnaire (November 2022-March 2023) among HCPs who manage patients with SBS-IF and who had prescribed teduglutide to ≥5 patients with SBS-IF receiving PS. Of the 70 HCPs who completed the survey, almost all reported managing patients with SBS-IF who achieved PS independence or PS volume stability (99%, 69/70 and 97%, 68/70, respectively) and maintained the standard teduglutide dose, without changes. A total of 52 HCPs managed patients who achieved PS independence and discontinued teduglutide. Of these HCPs, 73% (38/52) anticipated that these patients would remain PS-independent, not requiring PS reintroduction. Of the remainder, 79% (11/14) estimated that ≤40% of these patients would require PS reintroduction. While many HCPs (81%, 42/52) would reintroduce teduglutide in patients who discontinued its use after achieving PS independence, none would do so for patients who discontinued teduglutide after achieving PS volume stability if a patient's condition worsened. This survey found that patients with SBS-IF can achieve PS independence or PS volume stability with teduglutide treatment. However, some HCPs (27%, 14/52) believe that a proportion of patients discontinuing teduglutide after achieving PS independence will require PS reintroduction. This survey suggests that teduglutide treatment should continue uninterrupted, unless clinically indicated, but this requires confirmation in future studies.

Real-world experience with glucagon-like peptide 2 analogues in patients with short bowel syndrome and chronic intestinal failure: Results from an international survey in expert intestinal failure centers

Background and AimsGlucagon-like peptide 2 (GLP-2) analogues are the first available disease-modifying treatments for patients with intestinal failure (IF) due to short bowel syndrome (SBS). Efficacy in terms of reduction of parenteral support (PS) has been demonstrated in multiple studies and real-world reports. However, it remains unclear how many patients are eligible to receive the treatment, when treatment is started after intestinal resection, how treatment efficacy is assessed outside of clinical trials, and how the treatment is modified in case of non-response or adverse events. The aim of this study was to investigate the real-world management of patients treated with GLP-2 analogues in expert centers around the world. MethodsA survey questionnaire was developed by a multidisciplinary working group consisting of 52 questions related to various aspects of multidisciplinary care of SBS-IF patients. The 17 questions related to the use of GLP-2 analogues in clinical practice were analyzed for this study. The online survey was sent to 33 participating centers in a phase 3 study of a long-acting GLP-2 analogue. Only responses from countries with access to commercially available GLP-2 analogues were included in the study. A descriptive analysis was performed for each question. Results are presented as median (interquartile range). ResultsThe responses from the 19 expert IF centers with access to GLP-2 analogues indicated that 10 (10-20) % of patients with SBS-IF were treated with a GLP-2 analogue, which was less than the number of eligible patients (30 (25-40) %). In most centers (10 centers, 53%), GLP-2 therapy was started 6-12 months after the last intestinal resection, with 5 centers (26%) starting later (12-24 months). Multiple parameters were used in combination to determine the response to GLP-2 analogues of which the three most common were >20% decrease in PS (95%), at least 1 day of PS reduction per week (84%) and increased urinary output (68%). In non-responders GLP-2 therapy was stopped within the first year by 67% of the centers. Finally, strategies in case of significant adverse events include stopping the GLP-2 analogue (used by 79% of experts), dose reduction (67%) and temporary treatment interruption (62%). ConclusionThe results of this survey completed by expert IF centers show the real-life use of GLP-2 analogues in clinical practice. Key learning points identified include the accounting for a period of intestinal adaptation before starting GLP-2 analogues and not stopping the treatment too early in case of non-response. The best strategy in case of adverse effects should be studied further.

Online registry of neonatal necrotising enterocolitis in Shenzhen: protocol for a multicentre, prospective, open, observational cohort study

IntroductionNecrotising enterocolitis (NEC) of the intestine of preterm infants leads to the risk of abdominal surgery, short bowel syndrome, neurodevelopmental disorders and death. Although the risks of NEC and its...

Non-surgical Interventions for Improving Nutrient Absorption in Pediatric Short Bowel Syndrome

Non-surgical Interventions for Improving Nutrient Absorption in Pediatric Short Bowel Syndrome

007. Distal Ileal Atresia and Total Colon Agenesis with Anorectal Malformation: A Case Report

Background: Atresia of the distal ileum and colon agenesis are rare conditions and can be the cause of obstructive symptoms. Case: A 1 day old baby girl complained of no anus since birth. The patient has no bowel movements or vomiting. The patient's stomach appears increasingly bloated. On physical examination of the abdomen revealed distension accompanied by difficult to hear bowel sounds. There was no anus or fistula in the patient but there is a dimple in the anus. Another abnormality found in the patient was the presence of CTEV on both of the patient's legs. The patient was planned for exploratory laparotomy and colostomy surgery. the length of the jejunum and ileum proximal to the ligament of Treitz was found to be 50 cm. The distal ileum, caecum, colon, rectum and anus were not found. An ileostomy was performed on this patient. The patient died 13 hours after surgery. Conclusion: Total colonic agenesis is a rare case and usually occurs with other congenital abnormalities. Early diagnosis and appropriate initial treatment are very important in this case. colonic agenesis causes congenital short bowel (CSB). CSB causes malabsorption due to inadequate intestinal length, which interferes with absorption. Staged surgery is a good option in the management of total colonic agenesis.

Dipeptidyl peptidase IV inhibitors reduce hepatic fibrosis and lipid accumulation in rat intestinal failure-associated liver disease models.

This study aimed to investigate the effectiveness of dipeptidyl peptidase IV inhibitors (DPP4-I) against liver damage, especially fibrosis and lipid accumulation, in a rat intestinal failure-associated liver disease (IFALD) model. SD rats were divided into two groups: the Control (n = 7; normal saline + IFALD model) and DPP4-I (n = 7; DPP4-I + IFALD model; short bowel syndrome (SBS) + total parenteral nutrition) groups. All rats were euthanized 21days postoperatively to obtain tissue samples. Liver fibrosis was evaluated by Sirius Red and α-SMA staining. Liver damage was assessed using the steatosis, activity, and fibrosis score. Inflammation cytokines were examined by ELISA. The survival rate was comparatively different, being 87.5% in the DPP4-I group and 70.0% in the Control group. Two rats of the Control group showed progressive liver fibrosis in the periportal area with fibrous streaks. Further, the mean area percentage of α-SMA immune-positive cells was significantly lower in the DPP4-I group than in the Control group. TGF-β levels were significantly lower in the DPP4-I group than in the Control group. DPP4-I administration reduced liver fibrosis in IFALD, possibly by inhibiting DPP4-I-induced adipogenesis and suppressing TGF-β. These results may contribute to elucidating the mechanism of IFALD.

Treatment of concomitant persistent chylothorax and superior vena cava syndrome through innominate vein-right atrial bypass.

Persistent chylothorax is a major challenge in paediatric patients. We present a case of a 6.5 kg, 1-year-old boy with superior vena cava syndrome and persistent chylothorax who underwent successful surgery without cardiopulmonary bypass. His medical history included multiple comorbidities such as myeloproliferative disease, short bowel syndrome and central vein catheterizations. The patient also had innominate vein thrombosis, progressing to superior vena cava, and was on anticoagulants. Despite dietary changes and somatostatin, his high-output chylous pleural effusion persisted. He was treated with innominate vein-to-right atrial bypass using a 6-mm Dacron graft. Postoperatively, there was a significant reduction in effusion and accelerated recovery. Somatostatin failure was likely due to mechanical obstruction of the thoracic duct.

A budget impact analysis of exclusive human milk diet in very low birth weight infants in United Arab Emirates

Introduction Very low birth weight (VLBW) infants have the highest rate of neonatal intensive care unit (NICU) admissions owing to the higher comorbidities associated with premature birth and long hospital stays. VLBW infants fed a bovine (BOV)-based diet have higher mortality rates, necrotizing enterocolitis (NEC), late-onset sepsis (LOS), and other comorbidities than those fed 100% human milk-based products. This study aims to evaluate the budgetary impact of adopting an exclusive human milk diet (EHMD) instead of a BOV-based diet in VLBW infants from Al-Ain Hospital, Sheikh Shakhbout Medical City, and Dubai Health Authority (DHA) in the United Arab Emirates (UAE). Methods We use a decision-tree model to estimate the budget impact of adopting EHMD versus the current local practice (BOV) over 5 years. Patients enter the tree model and have a probability of transitioning to one of the following mutually exclusive health states: NEC, which may be treated medically or surgically; LOS, NEC, and LOS; or neither NEC nor LOS. Depending on the feeding strategy, infants in the aforementioned health states are likely to develop any of the following complications: retinopathy of prematurity, bronchopulmonary dysplasia, or short bowel syndrome. The model accounts for the costs associated with diet, management of health states and complications, and the follow-up period. Results Al-Ain Hospital saved United Arab Emirates Dirham (AED) 3.3 million ($1.4million) in the first year of the EHMD feeding arm and AED 16.6 million ($7.1million) over the course of 5 years. In comparison to Sheikh Shabout Medical City and DHA, AED 36.7 million ($15.8million) and AED 24 million ($10.3million) were saved over 5 years, respectively, with AED 7.3 million ($3.1million) and 4.8 million ($2million) saved in the first year. The estimated pooled results across the three institutions were AED 5.1 million ($2.2million) and AED 25.7 million ($11million) savings in the first year and over 5 years, respectively. Conclusion Implementing the EHMD feeding scheme in VLBWs that has significant clinical benefits has resulted in substantial budget savings from the payer’s perspective in the UAE owing to fewer comorbidities associated with premature birth and shorter hospital stays. It is highly recommended for the Emirati health care settings to evaluate the real-world neonatal complication rates.

Voriconazole trough levels after switching from an intravenous to oral formulation in a patient with short bowel syndrome.

Voriconazole trough levels after switching from an intravenous to oral formulation in a patient with short bowel syndrome.

Important practical aspects in the management of patients with shot bowel syndrome and intestinal failure

Short bowel syndrome is a gastrointestinal disorder resulting from massive resection of the small intestine. The most common causes of resection are acute mesenteric ischemia, Crohn’s disease, abdominal trauma, adhesive intestinal obstruction, intestinal malignancies, and radiation enteritis. Short bowel syndrome with intestinal failure is characterized by severe malabsorption-m aldigestion accompanied by abdominal pain, diarrhea, dehydration, and progression of protein-energy malnutrition. Many patients with SBS develop long-term clinical complications over time due to altered bowel anatomy and physiology or various therapeutic interventions such as parenteral nutrition and central venous catheterization. This review focuses on the most important aspects of diagnosis, treatment, and prevention of several complications that can occur in this patient population, including macronutrient and micronutrient imbalances, diarrhea, fluid and electrolyte imbalances, metabolic bone disease, biliary disorders, bacterial overgrowth in the small intestine, and complications related to venous access. The main therapeutic strategies for SBS with intestinal failure are: special diet, enteral nutritional support with semi-elemental mixtures, parenteral nutrition, oral rehydration, antimicrobal and antidiarrheal drugs, enzymes. Administration of glucagon-like peptide 2 analog, teduglutide, is clinically effective in reducing dependence on parenteral nutrition and improving quality of life. Management of patients with SBS with intestinal failure is a complex long-term process which requires dynamic clinical and laboratory monitoring.

Cost-Effectiveness of Teduglutide for Pediatric Patients with Short Bowel Syndrome in Japan, Including Caregiver Burden.

Short bowel syndrome (SBS) is associated with a significant mental and physical burden for patients and caregivers. Standard of care (SOC) for SBS includes parenteral support (PS) to optimize intestinal function. Teduglutide, a recombinant human glucagon-like peptide2 analogue, reduces the need for PS in patients with SBS. In this study, we assessed the cost-effectiveness of teduglutide in pediatric patients with SBS from multiple perspectives, considering the caregiver's burden. A Markov model was used to evaluate cost (Japanese yen, JPY) and effectiveness (quality-adjusted life years, QALYs) of teduglutide compared with SOC for pediatric patients with SBS in Japan. We conducted a base-case analysis and selected sensitivity and scenario analyses from three perspectives: (1) the public healthcare payer, (2) the public healthcare and long-term care payer, and (3) society. In the base-case analysis, the incremental cost-effectiveness ratio (ICER) was 9,533,412 JPY per QALY from the public healthcare payer perspective, 6,335,980 JPY per QALY from the public healthcare and long-term care payer perspective, and 3,510,371 JPY per QALY from the societal perspective. The probability that cost-effectiveness of teduglutide is favorable from a societal perspective was 59.3%. In all scenario analyses, consistent with the base-case analysis, ICERs for teduglutide compared with SOC were different depending on whether caregiver utility and productivity loss were considered. Incorporating the caregiver's burden in the cost-effectiveness analysis of teduglutide for pediatric patients with SBS provided a more comprehensive assessment of the value of teduglutide for patients, their families, and society. This approach enhances our understanding of the overall value of a treatment, especially for diseases with significant caregiver burden.

Efficacy and safety of apraglutide in short bowel syndrome with intestinal failure and colon–in–continuity: A multicenter, open-label, metabolic balance study

BackgroundApraglutide is a novel long-acting GLP-2 analog in development for short bowel syndrome with intestinal failure (SBS-IF). This multicenter, open-label, phase 2 study in SBS-IF and colon-in-continuity (CiC) investigates the safety and efficacy of apraglutide. MethodsThis was a 52-week phase 2 metabolic balance study (MBS) in 9 adult patients with SBS-IF-CiC receiving once-weekly subcutaneous apraglutide injections. Safety was the primary endpoint. Secondary endpoints included changes in absorption parameters (MBS at baseline, after 4 weeks with stable parenteral support (PS), and 48 weeks), PS needs (48-week PS adjustment period based on monthly 48-hour fluid balances) and intestinal morphology and motility (static and cine MRI at baseline and 4, 24 and 48 weeks). ResultsPS volume decreased by -4702mL/week (-52%; p<0.001) at week 52. Seven patients (78%) achieved ≥1 day off PS at week 52. At 4 weeks, fecal output was reduced by 253 g/day (p=0.013). At 48 weeks, increases in wet weight absorption by 316 g/day (p=0.039), energy absorption by 1134 kJ/day (p=0.041) and carbohydrate absorption by 56.1 g/day (p= 0.024) were observed. Moreover, small bowel length increased from 29.7 to 40.7 cm (p=0.012), duodenal wall thickness increased by 0.8 mm (p=0.02) and motility in the proximal colon was reduced (p=0.031). A total of 127 adverse events was reported, which were mostly mild to moderate. ConclusionApraglutide had an acceptable safety profile and was associated with significant reductions in PS needs and days off PS, improvements in intestinal absorption, and structural and functional intestinal changes in patients with SBS-IF-CiC.ClinicalTrials.gov, Number NCT04964986

Ultrasound for infantile midgut malrotation: Techniques, pearls, and pitfalls.

Midgut malrotation with volvulus is a surgical emergency with potentially devastating outcomes which include short gut syndrome necessitating long-term parenteral nutrition, overwhelming sepsis, and death. The clinical presentation is most frequently with bilious vomiting in the first days-weeks of life, which is non-specific and common. Timely imaging investigation is therefore crucial to prevent delays to diagnosis and treatment and avoid unnecessary surgical exploration in infants with non-surgical bilious vomiting. Fluoroscopic upper gastrointestinal contrast series (UGI) has been the first-line imaging modality to investigate midgut malrotation at pediatric surgical centers worldwide. However, there is a growing body of evidence to indicate that ultrasound (US) has greater diagnostic accuracy than UGI in this context. Furthermore, US offers the benefits of accessibility, portability, lack of ionizing radiation, and the ability to identify alternative diagnoses, and is beginning to attract significant attention and consideration in the literature. Over the last 3years, we have transitioned to an "US-first" pathway for the investigation of midgut malrotation in infants with bilious vomiting. This pictorial essay illustrates our comprehensive approach, describes unique troubleshooting techniques, and highlights the variably published pitfalls we have encountered with the aim of encouraging wider adoption.