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Background Vedolizumab has proven efficiency in adults but data in paediatric inflammatory bowel disease (pIBD) is limited. We present the outcome of treatment with vedolizumab in refractory pIBD cohort. Study Design Retrospective and ongoing prospective review of all patients commenced on Vedolizumab following loss of response to anti-tumour necrosis factor [TNF] between Nov 2017 and Nov 2020. Aims and Objectives The primary outcome was remission at Week 14 and last follow up (wPCDAI/PUCAI Results 11 children received vedolizumab (6[54%] males), mean age at time of diagnosis 12.45(8.34–15.48) with a median(IQR) time from diagnosis of 2.68(1.79–5.28)years; 6[54%] Crohn’s disease [CD] and 5[46%] Ulcerative colitis/IBD Unclassified [UC/IBDU] (table 1). For CD; 5/6 was treated previously with anti-TNF [40% primary failure, 60% secondary failure], all had colonic disease, 3/6 upper GI involvement and 3/6 perianal disease. One child with Bruton’s agammaglobulinemia was anti-TNF naive when commenced on vedolizumab. All UC children were treated with anti-TNF [40% primary failure, 60% secondary failure], 80% had pan-colitis. Median age at time of commencing vedolizumab (V0) was 14.99(13.0–17.6). Baseline characteristics at V0; faecal calprotectin(FC) 2851(92–6000), Hb 114(96–146), ESR 22(4–90), albumin 39(27–46) and CRP 16.7(4–39.5). 4/11(36%) required surgery, three of whom had colectomy. 8/11 remained on immunomodulators with vedolizumab. Transient raised transaminases and eczema was reported once and low mood with suboptimal response noted once. 6/11(54%) were in remission 14 weeks from commencing vedolizumab (V14) and 4/11(36%) were excluded. At last follow up from commencing vedolizumab (VF), median years 2.21(0.78–3.43), 3 remained in remission. In CD cohort, one child had a defunctioning ileostomy and remained in steroid free remission (SFR) at V14 and VF (3.43 years) on vedolizumab monotherapy. One had colectomy (FC-3296 wPCDAI-60), steroid dependency compounded by methotrexate induced interstitial nephritis and vedolizumab was discontinued at VF (2.19 years). Two continue to have active disease at V14 after commencing vedolizumab. One had SFR at V14 and was transitioned at 2 years (FC-2585, wPCDAI-25) on vedolizumab. One with anti-TNF resistant disease, achieved clinical remission 9 months after starting vedolizumab (wPCDAI 2.5, FC 598) before being transitioned. In UC cohort, two had vedolizumab primary non-response needing subtotal colectomy. One patient with PUCAI 5 at V14 needed regime intensification for low vedolizumab levels but had active disease (PUCAI-25, FC-366) when transitioned at VF (2.06 years). One patient, who achieved remission whilst on steroid at V14, remains in SFR at VF (0.58 years) on concomitant immunomodulation and optimal vedolizumab level at end of induction (>19). One who was lost to follow-up during COVID, was transitioned on 4 weekly vedolizumab regime. Conclusion At V14, 54% of patients achieved clinical remission and we see significant improvement with PUCAI/PCDAI scores and faecal calprotectin in both UC and CD cohort. We are continuing this study over a longer period to achieve a larger cohort.

Introduction Glucocorticoids (GCs) are used in all forms of paediatric inflammatory bowel disease (IBD) for their anti-inflammatory and immunosuppressive effect. Prolonged GC treatment may suppress the hypothalamic-pituitary-adrenal (HPA) axis causing secondary adrenal suppression (SAS), despite using a weaning regime, necessitating the use of hydrocortisone for replacement. The incidence and severity of SAS is poorly predictable and may be related to both the cumulative dose of GCs, but also to individual factors. Standard and low dose short Synacthen tests (SST, LDSST) are used to assess recovery of the HPA axis following a prolonged course of steroids. Aims To report: the incidence of SAS following GC treatment for IBD in our centre the time to HPA axis recovery in patients with SAS risk factors for SAS Patients and Methods 33 children with IBD (19M, 10–18 years) previously treated with GCs, who had been investigated for SAS from 01/01/2017 and 30/10/2020 were identified. Baseline information including age, sex, somatometric parameters, IBD diagnosis, age at diagnosis and maintenance treatment were collected. All their GC courses (n=47) in the defined period were reviewed, and those that led to SAS were compared for length and GC doses, previous exposure to GC, tests for HPA axis recovery and the requirement for hydrocortisone replacement therapy. Results Of the 33 children, 15/33 (45.5%) had ulcerative colitis (UC), 10/33 (30.3%) had Crohn’s Disease (CD) and 8/33 (24.2%) were unclassified (IBDU). 10 children that were tested using non-standard tests for adrenal suppression were excluded. 23 children had their HPA axis recovery tested after 24 GC courses with LDSST (19/24, 79.2%) or SST (5/24, 20.8%). 16/23 children had steroid courses of ≥12 weeks, and 7/23 had a shorter course ( The mean duration of courses that induced SAS was 154± 86.8 days compared to 131.6±63 days in those without SAS, t=0.44, p=0.33, and the previous days of exposure were 219±95 days for those with SAS compared 316.5±154.2 days for those without SAS, t=-1.2, p=0.13. High dose IV methylprednisolone (10–20 mg/kg) was used in 5/24 (20.8%) courses with 5/5 (100%) and 3/5 (60%) inducing HPA axis suppression on the first testing and retesting respectively. Standard dose (1.6 mg/kg) IV methylprednisolone followed by oral weaning GC course was used in 9/24 (37.5%) courses and 5/9 (55.6%) induced HPA axis suppression. Standard dose oral prednisolone (1–2 mg/kg) was used in 10/24 (41.7%) of the courses and 5/10 (50%) induced SAS. Conclusion SAS was detected in 60.9% of our IBD patients who were tested after GCs therapy. Our data support previous reports that additional factors to the duration of treatment with GCs, as the use of high dose of intravenous GCs, may influence the risk of SAS.

Background Anaemia is the most common extra intestinal manifestation in paediatric Inflammatory Bowel Disease (pIBD) needing monitoring and supplementation. Intravenous iron is often needed in moderate to severe disease or intolerance to oral iron. Ferric Carboxymaltose (FCM) infusion has been used in our centre. While the risk of hypophosphatemia (HP) associated with FCM is known, (Medicines and Healthcare Products Regulation Agency alert), it is not been quantified in clinical practice. Aims and Objectives Primary objectives’ to report incidence and severity of HP after infusion of FCM in pIBD. Assess time to recovery from HP with interventions. Secondary objectives’ to review patient characteristics and biochemical markers to identify risk factors for FCM infusions associated HP. Design study We conducted a retrospective electronic records review of pIBD patients who received FCM infusion from November 19-November 20. Results 24 patients (13 M) received 26 infusions in the period reviewed. The median age was 14.5(12.6–15.9). From all the children, 7(29.2%) were diagnosed with Ulcerative Colitis (UC), 16(66.7%) with Crohn’s Disease and 1(4.2%) with IBD-Unclassified (IBDU). The timing of infusion coincided with: admitted with new diagnosis of pIBD, [10(41.7%)], flare of disease [9(37.5%)] and elective admission [5(20.8%)] for correction. Median dose for the 26 infusions was 1000(500–1000)mg. Pre-infusion median (IQR) haemoglobin 107.5 (92.7–119.2), MCV 81(74.7–83.2), iron 3.4(2.1–5.5), ferritin 73.1(33.2–115.7) and transferrin saturation 4.85(3.6–10.0)%. All had normal renal function; median(IQR) urea 2.9(2.32–3.35) and creatinine, 50.5(37–61.7). Pre-transfusion Vitamin D (n=19) median was 38.5(27.5–53.5), with levels being deficient in 13(68.3%) with no association to recovery time. The biochemical markers are shown in table 1 below, with the change shown as delta change. The delta change was found to be statistically significant for serum phosphate levels. There were no statistically significant association, on univariate analysis, between the delta change in serum phosphate levels and the features of patient demographics or biochemical markers. All 24 patients had reduction in phosphate level post FCM infusion. In 14/26(53%), phosphate levels dropped to moderate-severe range; 10/14(71.4%) HP was moderate ( No serious sequelae of hypophosphatemia were seen. Treatment included a combination of IV phosphate infusion (6,25%), oral phosphate supplements (12,50%), oral calcium supplements (15,62.5%) and vitamin D supplements (17,70.8%). Conclusion HP is frequently seen with FCM infusion. The fall in phosphate post FCM infusion was found to be clinically and statistically significant; though none of the patient demographic features or serological markers were found to be associated with the delta change in phosphate to predict high risk patients. The median recovery time of 14 days is less than what is reported in adult reviews. FCM infusions need pre-assessment, counselling and post infusion monitoring to assess effectiveness and recovery.

Introduction Children with Peutz-Jeghers syndrome (PJS) are at high risk of intussusception and bowel resection from small intestinal polyp formation. Little data has been published on early warning signs and prevention. We contributed to recent ESPGHAN guidelines, which recommend to start screening investigations from the age of 8 years in 3-yearly intervals. In spite of meticulous endoscopic and imaging screening, three of our patients developed intussusceptions. Aim To identify patient characteristics and polyp features in PJS patients with intussusceptions compared to patients without this complication. To review if a more cautious surveillance protocol may be beneficial for children with PJS considered being at increased risk. Subjects and Methods All patients were confirmed to have PJS by genetic analysis (STK 11 mutation). Patient A underwent endoscopic surveillance every 2–3 years. At 9 years video capsule endoscopy (VCE) revealed one stalked small polyp and some areas of fresh blood in the small bowel, so double-balloon-enteroscopy (DBE) performed and two polyps removed. 4 years later the patient presented with sharp abdominal pain for two weeks associated with a mass in LIF, reduced appetite and required semi-urgent laparoscopic resection. Patient B was diagnosed de novo at the age of 11 years due to lip and mucosal freckling. In addition to upper and lower GI endoscopy, small bowel imaging was booked but patient did not adhere to recommendations. Following pathological VCE, urgent booking for DBE was made but postponed by the family. The child presented then acutely with small bowel intussusception, underwent an urgent laparotomy and resection. Patient C was diagnosed at the age of 8 years, presenting with anaemia, acute abdominal pain and non-bilious vomiting. An urgent ultrasound abdomen revealed small bowel intussusceptions, leading to laparotomy and resection. Intraoperative enteroscopy was performed with removal of two further polyps 10-15 cm from the resected bowel. Results Review of 7 PJS patients without intussusceptions during the same 10 year period did not demonstrate significant differences in age of presentation, presence of polyps in stomach, or duodenum. Regarding small intestinal polyp formation, 2 of control patients had small intestinal polyps, one of them referred for DBE and the other one did not require DBE resection. Summary and Conclusion In our cohort of children with PJS, presence of small intestinal polyps was the only risk factor for intussusceptions. Intussusceptions occurred in 5/10 of all children with small intestinal polyps. Dynamics of small intestinal polyps appear variable, but occurred earlier or in shorted intervals than recommended by ESPGHAN guidelines. Although small bowel investigations can be difficult for some children with PJS, we recommend that they should be performed not later than 8 years of age. Due to absence of early warning signs for intussusceptions, if small intestinal polyps are found, small bowel imaging under optimal imaging conditions should be repeated annually and also immediately when children are symptomatic. Centres are encouraged to enroll patients in the upcoming ESPGHAN polyposis group PJS registry.

Introduction/Background Chronic constipation has been shown to lead to poor school performance and consequently deficiencies in education, as well as poor health-related quality of life. In children who suffer from chronic constipation, colonic transit studies (CTS) are ordered by specialist services to provide information that aids clinical management decisions Aim The aim of this audit was to evaluate the impact of CTS outcomes on clinical management decisions involving patients with chronic constipation. It also looked at the radiology reports of included transit studies, specifically at whether they included the number and location of radio opaque markers. The NICE guideline ‘Constipation in children and young people: diagnosis and management’ and The Royal College of Radiologists audit template ‘Complete reporting of colonic transit marker studies’ were used to determine best practice. Subjects and Method A retrospective audit looking at the list of patients with chronic constipation who underwent CTS at Alder Hey Children’s Hospital. Working backwards from November 2019, the first 100 patients who met inclusion criteria were selected. Included patients had to best knowledge conducted CTS in full and also had a clinic letter following completion of the study. Management outcomes were grouped into 4 categories: decrease, no change to management, an increase of oral laxatives or an increase using management stronger than oral laxatives e.g. rectal medications or surgical interventions. Results The majority of included transit studies were requested by either paediatric surgery (n=71) or gastroenterology (n=20). Only 60% of CTS reports included both the number and location of markers and 13% included neither. There was a mean of 8 days from transit study to radiology report completion. The mean transit time was 72 hours, with a range of 0–144 hours. Management outcomes were varied for both normal and slow transit. Twice as many patients with slow transit were managed with therapies stronger than oral laxatives. Patients with normal transit time were over twice as likely to have no change to their management. A transit time of >100 hours resulted in almost 80% of patients being managed with treatment stronger than oral laxatives. Summary and Conclusion There appears to be a trend towards escalating management with intensive combination treatment regimes in patients whose CTS suggested slow transit and especially in patients with transit times greater than 100 hours. The range of the management choices used in patients with normal transit do however illustrate that clinicians within Alder Hey are making clinical decisions based upon the wider clinical picture of the patient, which fits with NICE guidance. This audit does illustrate that CTS radiology reports can be adapted to ensure each report contains the number and location of markers. Recommendations All CTS radiology reports should include the number and location of radio opaque markers. The location of markers should be reported into 3 regions (right colon, left colon and rectosigmoid colon) as suggested. A proforma has been distributed within the Alder Hey radiology department detailing results and recommendations. A re-audit to assess the application of these recommendations is currently underway.

BackgroundCOVID-19 has impacted on healthcare provision. Anecdotally, investigations for children with inflammatory bowel disease (IBD) have been restricted, resulting in diagnosis with no histological confirmation and potential secondary morbidity. In this study, we detail practice across the UK to assess impact on services and document the impact of the pandemic.MethodsFor the month of April 2020, 20 tertiary paediatric IBD centres were invited to contribute data detailing: (1) diagnosis/management of suspected new patients with IBD; (2) facilities available; (3) ongoing management of IBD; and (4) direct impact of COVID-19 on patients with IBD.ResultsAll centres contributed. Two centres retained routine endoscopy, with three unable to perform even urgent IBD endoscopy. 122 patients were diagnosed with IBD, and 53.3% (n=65) were presumed diagnoses and had not undergone endoscopy with histological confirmation. The most common induction was exclusive enteral nutrition (44.6%). No patients with a presumed rather than confirmed diagnosis were started on anti-tumour necrosis factor (TNF) therapy.Most IBD follow-up appointments were able to occur using phone/webcam or face to face. No biologics/immunomodulators were stopped. All centres were able to continue IBD surgery if required, with 14 procedures occurring across seven centres.ConclusionsDiagnostic IBD practice has been hugely impacted by COVID-19, with >50% of new diagnoses not having endoscopy. To date, therapy and review of known paediatric patients with IBD has continued. Planning and resourcing for recovery is crucial to minimise continued secondary morbidity.

Introduction: Paediatric Crohn’s disease (PCD) often presents with extensive and a frequent pan-enteric phenotype at onset. However, its long term evolution into adulthood, especially since the wid...

Isolated fetal ascites was diagnosed at 20 weeks in a primiparous woman with no significant medical history. Progressive fetal ascites worsened after 28 weeks and resulted in fetal hydroceles. Delivery was by caesarian section at 33 weeks, preceded by reduction of fetal ascites under ultrasound guidance. Following delivery, the baby required further reduction of abdominal fluid and endotracheal intubation to provide respiratory support. An extensive set of investigations, including metabolic and genetic screening, was performed; all results were negative. On day two of life, the baby developed bilious aspirates and an abdominal radiograph suggested intestinal obstruction. At laparotomy, an ‘apple peel’ jejunal atresia, abnormal mesentery with precarious blood supply and a proximal perforation were identified and the perforation ‘sewn over’. The postoperative course was unremarkable, with Monogen feeds tolerated three weeks later. The baby continued to thrive at one year, tolerating increasing amount of long-chain fatty acids in diet.

A 12-year-old girl with Smith-Lemli-Opitz syndrome and gastrostomy dependency presented with multiple episodes of coffee ground vomits. An upper gastrointestinal endoscopy revealed a trichobezoar in the lower oesophagus, with a ‘hidden treasure’—a retained end of a G tube at the core. Endoscopic retrieval led to resolution of symptoms. Literature is scant with only one previous report of an oesophageal trichobezoar. Techniques of removal of percutaneous endoscopic gastrostomy in children are reviewed. The pathogenesis, preventative measures and management for oesophageal trichobezoars are discussed.