Inflammatory Cutaneous Lesions in Inflammatory Bowel Disease Treated With Vedolizumab or Ustekinumab: An ECCO CONFER Multicentre Case Series.

This was a multicentre case series supported by the European Crohn's and Colitis Organisation [ECCO] and performed as part of the Collaborative Network of Exceptionally Rare case reports [CONFER] project. The aim was to report on whether cutaneous lesions associated with inflammatory bowel disease [IBD] and refractory to standard medical therapy including anti-tumour necrosis factors [anti-TNFs], would respond to the newer biologic agents ustekinumab [UST] or vedolizumab [VDZ]. This report includes 28 patients with cutaneous lesions from 14 centres, all of whom had failed immunomodulator and anti-TNF therapy. Metastatic Crohn's disease [MCD] was diagnosed in 10 patients: UST led to remission in five cases and partial response in four cases, with a single report of VDZ inducing remission. All cases of MCD treated with UST responded after the first or second dose, and the median time for the five cases that attained remission was 5 months. Pyoderma gangrenosum [PG] was diagnosed in four cases: three of these attained remission with UST [median time to remission 4 months] and one case did not respond to VDZ. There were seven cases of erythema nodosum [EN]: UST led to remission in four cases and partial response in 1 case whilst VDZ had partial response in 2 cases and non-response in two cases. There were seven single cases of other inflammatory lesions. In summary, UST appears to be useful for different cutaneous lesions including MCD, PG, and EN, whereas VDZ does not appear to be useful for lesions that are independent of disease activity.

Applications of wireless capsule endoscopy in pediatric age: an update.

BACKGROUND: The small bowel has often been considered the mysterious "black hole" of the gastrointestinal tract. With regards to this, the development of the wireless capsule endoscopy (WCE) has represented a turning point. It is a non-invasive technique, enabling an excellent visualization of the small bowel (SB) mucosa without the use of radiation. The WCE was approved by the Food and Drug Administration (FDA) in 2001 for adults and in 2004 for children. The aim of the present review is to provide an update on indications, diagnostic yield, safety and limitations of WCE in children. Even though literature regarding the use of WCE in pediatric age is more limited than in adults, WCE is a useful and safe diagnostic tool for the exploration of the small bowel also in children. The indications for WCE are similar at any age, however the main indication in children is Crohn's disease (CD), while in the adults is the research of SB bleeding. The main limitation in pediatric age is the possibility for younger children to swallow the capsule. WCE in pediatric is a rapidly advancing technology and has the potential to further transform the evaluation and management of SB disease.

Hemolytic uremic syndrome: differential diagnosis with the onset of inflammatory bowel diseases.

BACKGROUND: Shiga-toxin Escherichia coli productor (STEC) provokes frequently an important intestinal damage that may be considered in differential diagnosis with the onset of Inflammatory Bowel Disease (IBD). The aim of this work is to review in the current literature about Hemolytic Uremic Syndrome (HUS) and IBD symptoms at the onset, comparing the clinical presentation and symptoms, as the timing of diagnosis and of the correct treatment of both these conditions is a fundamental prognostic factor. A focus is made about the association between typical or atypical HUS and IBD and a possible renal involvement in patient with IBD (IgA-nephropathy). METHODS: A systematic review of scientific articles was performed consulting the databases PubMed, Medline, Google Scholar, and consulting most recent textbooks of Pediatric Nephrology. RESULTS: In STEC-associated HUS, that accounts for 90% of cases of HUS in children, the microangiopathic manifestations are usually preceded by gastrointestinal symptoms. Initial presentation may be considered in differential diagnosis with IBD onset. The transverse and ascending colon are the segments most commonly affected, but any area from the esophagus to the perianal area can be involved. The more serious manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis and intussusception. Severe gastrointestinal involvement may result in life-threatening complications as toxic megacolon and transmural necrosis of the colon with perforation, as in Ulcerative Colitis (UC). Transmural necrosis of the colon may lead to subsequent colonic stricture, as in Crohn Disease (CD). Perianal lesions and strictures are described. In some studies, intestinal biopsies were performed to exclude IBD. Elevation of pancreatic enzymes is common. Liver damage and cholecystitis are other described complications. There is no specific form of therapy for STEC HUS, but appropriate fluid and electrolyte management (better hyperhydration when possible), avoiding antidiarrheal drugs, and possibly avoiding antibiotic therapy, are recommended as the best practice. In atypical HUS (aHUS) gastrointestinal manifestation are rare, but recently a study evidenced that gastrointestinal complications are common in aHUS in presence of factor-H autoantibodies. Some report of patients with IBD and contemporary atypical-HUS were found, both for CD and UC. The authors conclude that deregulation of the alternative complement pathway may manifest in other organs besides the kidney. Finally, searching for STEC-infection, or broadly for Escherichia coli (E. coli) infection, and IBD onset, some reviews suggest a possible role of adherent invasive E. coli (AIEC) on the pathogenesis of IBD. CONCLUSIONS: The current literature shows that gastrointestinal complications of HUS are quite exclusive of STEC-associated HUS, whereas aHUS have usually mild or absent intestinal involvement. Severe presentation as toxic megacolon, perforation, ulcerative colitis, peritonitis is similar to IBD at the onset. Moreover, some types of E. coli (AIEC) have been considered a risk factor for IBD. Recent literature on aHUS shows that intestinal complications are more common than described before, particularly for patients with anti-H factor antibodies. Moreover, we found some report of patient with both aHUS and IBD, who benefit from anti-C5 antibodies injection (Eculizumab).

Eosinophilic esophagitis in pediatric age, state of the art and review of the literature.

Eosinophilic esophagitis (EoE) is a chronic immune-mediated relapsing disease caused by eosinophilic infiltration of the esophageal mucosa which is normally lacking these cells. EoE belongs to the group of the so called Eosinophilic Gastrointestinal Disorders (EGIDs). From a rare and unusual disease, EoE has become an emerging entity and in recent years its incidence and prevalence have increased all over the world, also in children. The pathogenesis is very complex and still not completely clear. Esophageal disfunction symptoms (e.g. dysphagia and food impaction) represent the typical manifestation of EoE and this condition could be difficult to recognize, more in pediatric age than in adults. Moreover, symptoms can often overlap with those of gastro-esophageal reflux disease (GERD), leading to a delayed diagnosis. EoE is often related to atopy and an allergological evaluation is recommended. Untreated EoE could provoke complications such as strictures, esophageal rings, narrowing of the esophagus. Diagnosis is confirmed by the demonstration in biopsy specimens obtained through upper endoscopy of eosinophilic inflammation (>15 for high powered field) of the esophageal mucosa and other histological features. Other tests could be useful not specifically for the diagnosis, but for the characterization of the subtype of EoE. Since EoE incidence and knowledge about physiopathology and natural history have increased, the goal of the review is to provide some helpful tools for the correct management in pediatric age together with an overview about epidemiology, pathogenesis, clinical, diagnosis and treatment of the disease.

Risk of tumor implantation in percutaneous endoscopic gastrostomy in the upper aerodigestive tumors.

Percutaneous endoscopic gastrostomy (PEG) has become a mainstay in providing enteral access for patients with obstructive head, neck and esophageal tumors. Tumor cell implantation is a rare complication in patients with aerodigestive cancers, who have undergone PEG tube placement. The objective of this review is to determine the incidence and contributing risk factors leading to the implantation of metastases into the abdominal wall following PEG placement. A comprehensive review of the literature in PUBMED (2008-2018) was performed.  The literature search revealed reports of more than 50 cases of abdominal wall metastases after PEG placement. As most of these studies were case reports, the exact rate of metastasis remains unknown. Generally pharyngoesophageal location of primary cancer (100%), squamous cell histology (98%), poorly differentiated tumor cells (92%), advanced pathological stage (97%), and large primary cancer size were identified as strong risk factors for the development of stomal metastasis. Abdominal wall metastases following PEG placement are a rare but serious complication in patients with head and neck malignancy.

Short- and long-term efficacy of endoscopic balloon dilation in Crohn's disease strictures.

AIM: To evaluate short- and long-term efficacy of endoscopic balloon dilation in a cohort of consecutive patients with symptomatic Crohn's disease (CD)-related strictures. METHODS: Twenty-six CD patients (11 men; median age 36.8 year, range 11-65 years) with 27 symptomatic strictures underwent endoscopic balloon dilation (EBD). Both naive and post-operative strictures, of any length and diameter, with or without associated fistula were included. After a clinical and radiological assessment, EBD was performed with a Microvasive Rigiflex through the scope balloon system. The procedure was considered successful if no symptom reoccurred in the following 6 mo. The long-term clinical outcome was to avoid surgery. RESULTS: The mean follow-up time was 40.7 ± 5.7 mo (range 10-94 mo). In this period, forty-six EBD were performed with a technical success of 100%. No procedure-related complication was reported. Surgery was avoided in 92.6% of the patients during the entire follow-up. Two patients, both presenting ileocecal strictures associated with fistula, failed to respond to the treatment and underwent surgical strictures resection. Of the 24 patients who did not undergo surgery, 11 patients received 1 EBD, and 13 required further dilations over time for the treatment of relapsing strictures (7 patients underwent 2 dilations, 5 patients 3 dilations, and 1 patient 4 dilations). Overall, the EBD success rate after the first dilation was 81.5%. No difference was observed between the EBD success rate for naive (n = 12) and post-operative (n = 15) CD related strictures (P > 0.05). CONCLUSION: EBD appears to be a safe and effective procedure in the therapeutic management of CD-related strictures of any origin and dimension in order to prevent surgery.

Cystic fibrosis and Crohn's disease: successful treatment and long term remission with infliximab.

The association of cystic fibrosis and Crohn's disease (CD) is well known, but to date, there are very few cases in the literature of patients suffering from mucoviscidosis who have required treatment with infliximab. We report the case of a 23-year-old patient suffering from cystic fibrosis and severe CD treated successfully with infliximab without any infective complications or worsening of the pulmonary disease and with a long term (2 years) complete remission.