Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular malformation disorder with cutaneous and visceral lesions frequently associated with serious, even fatal bleeding and anemia. The syndrome is considered to be autosomaly predominantly inherited. Intra-operative enteroscopy (IOE) is the best method of identification of all lesions (particularly the small ones, less than 3 mm) and treatment by endoscopic electro-coagulation or surgical excision. Capsule wireless endoscopy is optimal for screening before the IOE and for monitoring the effect of therapy (in patients with BRBNS). We report two cases of BRBNS. Anemia, gastrointestinal bleeding, gastrointestinal malformations and multifocal venous malformations of the skin were present in both of our cases. Gastrointestinal lesions were identified by gastroscopy, colonoscopy and capsule endoscopy. The multiple venous malformations were treated partly by endoscopic electro-coagulation (lesions up to 4 mm in diameter) and by wedge resection. Both of our patients were 12-year-old girls at the time of operation. In the first patient 31 venous malformations of the small bowel were coagulated, two were resected by the surgeon. In the second patient 20 lesions were coagulated endoscopically and another 31 nevi were resected during an 8 h procedure. The first girl is doing fine 4 years after the procedure, the second was allowed home 2 weeks after the procedure in excellent condition. IOE is a unique method of small bowel investigation and concurrently provides a solution for pathological findings. Capsule endoscopy is a feasible non-invasive screening procedure. We believe that a radical eliminatory approach by means of combined surgery and IOE is indicated for the BRBNS to prevent ongoing gastrointestinal bleeding.

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Background Mucosal healing (MH) has become a perspective treatment target in patients with Crohn’s disease (CD). Data about the impact of MH on long-term outcome in pediatric patients are still scarce. Methods 76 pediatric patients with CD were evaluated retrospectively (2000–2015) in a tertiary care center. Based on MH achievement, they were divided into two groups (MH, n = 17 and No MH, n = 59). The primary endpoint was to assess the association of MH and the need for CD-related hospitalizations or surgery in pediatric patients with CD. Results The number of hospitalized patients was 24% in the MH group and 42% in the No MH group, P = 0.26. The total number of CD-related hospitalizations was not significant between the MH group and the No MH group (5 vs. 41, P = 0.15). The time to the first hospitalization was 24 months in MH and 21 months in No MH, p > 0.99. 24% patients in the MH group and 39% patients in the No MH group underwent CD-related operation, P = 0.39. Time to the first operation was 43 months for MH and 19 months for the No MH group, P = 0.13. The follow-up period was 91 months in the MH group and 80 months in the No MH group, P = 0.74. The use of infliximab was positively associated with MH, P = 0.002. Conclusions MH was not associated with fewer CD-related hospitalizations or operations in pediatric patients with CD during seven years of follow-up.

BackgroundMucosal healing (MH) has become a perspective treatment target in patients with Crohn's disease (CD). Data about the impact of MH on long-term outcome in pediatric patients are still scarce. Methods 76 pediatric patients with CD were evaluated retrospectively (2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015) in a tertiary care center. Based on MH achievement, they were divided into two groups (MH, n = 17 and No MH, n = 59). The primary endpoint was to assess the association of MH and the need for CD-related hospitalizations or surgery in pediatric patients with CD. ResultsThe number of hospitalized patients was 24% in the MH group and 42% in the No MH group, P = 0.26.The total number of CD-related hospitalizations was not signi cant between the MH group and the No MH group (5 vs. 41, P = 0.15). The time to the rst hospitalization was 24 months in MH and 21 months in No MH, p > 0.99. 24% patients in the MH group and 39% patients in the No MH group underwent CDrelated operation, P = 0.39. Time to the rst operation was 43 months for MH and 19 months for the No MH group, P = 0.13. The follow-up period was 91 months in the MH group and 80 months in the No MH group, P = 0.74. The use of in iximab was positively associated with MH, P = 0.002. ConclusionsMH was not associated with fewer CD-related hospitalizations or operations in pediatric patients with CD during seven years of follow-up.