Question: A 17-year-old boy with high risk acute lymphoblastic leukemia, post bone marrow transplant (BMT), presented with 6 months of progressively worsening diarrhea and weight loss. He was treated for skin graft-versus-host disease (GVHD) 2 months earlier, and had also been treated twice for clostridium difficile infections. He was admitted to hospital with lower abdominal pain that was severe enough to require narcotics. In hospital, he was passing up to 10 loose nonbloody stools per day and vomiting 3-4 times a day (nonbilious). These symptoms were limiting his oral intake and he was put on continuous nasogastric tube feeds. There was no history of fever, travel or sick contacts.On examination he was hemodynamically stable. His abdomen was diffusely tender to palpation, with normal bowel sounds, no peritoneal signs and no organomegaly. The perianal examination was normal. He also had a generalized erythematous sand-paper-like rash.Laboratory tests showed a normocytic normochromic anemia (hemoglobin 88 g/L), mild leukopenia, and mild thrombocytopenia. Albumin was 25 g/L. His electrolytes, creatinine, and liver panel were normal. Inflammatory markers had not been done on admission. His stool test came back positive again for Clostridium difficile.He was treated for C difficile, initially receiving oral vancomycin, after which IV metronidazole was added. Despite appropriate management for the c. difficile, there was no improvement in the diarrhea or pain. Upper endoscopy and flexible sigmoidoscopy were therefore done for further assessment.What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.

Background Very-early-onset inflammatory bowel disease (VEOIBD) refers to an IBD diagnosis established before the 6th year of life, including a subset of patients with disease onset before the age of 2 years, known as infantile-onset IBD (IO-IBD) (1). VEO-IBD accounts for 15% of pediatric IBD and infantile IBD in approximately 1% (2,3). VEOIBD is considered to be a unique entity, and compared to adults with IBD, VEO-IBD children are more likely to present with extensive and treatment-resistant disease (4). Aims To analyze the clinical characteristics and management of patients diagnosed with very early onset inflammatory bowel disease (VEO-IBD). Methods A retrospective analysis of children diagnosed with VEO-IBD (age <6 years) at the Montreal Children’s Hospital from 2003–2018 was performed. Clinical data for VEO-IBD patients was collected to identify the clinical, biochemical, endoscopic and histological features of these patients and their clinical course until 2018. Results A total of 28 VEO-IBD patients (71% male) were included in this study. The median age of disease onset was 52 months. A diagnosis of Crohn’s disease (CD) or CD-like intestinal manifestations accounted for 89% of the VEO-IBD cases. Most patients had Crohn’s colitis (36%) of whom 50% had evidence of granulomatous Crohn’s disease; 11 patients[NAD1] [AQAB2] (39%) had upper gastrointestinal involvement. Over their progress of the disease, 4 patients (14%) required surgical intervention, while 11 patients (39%) required biologic therapy for maintenance therapy. Genetic[NAD3] [AQAB4] results were available for 5 patients out of 28 (18%) and 3 of them were identified to have monogenic IBD. Conclusions In our center, the majority of patients with VEO-IBD had typical Crohn’s disease presentation. Most of the VEO-IBD patients responded well to the standard IBD treatment. Genetic studies were not done regularly for all of our patients, however, among those who had this testing performed 3/5 had an identifiable cause, suggesting that these patients should be investigated for an underlying genetic abnormality. Funding Agencies None

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