G47(P) Non-caseating splenic granulomas, as the first manifestation of very early-onset inflammatory bowel disease. (12th March 2018)

• Record Type: Journal Article
• Authors: Rajendran, AP; Mardare, R; Modi, A; Ashworth, M; Ramkumar, N; Rollins, H
• Is Part Of: Archives of disease in childhood. Volume 103(2018)Supplement 1
• Page Start: A19
• Page End: A19
• Publication Date: 2018-03-12
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2018-rcpch.45
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Background: Splenic granulomas (SG), are rare and a challenge in terms of diagnosis and treatment. There is no reported incidence for SG and they usually appear in young adults. Our patient presented with SG, as an extra-intestinal manifestation of Very Early-Onset Inflammatory Bowel Disease (VEO-IBD). Case report: We report the case of a 3 year old girl who developed multiple SG at the onset of Crohn's Disease (CD), making her, to our knowledge, the youngest patient reported to have this extra-intestinal manifestation of IBD. She presented with a history of recurrent episodes of fever, vomiting, abdominal pain, weight loss and mouth ulcers. No infectious aetiology was found and she had normal immunological workup. Ultrasound (USS) of the abdomen showed hypoechoic splenic lesions. Splenic biopsy was performed, as despite an 8 week course of antibiotics there was no resolution of her symptoms and USS findings. Splenic biopsy showed non-caseating granulomas. Her faecal calprotectin (FC) was raised, hence she had upper and lower gastrointestinal tract endoscopies and Video capsule endoscopy (VCE), which showed multiple ulcers throughout the small bowel, confirming a diagnosis of VEO-CD. Results: She was started on Modulen and Azathioprine, which improved her gut symptoms and the SG resolved on the USS. After the initial improvement her gut symptoms returned, so Infliximab was added to her management. Four months after commencing Infliximab, she started developing symptoms again and her repeat FC was elevated. Repeat endoscopies and MRI reconfirmed the diagnosis of active CD. Both Azathioprine and Infliximab doses were increased, with some improvement of her symptoms. But Infliximab was later changed to Adalimumab as the Infliximab levels were persistently low. With just one loading dose of Adalimumab, she developing beningn intracranial hypertension, hence was discontinued. She is currently on Thalidomide and Azathioprine, thriving well, while being monitored with regular abdominal ultrasound. Conclusion: Patients with VEO-IBD usually do not respond to first line treatment, therefore biologics are often used early on in this disorder. Further studies on VEO-IBD need to be undertaken to help us manage this unusual disease entity.