PAEDIATRIC GENITAL CROHN'S DISEASE: A COMBINED CASE SERIES.
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All Authors
Kontaki, E.
Sethuraman A.
Gaynor E.
Zamvar V.
Kiparissi F.
LTHT Author
Zamvar, V.
LTHT Department
Leeds Children's Hospital
Non Medic
Publication Date
2024
Item Type
Conference Abstract
Language
Subject
Subject Headings
Abstract
Metastatic Crohn's disease (MCD) of the genitalia is a rare extraintestinal manifestation of Crohn's disease characterized by granulomatous inflammation of the genital skin without contact with the gastrointestinal tract (Dederichs F et al, 2018). Only few paediatric cases have been reported in the literature with scanty data with regards to treatment and outcome (Granese R et al, 2018). To describe the clinical presentation, associated features, and response to treatment in a cohort of paediatric patients diagnosed with genital metastatic Crohn's disease. Cases of metastatic genital Crohn's disease were retrospectively collected from two tertiary centers between 2001 and 2023. A total of 12 patients (7 males, 5 females) were identified with metastatic genital Crohn's disease, mostly presented as genital oedema. The mean age at the time of metastatic genital Crohn's disease occurrence was 8.9 years with a range of 1.5 years to 14 years. Genital Crohn's disease was the first presentation of inflammatory bowel disease in 58% of the cases. Genital symptoms precede the onset of gastrointestinal disease in 33% of the cases whereas gastrointestinal disease presented in 42% of the cases prior to the onset of genital Crohn's disease. Perianal disease was documented in 50%. Genital Crohn's disease occurred in the absence of gastrointestinal disease in 25% of the cases. All patients underwent endoscopic and radiologic assessment. Histological confirmation of granulomas was obtained in 50% of the cases. Treatment of the patients included prednisolone, azathioprine, methotrexate, mesalazine, ciprofloxacin, metronidazole, topical tacrolimus, intralesional triamcinolone and anti-TNFa inhibitors. Eleven of twelve patients (92%) found clinical benefit of MCD from systemic anti-TNFa inhibitors monotherapy or anti-TNFa inhibitor and azathioprine combination therapy. Metastatic Crohn's disease of the genitalia is rare. It may be the first presentation of Crohn's disease and not necessarily associated with gastrointestinal disease. Perianal disease is often present. Anti-TNFa inhibitors have exhibited a favorable response. Multicentre retrospective case series analysis would help to identify the prevalence of genital metastatic disease and formulate an integrated diagnostic and management approach.
Journal
Frontline Gastroenterology