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Distinct phenotypes of children with perianal perforating Crohn's disease
Scott S Short1, Marla C Dubinsky, Shervin Rabizadeh
1Division of Pediatric Surgery, Department of Surgery, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA.
Insights
Children with Crohn's disease (CD) who develop perianal perforating disease (PF) during therapy face a more severe condition. These patients are more likely to need surgery, such as diverting ileostomy or colectomy, compared to those diagnosed with PF at the outset.
Area of Science:
- Pediatric Gastroenterology
- Inflammatory Bowel Disease Research
- Surgical Outcomes in Crohn's Disease
Background:
- Perianal perforating disease (PF) affects approximately 15% of pediatric Crohn's disease (CD) patients.
- The clinical course of pediatric CD with PF presenting at diagnosis versus developing during therapy is not well understood.
Purpose of the Study:
- To compare the disease course and treatment outcomes of pediatric CD patients who present with perianal perforating disease (PF-CD0) at diagnosis versus those who develop PF during therapy (PF-CD1).
Main Methods:
- A prospective, single-institution observational registry of pediatric CD patients was utilized.
- Children diagnosed with perianal perforating CD (abscesses and/or fistulae) were identified.
- Patients were categorized into PF-CD0 (present at diagnosis) and PF-CD1 (developed during therapy) groups for comparison.
Main Results:
- Of 215 children with CD, 38 (18%) developed PF over a median follow-up of 4.5 years.
- PF-CD0 patients (n=26) were more likely to be younger males.
- PF-CD1 patients (n=12) showed a significantly higher likelihood of requiring diverting ileostomy (42% vs. 8%) and colectomy (33% vs. 4%).
- Multivariable analysis indicated that PF-CD1 independently predicted an increased rate of diverting ileostomy (OR 19.1).
Conclusions:
- Pediatric CD patients developing PF during therapy exhibit a more severe disease phenotype.
- These patients have a substantially higher probability of requiring surgical interventions like diverting ileostomy or colectomy compared to those presenting with PF at diagnosis.
Purpose:
Perianal perforating disease (PF) has been reported in approximately 15% of children with Crohn's disease (CD). It is unknown whether children who present with PF at the time of diagnosis have a different course than those that develop PF while on therapy.
Methods:
From a prospective, single institution observational registry of children diagnosed with CD, we identified children with perianal perforating CD, defined as perianal abscesses and/or fistulae. Patients who presented with perianal perforating CD (PF-CD0) were compared to those who developed perianal perforating CD (PF-CD1) after initial diagnosis.
Results:
Thirty-eight of 215 (18%) children with CD had PF-CD during a median follow up of 4.5 years. Patients with PF-CD0 (n=26) tended to be more likely male (81% vs. 50%, p=0.07) and younger (9.3 yrs vs. 12.5 yrs, p=0.02). PF-CD1 (n=12) patients were more likely to require diverting ileostomy (42% vs. 8%, p=0.02) and colectomy (33% vs. 4%, p=0.03). Multivariable analysis predicted increased rate of diverting ileostomy in the PF-CD1 group (p=0.007, OR 19.1, 95% CI 1.6-234.8).
Conclusion:
Pediatric CD patients who develop PF while on therapy for CD have a more severe phenotype and are more likely to require diverting ileostomy or colectomy compared to those who present with PF-CD.
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