Facebook Twitter YouTube

18027

Distinct Phenotypes of Children with Perianal Perforating Crohn Disease

Saturday, October 20, 2012: 1:24 PM
Napoleon Ballroom (Hilton Riverside)
Scott S. Short, M.D.1, Marla Dubinsky, M.D.2, Shervin Rabizadeh, M.D., M.B.A.2, Sharmayne Farrior, B.S.N.2 and Philip K. Frykman, M.D., Ph.D.3, (1)Surgery, Children's Hospital Los Angeles, Los Angeles, CA, (2)Pediatric Gastroenterology, Cedars Sinai Medical Center, Los Angeles, CA, (3)Surgery, Cedars Sinai Medical Center, Los Angeles, CA

Purpose:  Perianal perforating disease (PF) has been reported in approximately 15% of children with Crohn 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.  The aim of this study was to compare phenotype and outcome differences in children who present with PF to those who developed PF while on therapy for CD.

Methods:  Data were obtained from a prospective, single institution observational registry recording clinical phenotype and laboratory data in children diagnosed with CD < 18 years of age.  We identified all patients with perianal perforating CD, defined as perianal abscesses and/or fistulae; anal fissures and skin tags were excluded.  We compared patients who presented with perianal perforating CD (PF0) to those who developed perianal perforating CD (PF1) after initial diagnosis. Wilcoxon rank test was performed on continuous variables and Fisher exact test on categorical data. P <0.05 was considered significant.

Results:   We identified 215 CD patients.  Thirty-eight (18%) presented with, or developed PF during a median follow up of 4.5 years.  Twenty-six (68%) of patients presented with PF and twelve (32%) developed PF at a median follow up of 13.5 months. Patients presenting with PF were more likely male (81% vs. 50%, p=0.07), younger (9.3 yrs vs. 12.5 yrs, p=0.02), and have a lower BMI (17.6 vs. 22.5, p = 0.001). While PF1 patients were more likely to require diverting ileostomy (42% vs. 8%, p=0.02) and had a trend toward colectomy (25% vs. 4%, p =0.08) (Table). Use of immunomodulatory and biologic therapy was similar in both groups.

Procedure

PF0

PF1

P value

OR

95% CI

Any Crohn's Surgery (%)

69

67

1.0

1.12

0.26 – 4.85

Colectomy (%)

4

25

0.08

0.12

0.1 – 1.31

Small Bowel Resection (%)

15

17

1.0

0.91

0.14 – 5.81

Diverting Ileostomy (%)

8

42

0.02

0.12

0.02 – 0.74

I &D Perianal Abscess (%)

62

50

0.75

1.6

0.4 – 6.36

Seton Placement (%)

27

17

0.69

1.84

0.32 – 10.58

Conclusion:   Pediatric Crohn disease patients who present with PF at the initial time of CD diagnosis have a distinct phenotype and require less frequent diverting ileostomy and colectomy compared with those who develop PF after the diagnosis of CD.  Continued longitudinal study of this cohort may yield further insight in disease phenotype and hence have implications on management.