Crohn's disease presenting in an adult surgically treated for Hirschsprung's disease

IntroductionHirschsprung's disease (HD) is the most common cause of lower intestinal obstruction in infants and is generally curable through surgical resection. Common post-operative complications that result from residual aganglionic bowel can include recurrent intestinal obstruction, enterocolitis and anastomotic ulcers. Manifestation of these complications can appear many years after surgery and may mimic symptoms of inflammatory bowel disease (IBD). We present a rare case of fibrostenotic ileal Crohn's with perianal disease in an adult male previously treated for Hirschprung's disease.Case PresentationA 37 year old male with a history of long segment HD, who underwent a subtotal colectomy and ileorectal anastomosis as a newborn presented for complaints of rectal pain, diarrhea, tenesmus and urgency. Both erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated and stool studies were negative for infection. Ileoscopy demonstrated a normal appearing anastomosis but was significant for skip areas of erythema and friable mucosa at 30 cm and again at 50cm from the anal verge. Biopsies showed evidence of chronic active ileitis with architectural distortion. The patient was treated with a course of ciprofloxacin and metronidazole for presumed enterocolitis. Although he had an initial clinical response, he developed worsening abdominal pain and a Computed Tomography (CT) scan of the abdomen and pelvis revealed a 7 cm segment of severe transmural inflammation and proximal small bowel dilation in distal ileum. The patient underwent an explorative laparotomy with a small bowel resection. Histology of the strictured segment demonstrated acute cryptitis with architectural distortion, submucosal edema and fibrosis, granulomas and transmural inflammation consistant with a diagnosis of Crohn's disease. Four weeks post-op the patient developed perianal pain during bowel movements. Exam revealed the presence of an anal fissure and a magnetic resonance imaging (MRI) of the pelvis was significant for a low intrasphinteric fistula. Magnetic resonance enterography did not show evidence of additional proximal small bowel disease. The patient was started on adalimumab and had complete resolution of his perianal symptoms during induction. Repeat ileoscopy 12 weeks post-op revealed improved ileal inflammation. The patient currently remains in remission on adalimumab monotherapy.DiscussionThe development of IBD in patients with history of Hirschsprung's di