Onset of Crohnʼs disease post-proctocolectomy for Familial Adenomatous Polyposis

Abstract

Introduction: Patients diagnosed with Familial Adenomatosis Polyposis (FAP)have an increased risk for colorectal carcinoma and as a result undergo proctocolectomyand regular surveillance regimens. Pouchitis is rarely seen in thesepatients, and the possibility of de novo inflammatory bowel disease has not beenwell described. Here we present a patient who developed Crohn's disease in anS-pouch procedure for FAP.CASE DESCRIPTION: We present the case of a 60 year old male who was diagnosedwith FAP at the age of 45 and underwent a staged S-pouch procedure.Since then, he has had regular surveillance with both upper and lower endoscopies.He was noted to have recurrence of polyps including one just abovethe surgical anastomosis and a polyp in the S-pouch that required treatmentseveral times. He underwent an ampullectomy in 2004 under endoscopic retrogradecholangiopancreatography with stent placement due to the presence ofnumerous duodenal adenomas which subsequently recurred in March of 2011.In the last two years prior to the current presentation, he described anincreased frequency of loose stools, abdominal pain, and fatigue. A pouchoscopyperformed in July of 2010 revealed aphthous ulcerations. In March of2011, pouchoscopy showed two areas of active disease, one at 30 and theother in the pre-pouch neoterminal ileum with deep, irregular ulcers, erythemaand edema. Biopsies of this area demonstrated mild to moderate activechronic ileitis with ulceration. A CT scan of the abdomen showed ileal wallthickening. His course was complicated by the development of a perianalabscess with multiple perineal fistulous tracts, which were managed withciprofloxacin and metronidazole. Based on the above, he was diagnosed withCrohn's Disease and started on 6-mercaptopurine, which had to be discontinueddue to a rash. Pouchoscopy with biopsy in June 2011 showed minimalactive disease. He is currently managed with ciprofloxacin and metronidazoleto promote healing of his fistulous tracts. Treatment with anti-tumor necrosisfactor-a was not started due to the concern of neoplastic transformation ofany polyps.CASE DISCUSSION: The association between patients with FAP and IBD is veryuncommon and not well described in the literature. Fukushima et al described a40-year-old man with FAP and concurrent Crohn's Disease that was incidentallydiscovered during surgery.1 In addition, Brignola et al presented the familial associationbetween FAP and IBD in two patients.2 In our patient described above, hispost operative course has been complicated by the development of Crohn's diseaserequiring an alteration in surveillance and initiation of chronic therapy.Based on the rarity of pouchitis in FAP, the differential diagnosis for a patientwith FAP presenting with diarrhea, hematochezia, or abdominal pain shouldinclude Crohn's disease.