Inflammatory Bowel Disease: Updates and Controversies CASE #1 CASE #1 8/6/2015. What is the most likely diagnosis?

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1 Inflammatory Bowel Disease: Updates and Controversies Tehttp:// /~paulkeij/wpcontent/uploads/2013/07/collaboration.jpgxt August 7, 2015 Meagan M Costedio, MD; Colorectal Surgery; Cleveland Clinic Foundation Anthony L DeRoss, MD; Pediatric Surgery; Cleveland Clinic Children s 4 yo female One-year history of constipation, anal skin tags, and painful BMs Symptoms began at time of toilet training Worse over past month Holding BMs Pain with wiping Soiling of underpants Occasional bright red blood in BMs gn.com/wpcontent/uploads/2011/0 9/doctor_11.jpg What is the most likely diagnosis? A) Severe constipation with anal fissures B) Infectious disease C) Anal Crohn s disease D) Sexual abuse A) EGD and colonoscopy B) Fungal culture C) Swab for STDs D) Punch biopsy Sed Rate 9 HCT 38.2 Fecal calprotectin 178 (nl <50) Calprotectin is a protein released due to the degranulation of neutrophil granulocytes. In bowel inflammation, calprotectin may be detected in the stool. 1

2 Endoscopy Perianal ulcers with large skin tags Scattered aphthous ulcers in proximal transverse colon and rectum Terminal ileum normal Endoscopy Ileum, biopsy (A) - Ileal mucosa with a rare ill-formed mucosal granuloma Colon, cecum, ascending, transverse, biopsies - Colonic mucosa with no diagnostic alteration Colon, descending, biopsy (E) Colonic mucosa with rare granuloma Colon, sigmoid, biopsy (F) Focal active colitis Rectum, biopsy (G) - Focal active colitis with scattered nonnecrotizing granulomas tics.com/common/im ages/r_flexendoscopes.jpg A) Diverting stoma B) Proctectomy C) Steroid injection D) Antimicrobial / Antiviral E) Systemic Crohn s therapy Colorectal - Nifedipine - Kenalog - Tacrolimus - Diverting colostomy - Proctectomy Ped Surg -??? - Diverting ileostomy - Mediport for medical therapy - Ped GI - Remicade - Imuran - Steroids 10 yo female UC diagnosed at age 4 Total proctocolectomy and IPAA 1/2009 Failed ileostomy closure x 2 - Distension, emesis, obstruction Sent to CCF for second opinion 2

3 What is the diagnosis? - A) Crohn s disease - B) Chronic adhesive bowel obst. - C) Pelvic floor dysfunction - D) Pouchitis - E) Technical complication Which test is most diagnostic? - A) CT scan A/P, oral and IV contrast - B) MRI / MRE A/P - C) Contrast enema - D) Lower endoscopy - E) Defecography MRI - No narrowing, stricture, inflammation Contrast enema - Normal J pouch with post evac residual Defecography - Acute angle between pouch inlet and pouch - Redundant anterior, inferior small bowel proximal to inlet - A) Remove pouch, permanent stoma - B) Pouch revision - C) Antibiotics for pouchitis - D) Remove pouch, straight ileoanal anastomosis 3

4 IBD IBD can occur in children in all age groups. Mean age at diagnosis ~ 10 years 6% <3 years 15% 3-6 years 48% 6-12 years 37% at years Crohn s Disease 30% pediatric patients with Crohn s disease are corticosteroid dependent at 1 year after diagnosis 8% will require surgery over the same time period Heyman MB, Kirschner BS, Gold BD, et al. Children with early-onset inflammatory bowel disease (IBD): analysis of a pediatric IBD consortium registry. J Pediatr 2005;146(1): Markowitz J, Hyams J, Mack D, et al. Corticosteroid therapy in the age of infliximab: acute and 1- year outcomes in newly diagnosed children with Crohn s disease. Clin Gastroenterol Hepatol 2006;4(9): Crohn s Disease Laparoscopic and single-incision laparoscopic procedures for pediatric patients with IBD have been described Recurrence rate after surgical treatment of Crohn s disease in children 17% at 1 year 38% at 3 years 60% at 5 years Baldassano RN, Han PD, Jeshion WC, et al. Pediatric Crohn s disease: risk factors for postoperative recurrence. Am J Gastroenterol 2001;96(7): Pacilli M, Eaton S, Fell JM, et al. Surgery in children with Crohn disease refractory to medical therapy. J Pediatr Gastroenterol Nutr 2011;52(3): Asia /100,000 Prevalence US /100,000 Europe 35-70/100,000 Middle Eastern Jewish /100,000 Middle Eastern Arab 5-20/100,000 Why to Operate Failure of medical management - Allergies - Intolerance - Want to conceive Dysplasia - Low grade 11% cancer - High grade 40% cancer Cancer What Operation? 3 Stage - Biologics - >20mg prednisone - Fecundity 4

5 What Operation? 2 Stage - Dysplasia/Cancer - Weaned off medications Total Proctocolectomy in Children Proctocolectomy with ileal pullthrough Straight and pouch anastomotic procedures are described J-pouch results in better short term outcomes than either a straight or a more complex pouch 6-8 bowel movements a day Most pediatric patients achieve daytime continence <5% report occasional nighttime incontinence Most common long-term complication is pouchitis Coran AG. A personal experience with 100 consecutive total colectomies and straight ileoanal endorectal pull-throughs for benign disease of the colon and rectum in children and adults. Ann Surg 1990;212(3): Telander RL, Spencer M, Perrault J, et al. Long-term follow-up of the ileoanal anastomosis in children and young adults. Surgery 1990;108(4): Durno C, Sherman P, Harris K, et al. Outcome after ileoanal anastomosis in pediatric patients with ulcerative colitis. J Pediatr Gastroenterol Nutr 1998;27(5): Fonkalsrud EW, Thakur A, Beanes S. Ileoanal pouch procedures in children. J Pediatr Surg 2001;36(11): Laparoscopic IPAA: Advantages Over Open - Short term benefits Hospitalization Blood loss Cosmesis - Long Term benefits Improved fecundity Laparoscopic % Bartels et. al. Ann Surg 2012, Beyer-Berjot Ann Surg 2013 Open 39-50%? Decreased hernia rate? Decreased SBO 5