Colonoscopic diagnosis of appendiceal intussusception with cystic fibrosis is important to avoid colonoscopic “polypectomy” of an inverted appendix causing inadvertent cecal perforation.1Fazio R.A. Wickremesinghe P.C. Arsura E.L. et al.Endoscopic removal of an intussuscepted appendix mimicking a polyp: an endoscopic hazard.Am J Gastroenterol. 1982; 77: 556-558PubMed Google Scholar Yet little is known about the colonoscopic findings.2Bradly D.P. Gallegos M. Jakate S. Appendiceal mass in an adolescent with cystic fibrosis.Clin Gastroenterol Hepatol. 2009; (A24): 7Google Scholar We report a striking colonoscopic appearance of this intussusception. A 19-year-old woman with cystic fibrosis, complicated by pulmonary bronchiectasis, chronic pancreatic insufficiency, and 2 admissions for right lower quadrant (RLQ) pain attributed to distal intestinal obstruction syndrome (DIOS), presented with increasingly severe RLQ pain and vomiting for 2 weeks. The abdomen was soft, but was mildly tender in the RLQ. Routine blood test results and esophagogastroduodenoscopy were within normal limits. Abdominopelvic computed tomography revealed a tubular structure (extracolonic appendix, black arrows) extending 8 cm outside cecum, penetrating through the cecal wall (white arrow), to become a multiwalled structure within the cecum (intracecal, inverted appendix), and containing a 1.3-cm–wide lumen, findings consistent with appendiceal intussusception (Figure A, sagittal view). Colonoscopy, performed to exclude cecal cancer, revealed a 5-cm long, smooth, tubular structure covered by normal-appearing mucosa, arising perpendicularly from cecal caput, and containing a central 1.3-cm–wide lumen filled beyond the brim with impacted, inspissated secretions (Figure B, colonoscopic videophotograph at ileocecal valve level). This structure was identified as a partly inverted appendix by its attachment to cecal caput and absence of an alternative appendiceal orifice. Cautious colonoscopic irrigation with water, serial luminal biopsies, and suctioning removed only 10% of impacted secretions, in an unsuccessful attempt at disimpaction to reduce the intussusception. Colonoscopic biopsies of intussuscepted appendiceal mucosa were safely performed, revealing nonneoplastic, colonic-type mucosa with infiltration of eosinophils, findings associated with chronic appendicitis/appendiceal intussusception. The patient underwent laparoscopic appendectomy. Gross examination of resected specimen revealed a distended appendix with a dilated, 1.3-cm–wide lumen, filled with inspissated mucous. Microscopic examination in cross-section revealed thinned appendiceal mucosa from compression by a greatly expanded central lumen, filled with impacted, inspissated, eosinophilic secretions (Figure C, low-power photomicrograph, H&E, 20×). High-power photomicrograph revealed inspissated secretions arising from a crypt (arrows) and flowing, similar to lava, into the appendiceal lumen, which are characteristic appendiceal findings with cystic fibrosis (Figure C, inset, 400×). Patients with cystic fibrosis can develop appendiceal intussusception from impacted appendiceal secretions that provide a lead point for intussusception.3Chaar C.I.O. Wexelman B. Zuckerman K. et al.Intussusception of the appendix: comprehensive review of the literature.Am J Surg. 2009; 198: 122-128Abstract Full Text Full Text PDF PubMed Scopus (59) Google Scholar They also develop other obstructed ducts/lumens from inspissated secretions, including DIOS, pancreatic obstruction with pancreatic insufficiency, and biliary obstruction. The following, currently reported, colonoscopic and colonoscopic biopsy findings may be diagnostic of appendiceal intussusception with cystic fibrosis: a tubular structure within cecum, containing impacted, inspissated secretions within its lumen, arising from the cecal caput, and without an alternative appendiceal orifice, with colonoscopic biopsies showing an eosinophilic mucosal inflammatory infiltrate without neoplasia. Endoscopic therapy to relieve obstruction currently is performed for esophageal food impaction. As a speculation, gentle colonoscopic appendiceal luminal irrigation, perhaps using N-acetylcysteine, might disimpact and potentially reduce the intussusception, just as colonic irrigation at colonoscopy with N-acetylcysteine may relieve DIOS.4Shah U. Shafiq Y. Khan M.A. Gastrograffin use in distal intestinal obstruction syndrome of cystic fibrosis.J Ayub Med Coll Abbottabad. 2007; 19: 58-60PubMed Google Scholar
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