Crohn’s disease limited to the appendix: a case report in a pediatric patient

Abstract

In the original description of Crohn's disease, the appendix was not believed to be involved in the inflammatory process. Later on, case reports started to appear in publications demonstrating that the appendix could be involved in the inflammatory changes of Crohn's disease, and it could also be the primary or the sole manifestation of the disease. Being that appendectomies are one of the most common procedures performed by pediatric surgeons, the knowledge about this diagnosis, all be it rare, is important. Our aim was to report a case and discuss the results of our literature review in order to elucidate the probability of a pediatric patient subsequently developing full Crohn's disease and the follow up that is indicated in such patients. A 12-year-old male patient presented with a history of chronic abdominal pain (3-4 times per week) for 1 year, crampy in nature, localized in the left lower quadrant, and associated with diarrhea (2 episodes per day). There were no extraintestinal manifestations of Crohn's, such as arthralgia or uveitis. Important family history included two paternal uncles with ulcerative colitis both of whom currently have stomas. The only abnormal laboratory value in our patient was an elevated fecal calprotectin level. An esophagogastroduodenoscopy and colonoscopy were performed and found to be unremarkable except for the cecum where it appeared that an exudate was emanating from the appendiceal orifice. A magnetic resonance enterography was ordered and showed an enlarged enhancing appendix. An exploratory laparoscopy identified an appendix with macroscopic cobblestone or lymphoid reaction that histologically was consistent with Crohn's disease. It appears that the Crohn's appendix is more indolent than Crohn's disease of the ileum or colon, with a recurrence rate in the largest series of 8%. The interval time from diagnosis to recurrence varied from 1 to 48 months with an average of 19 months. Some authors debate the need of follow up at all in those patients, believing that the appendectomy alone is curative in the majority of patients. Others recommend follow up for up to 5 years.

Local peritonitis as the first manifestation of Crohn′s disease in a child

“…Furthermore, the initial presentation of pediatric CD may resemble acute appendicitis. [456] Although the intraoperative findings in our patient with ileocecal CD suggested inflammation of the appendix with local peritonitis, the histological investigation confirmed only periappendicular inflammation without involvement of the appendix. The periappendicular inflammation and the pus were probably due to extension of the inflammatory process from the microperforation of the terminal ileum.…”

Actinomyces in Crohn’s‐like appendicitis

“…The concept of Crohn’s disease limited to the appendix was once generally accepted, on the basis of the histological resemblance of some cases of granulomatous appendicitis to Crohn’s disease. However, the clinical presentation was often acute or subacute abdominal pain, and these patients did not present with long‐term symptoms of Crohn’s disease . More significantly, whereas Crohn’s disease often recurs after surgical resection of a diseased segment of intestine, patients with Crohn’s‐like appendicitis develop Crohn’s disease in other intestinal segments in <10% of cases …”

“…However, the clinical presentation was often acute or subacute abdominal pain, and these patients did not present with long-term symptoms of Crohn's disease. [4][5][6] More significantly, whereas Crohn's disease often recurs after surgical resection of a diseased segment of intestine, patients with Crohn's-like appendicitis develop Crohn's disease in other intestinal segments in <10% of cases. 7,8 The differential diagnosis of Crohn's-like appendicitis or granulomatous appendicitis includes several infections, although some, such as tuberculosis or parasitic infection, are uncommon in Western populations.…”

A case report of unexpected pathology within an incarcerated ventral hernia

“…Regardless, these reports have concluded that compared to antibiotics-only groups, surgical therapy results in a significantly higher efficacy rate at 1 year, equivalent length of hospital stay, sick leave, and post-hospital symptom duration, and has been recommended as the definitive treatment of acute appendicitis [12], [14], [15]. While laparotomy with washout and appendectomy has been the standard of care for the surgical management of perforated hernial appendicitis, [16]; more recent reports describe the safety and advantages to laparoscopy [9]. The laparoscopic approach proved beneficial for our patient, as it simultaneously allowed excellent visualization of her intra-abdominal contents, including the viability of the bowel within and on ingress into the hernia sac, reduction of the hernial contents, comprehensive peritoneal washout, and appendectomy, without incurring the additional morbidity of a large herniotomy.…”

“…Twelve to 16% of patients with Crohn’s disease who have ileal involvement and 20–50% with colonic involvement present with the inflammatory process extending to the appendix [18], [19]. About 85% of patients with appendiceal Crohn’s present with acute abdominal pain in the right iliac fossa, similar to classic appendicitis symptoms [5], [16].…”