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Journal of Indian Association of Pediatric Surgeons
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Year : 2014  |  Volume : 19  |  Issue : 2  |  Page : 100-102

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

Department of Pediatric Surgery, Alexandroupolis University Hospital, Democritus University of Thrace School of Medicine, Alexandroupolis, Greece

Date of Web Publication29-Mar-2014

Correspondence Address:
George Vaos
Department of Pediatric Surgery, Alexandroupolis University Hospital, Democritus University of Thrace School of Medicine, Alexandroupolis 68100
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.129606

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Crohn's disease (CD) can occur anywhere in the gastrointestinal tract from the mouth to the anus and sometimes it presents in a nonspecific manner. Herein we describe a case of local peritonitis as the first manifestation of CD in an 11-year-old girl, and the subsequent method of treatment.

Keywords: Children, Crohn′s disease, intestinal perforation, local peritonitis

How to cite this article:
Kambouri K, Gardikis S, Agelidou M, Vaos G. Local peritonitis as the first manifestation of Crohn's disease in a child. J Indian Assoc Pediatr Surg 2014;19:100-2

How to cite this URL:
Kambouri K, Gardikis S, Agelidou M, Vaos G. Local peritonitis as the first manifestation of Crohn's disease in a child. J Indian Assoc Pediatr Surg [serial online] 2014 [cited 2023 Jun 8];19:100-2. Available from: https://www.jiaps.com/text.asp?2014/19/2/100/129606

   Introduction Top

Crohn's disease (CD) is a chronic condition characterized by an inflammatory process that extends across all tissue layers of the small intestine, and may appear in any section of the gastrointestinal tract. [1] Free or sealed perforation with local peritonitis is a rare complication of CD. The incidence of free perforation in CD has been reported to be 1-2% in western countries, manifesting either initially or eventually in the disease course. [2] Herein we describe a case of suspected acute appendicitis but with the operative finding of local peritonitis as the first manifestation of CD, and the subsequent method of treatment.

   Case Report Top

An 11-year-old girl presented to our hospital with abdominal pain and vomiting lasting 3 days. Her past medical history was uneventful. At the time of presentation the patient was quite unwell and had fever (37.8°C). Clinical examination revealed abdominal tenderness with a positive McBurney sign. She had a white cell count of 15,000 K/ml (neutrophils, 86.2%; lymphocytes, 5.9%) and an erythrocyte sedimentation rate of 25 mm; all other hematological findings were within normal limits. An abdominal ultrasound investigation of the patient revealed the presence of free fluid in the right iliac fossa and an inflamed appendix. The preoperative diagnosis was acute appendicitis, and the child was immediately prepared for surgery. At the operation, pus was found in the right abdominal cavity and the appendix appeared to be swollen, thickened, and covered by inflamed omentum. The adjacent terminal ileum was reddened and edematous. An appendicectomy was performed and a drain was placed in the Douglas space following irrigation of the abdominal cavity. On the 7 th postoperative day, the patient developed fever (39°C) and wound infection despite administration of antibiotics. The wound was drained and her clinical condition improved. Nevertheless, on the 11 th postoperative day the patient again developed pyrexia (40°C) and enteric discharge from the wound. Histological evaluation of the resected appendix revealed only periappendicular inflammation containing prominent lymphoid follicles. The pus culture from the abdominal cavity revealed Escherichia coli.

Computed tomography (CT) of the abdomen revealed a pericecal collection, two enterocutaneous fistulae, and two strictures in the ileum, suggesting CD [Figure 1]a and b. A colonoscopy was also performed along with biopsy of the terminal ileum [Figure 2]. The histological findings were typical of those for CD. The patient was managed with total parenteral nutrition, antibiotics, and corticosteroids. Despite this conservative treatment, the child deteriorated and a reoperation was performed on the 45 th postoperative day. A mass measuring 5 × 4 cm containing the cecum, terminal ileum, and part of the omentum were noted; probably resulting from postinfectional adhesions. A right limited hemicolectomy including the fistulae and strictures was performed using a stapler, with anastomosis of the macroscopically appearing healthy bowel of the ascending colon and ileum. Histological investigation of the resected specimen confirmed the presence of CD in the terminal ileum. The patient's postoperative recovery was uneventful. The child was followed-up for 1 year and remained asymptomatic thereafter, receiving long-term azathioprine to keep the disease inactive.
Figure 1: Computed tomography (CT) scan of the abdomen showing with white arrows the two enterocutaneous fistulae (a) and stricture in the ileum (b)

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Figure 2: Colonoscopy showing edematous, reddish ileocecal valve with erosions

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   Discussion Top

CD typically affects the ileum, colon, and perianal regions. The associated inflammatory process in the intestine is a full-thickness injury that can lead to significant complications such as fistulae, abscesses, and phlegmons from microperforation of the intestine. [1] Free perforation is unusual in CD, except in areas of longstanding active CD with obstruction, where increased luminal pressure resulting from the obstruction readily leads to perforation. [3]

CD diagnosis is based on a combination of clinical manifestations and radiological findings and can be confirmed only after histopathology. The disease can sometimes present in a nonspecific manner. Uncommon initial presentations of CD may be revealed by a single symptom or sign such as mouth ulcers, eating disorders, pathological fractures, gastropathy, and perianal disease. Furthermore, the initial presentation of pediatric CD may resemble acute appendicitis. [4],[5],[6] 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. Our patient was not previously known to have CD. Local peritonitis due to intestinal perforation in CD is very rare. An English-language review revealed only two clinical reports of spontaneous free perforation of the small intestine in CD. [7],[8] Those reports did not make clear whether the perforation was the initial symptom of CD in the patients.

The surgical management of CD has changed considerably during the past decade as a result of developments in medical therapy. [9] Surgical interventions are employed in children only after acute complications such as strictures, perforations, abscesses, fistulae, or severe perianal disease. [10] Medical intervention and surgery have traditionally been regarded as complementary treatments for CD, because despite improvements in medical treatments most patients (70-90%) with CD will eventually require surgical intervention. [11] The spontaneous closure of an enterocutaneous fistula is the ideal outcome. However, there are some unfavorable factors that preclude fistula closure, including poor nutritional status, presence of sepsis, and active CD. [12] A patient should be prepared for surgery when a fistula does not close within the expected time frame (i.e., within 4-5 weeks). [12] Long-term studies have demonstrated that there is a 50% chance that the patient will never require further related surgery following resection of the ileocecal segment. [13] Periodic surveillance visits with radiological and endoscopic examination of the small intestine and colon for a period of at least 3 years is recommended by most authors to allow prompt detection of any recurrence. [14]

Free or sealed perforation of the bowel is an uncommon event in the natural history of CD in children, and occurs mainly in the course of the disease; it is only rarely an initial presentation. Although it is an uncommon occurrence, perforation of the bowel with local peritonitis should be included in the differential diagnosis of CD, even in young patients.

   References Top

1.Haddad M, Azim F, Koren A, Stelman E, Mor C, Zelikovski A. Crohn's disease of the appendix. Eur J Surg 1993;159:191-2.  Back to cited text no. 1
2.Ikeuchi H, Yamamura T. Free perforation in Crohn's disease: Review of the Japanese literature. J Gastroenterol 2002;37:1020-7.  Back to cited text no. 2
3.Greenberg GR, Fedora RN, Thomson AB. Inflammatory bowel disease. In, Thomson ABR, Shaffer EA, editors. First Principles of Gastroenterology. The Basis of Disease and an Approach to Management. 5 th ed. Janssen-Ortho;2008;307-56. Available from: http://www.cag-acg.org/uploads/firstprinciples/z_pdf/EN_GAST_09B.pdf [Last accessed on 2013 May 14].  Back to cited text no. 3
4.Nattiv R, Dinari G, Amir J, Avitzur Y. Isolated severe gastropathy- an unusual presentation of Crohn's disease in child. Isr Med Assoc J 2008;10:322-4.  Back to cited text no. 4
5.Bischoff A, Gupta A, DMello S, Mezoff A, Podberesky D, Barnett S, et al. Crohn's disease limited to the appendix: A case report in a pediatric patient. Pediatr Surg Int 2010;26:1125-8.  Back to cited text no. 5
6.Bass JA, Goldman J, Jackson MA, Gasior AC, Sharp SW, Drews AA, et al. Pediatric Crohn disease presenting as appendicitis: Differentiating features from typical appendicitis. Eur J Pediatr Surg 2012;22:274-8.  Back to cited text no. 6
7.Freeman HJ. Spontaneous free perforation of the small intestine in Crohn's disease. Can J Gastroenterol 2002;16:23-7.  Back to cited text no. 7
8.Greenstein AJ, Sachar DB, Mann D, Lachman P, Heimann T, Aufses AH Jr. Spontaneous free perforation a perforated abscess in 30 patients with Crohn's disease. Ann Surg 1987;205:72-6.  Back to cited text no. 8
9.Dignass A, Van Assche G, Lindsay JO, Lémann M, Söderholm J, Colombel JF, et al. European Crohn's and Colitis Organisation (ECCO). The second european evidence-based consensus on the diagnosis and management of Crohn's disease: Current management. J Crohns Colitis 2010;4:28-62.  Back to cited text no. 9
10.Besnard M, Jaby O, Mougenot JF, Ferkdadji L, Debrun A, Faure C, et al. Postoperative outcome of Crohn's disease in 30 children. Gut 1998;43:634-8.  Back to cited text no. 10
11.Polle SW, Wind J, Ubbink DT, Hommes DW, Gouma DJ, Bemelman WA. Short-term outcomes after laparoscopic ileocolic resection for Crohn's disease. A systematic review. Dig Surg 2006;23:346-57.  Back to cited text no. 11
12.Pritts TA, Fischer DR, Fischer JE. Postoperative enterocutaneous fistula. In: Holzheimer RG, Mannick JA, editors. Surgical Treatment. Evidence-Based and Problem- Oriented. Munich: Zuckschwerdt Verlag; 2001. Available from: http://www.ncbi.nlm.nih.gov/books/NBK6914/ [Last accessed on 2013 May 14].  Back to cited text no. 12
13.Kim NK, Senagore AJ, Luchtefeld MA, MacKeigan JM, Mazier WP, Belknap K, et al. Long-term outcome after ileocecal resection for Crohn's disease. Am Surg 1997;63:627-33.  Back to cited text no. 13
14.Prieto-Nieto I, Perez-Robledo JP, Hardisson D, Rodriguez-Montes JA, Larrauri-Martinez J, Garcia-Sancho-Martin L. Crohn's disease limited to appendix. Am J Surg 2001;182:531-3.  Back to cited text no. 14


  [Figure 1], [Figure 2]


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