Journal of Pediatric Surgery (2010) 45, E27–E29

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Granulomatous appendicitis in a 12-year-old boy

Derya Yayla a,⁎, Bedriye Nuray Alpman b , Yasemin Dolek c
a
Department of Pediatrics Surgery, Cankiri State Hospital, 18200 Cankiri, Turkey
b
Department of Pediatrics, Cankiri State Hospital, 18200 Cankiri, Turkey
c
Department of Pathology, Cankiri State Hospital, 18200 Cankiri, Turkey

Received 9 December 2009; revised 1 June 2010; accepted 7 June 2010

Key words:
Abstract Isolated granulomatous inflammation of the appendix is extremely rare, and its etiology is still
Granulomatous
unknown. We describe a 12-year-old boy with isolated granulomatous appendicitis where the etiology
appendicitis;
could not be clarified despite infectious criteria such as high fever and gastroenteritis. Children with
Etiology;
epithelioid granulomatous appendicitis have a good prognosis following appendectomy.
Children;
© 2010 Elsevier Inc. All rights reserved.
Prognosis

Granulomatous inflammation of the appendix is un- 1. Case report

common [1,2]. This condition is characterized by the
presence of activated macrophages known as epithelioid A 12-year-old boy with no previous surgical problems
histiocytes because of their modified epithelial-like appear- was referred for right lower abdomen pain, fever, fatigue,
ance [3]. The etiology includes systemic disorders, such as and diarrhea. His family history was unremarkable, and he
Crohn's disease and sarcoidosis, and infections, such as had never suffered from bowel disease. He had seen his
those caused by Mycobacterium tuberculosis, Yersinia pediatrician 7 days earlier for acute gastroenteritis, and
pseudotuberculosis, parasites, and fungi. Isolated granulo- cefuroxime had been prescribed. He experienced fever as
matous inflammation of the appendix is extremely rare, high as 38.5°C, loss of appetite, and fatigue in this period and
and its etiology is still unknown [4]. However, it is developed abdominal pain for the last 2 days. He was
beneficial to exclude systemic and infectious causes of admitted to the hospital; and on examination, his temperature
granulomatous inflammation by appropriate investigations was 38.5°C (remittent course), and the pulse rate was
and follow-up. 110 beats per minute. He had right lower abdominal
An English literature survey revealed 200 published cases tenderness, and the liver was palpable 3 cm below the costal
of granulomatous appendicitis (GA) including 17 instances in margin in the midclavicular line. The hemoglobin level was
children [5-15]. We report a pediatric patient suffering from 11 g/dL, white cell count was 20,000/μL, C-reactive protein
idiopathic GA who presented with an acute abdomen. was 15 mg/dL, and sedimentation rate was 60 mm/h. Plain
abdominal radiographs showed a bowel segment with an air-
fluid level in the right iliac region. Hepatosplenomegaly
(liver 17 cm and spleen 16.7 cm on the midclavicular line)
⁎ Corresponding author. Tel.: +90 505 3572662; fax: +9037602130783. and normal kidneys were noted on ultrasonography. Cultures
E-mail address: dryayla@yahoo.com (D. Yayla). (throat, urine, blood, stool) taken for microbiological

0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved.
doi:10.1016/j.jpedsurg.2010.06.017
E28 D. Yayla et al.

assessment grew only normal flora. Abundant fecal leuko-

cytes were detected on stool microscopy, but result of stool
culture and Gram stain was negative. A nasogastric tube was
passed and returned bilious material.
Surgical exploration was performed because of findings
of an acute abdomen associated with bilious drainage from
the nasogastric tube. A retrocecal edematous appendix 12 cm
in length, 12 mm in diameter, and congested on the outer
surface (Fig. 1) was detected; and appendectomy was
performed. The remaining bowel segments looked normal.
The patient was fed per oral on postoperative day 1. The
antibiotic regimen was changed on postoperative day 3
because of fever and leukocytosis. Chest radiograph showed
pneumonic infiltrations in the posterior segments of the
inferior pulmonary lobes. Serologic test results for Yersinia,
Salmonella, and Brucella were all negative. Histologic Fig. 2 Histologic examination revealed granulomas (with central
necrosis in some of them and multinuclear giant cells surrounded by
examination of the appendix revealed epithelioid granulomas
lymphocytes) composed of epithelioid histiocytes on mucosal,
in the wall of the appendix, but no foreign bodies, muscular, and serosal surfaces in appendix sections (hematoxylin
obstructing lesions, or parasites. Results of stains for acid- and eosin, magnification ×40). CN indicates central necrosis; GC,
fast bacillus and fungi, and serology for Yersinia were all giant cells.
negative. The pathology described diffuse granulomas in the
mucosa, muscularis propria, and serosa of the specimen
consistent with a diagnosis of “granulomatous appendicitis” Leukocytosis decreased remarkably after postoperative day
(Fig. 2). 6 without fever. The patient had an uneventful postoperative
Chest radiographs and thoracic, abdominal, and cranial course. He was discharged 14 days after the procedure. Tests
computed tomography revealed no pathology except hepa- for Salmonella and Brucella were repeated a month later,
tosplenomegaly and pneumonic infiltrations in the posterior and no pathogen was detected in an attempt to clarify the
segments of both lower lobes of the lungs on postoperative etiology. He has been followed in the outpatient department
day 4. The patient had no history of tuberculosis or any for 1 year and remains well and asymptomatic with no
evidence of pulmonary or systemic involvement but was medical treatment.
nevertheless investigated for tuberculosis. The purified
protein derivative test was performed in the postoperative
period, and the result was negative. Result of search for acid-
resistant bacteria in the gastric lavage and sputum was 2. Discussion
negative 3 times, and no tuberculosis bacilli were demon-
strated on pathology. Granulomatous appendicitis is a rare condition, account-
The patient received ampicillin/sulbactam and amikacin ing for less than 2% of all cases of appendicitis [2,16]. The
for 3 days and vancomycin-meropenem for 11 days. reported incidence in Western countries ranges from 0.14%
to 0.3%, with an incidence of 1.3% to 2.3% in developing
countries [17]. Granulomas are known to occur in the
gastrointestinal tract in association with a number of
systemic granulomatous conditions and infections. The
higher incidence in developing countries is believed to be
because of increased incidence of tuberculosis and schisto-
somiasis [18].
Although granulomas are a manifestation of Crohn's
disease, only 5% to 10% of patients with GA develop
Crohn's disease elsewhere in their gastrointestinal tract [2].
There are 2 types of isolated granulomatous inflammation of
the appendix, namely, idiopathic GA and isolated appendi-
ceal Crohn's disease [4]. Isolated appendiceal Crohn's
disease is rare, with a reported incidence of only 0.2% of
all patients diagnosed with Crohn's disease. The clinical
course of isolated appendiceal Crohn's disease also differs
Fig. 1 Intraoperative appearance in which the appendix was from that of ileal or colonic Crohn's disease [1]. Postoper-
thickened and enlarged. The cecum and terminal were grossly normal. ative fistula formation or recurrence is rare in isolated
Granulomatous appendicitis in a 12-year-old boy E29

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