MARIA NIÑA U.

PEDROZO-14J GROUP-39

Acute jejunoileal obstruction due to a pseudopolyp in a child with

undiagnosed crohn's disease

Efstratios Christianakis, Nikolaos Pashalidis, Stavroula Kokkinou, Michael Pitiakoudis, Evangelos

Mplevrakis, Maria Chorti, Spiros Rizos and Dimitrios Filippou

Introduction

Crohn's disease (CD) can affect any part of the alimentary tract from the mouth to
the anus, with most common site being the terminal ileum. Bowel obstruction is a well-
known complication of CD, usually as the result of stricture formation, or more rarely as
mechanical obstruction. Intestinal obstruction due to a large pseudopolyp is a rare event in
CD .

Crohn's disease is thought to be an autoimmune disease, in which the body's

immune system attacks the gastrointestinal tract, causing inflammation; it is classified as a
type of inflammatory bowel disease. There has been evidence of a genetic link to Crohn's
disease, putting individuals with siblings afflicted with the disease at higher risk. It is
understood to have a large environmental component as evidenced by the higher number
of cases in western industrialized nations. Males and females are equally affected. Smokers
are three times more likely to develop Crohn's disease. Crohn's disease affects between
400,000 and 600,000 people in North America. Prevalence estimates for Northern Europe
have ranged from 27–48 per 100,000. Crohn's disease tends to present initially in the teens
and twenties, with another peak incidence in the fifties to seventies, although the disease
can occur at any age.

The disease was named for American gastroenterologist Burrill Bernard Crohn, who
in 1932, along with two colleagues, described a series of patients with inflammation of the
terminal ileum, the area most commonly affected by the illness. For this reason, the disease
has also been called regional ileitis or regional enteritis. The condition, however, has been
independently identified by others in the literature prior, most notably in 1904 by Polish
surgeon Antoni Leśniowski for whom the condition is additionally named (Leśniowski-
Crohn's disease) in the Polish literature.
Case presentation

A 12-year-old boy was brought to the emergency department with acute abdominal
pain lasting 12 hours, and associated abdominal distension, absolute constipation for two
days, vomiting and fever of 38.5°C. When examined he had general abdominal tenderness.
White blood cell count was 17.5 k/ml with 85.5% neutrophils, hemoglobin was 10.9 gr/dl,
hematocrit 34.7% and platelets 820 k/ml. Abdominal X-rays showed air-fluid levels.
Abdominal ultrasound examination revealed a solid intraluminal pattern. The patient gave
a history of referred intermittent abdominal pain for a period of 6 months. More detailed
clinical information, such as diarrhea for the past 6 months, quick tiredness, no mood to
play, lethargy and paleness, was obtained postoperatively.

The patient underwent laparotomy and 30 cm of small bowel with multiple areas of
damage was found. The damage included macroscopically a rigid and thickened mass in the
ileal portion, creeping fat, multiple granulomas in the external intestinal surface and ulcers,
two of which had parietal ruptures with fluid escape. A resection of 45 cm of the ileo-
jejunal portion, including all areas of intestinal damage, was performed and a primary end
to end ileo-jejunal anastomosis completed the operation. Longitudinal incision of the
intestine showed a cobblestone appearance, due to linear ulcers crossing with transverse
folds. Linear ulcers were created from interconnected rows of aphthous ulcers. A
characteristic large pseudopolyp, 4 cm in diameter, was in the obstructed portion of the
mass. Microscopically, edema and diffuse inflammation of the whole intestinal wall,
fissures, granulomas, vascular dilatation, pseudopolyps, mucosal inflammation of the small
and large bowel, and granulomas in local lymph nodes were observed. The tip of the
appendix was inflamed to One month later, endoscopic examinations showed granulomas
and other Crohn's lesions in the stomach and colorectum.The patient was treated with 1.5 g
daily dose of Mesalamine for one year, without recurrence of the disease. During this
period he also received Modulen complementary oral feeds and his growth was normal.

Cause
Although the exact cause of Crohn's disease is still unknown, a combination of
environmental factors and genetic predisposition seems to cause the disease.T he genetic
risk factors have now more or less been comprehensively elucidated, making Crohn's
disease the first genetically complex disease of which the genetic background has been
resolved. The relative risks of contracting the disease when one has a mutation in one of
the risk genes, however, are actually very low (approximately 1:200). Broadly speaking, the
genetic data indicate that innate immune systems in patients with Crohn's disease
malfunction, and direct assessment of patient immunity confirms this notion. This had led
to the notion that Crohn's disease should be viewed as innate immune deficiency, chronic
inflammation being caused by adaptive immunity trying to compensate for the reduced
function of the innate immune system
http://search.creativecommons.org/?q=Intestinal+pseudoobstruction+ARTICLE&sourceid=Mozilla-
search

Fred A: Crohn's disease. In Pediatric Surgery. Volume 2. 6th edition. Edited by: Grosfeld JL, ONeill JA,
Fonkalsrud EW, Coran AG. Philadelphia: Mosby Elsevier; 2006:1453-1461.

CD can affect any part of the alimentary tract from the mouth to the anus, with the most
common site being the terminal ileum [1,2]. Approximately 15% of all patients with CD
are children. There are special pathological features that distinguish CD from Ulcerative
Colitis [3]. A cobblestone appearance is not uncommon, due to linear ulcers crossed
with transverse folds. Linear ulcers are created from interconnected rows of aphthous
ulcers. CD is not cured surgically [1]. More than 50 % of children with CD require
surgery because of complications, failure of medical therapy or growth failure. Growth
failure is a common manifestation that is the result from both the decreased caloric
intake of the inflammatory bowel and the circulation of inflammatory cytokines [2].
Complications of CD include intestinal obstruction because of strictures, intestinal
perforation, bleeding or fistulas. The main goal of surgical therapy is the removal of
damaged bowel, maintaining the maximal amount of intestine possible. Other possible
operations include strictureplasty without bowel resection, segmental or subtotal
colectomy, and proctocolectomy with Brooke ileostomy. Proximal diversion alone does
not secure healing of the excluded segments of bowel [3].

It is not unusual to find segmental CD and frequently the rectum is spared of disease.
Bowel obstruction is a well-known complication of CD usually as the result of stricture
formation or more rarely as mechanical obstruction. Intestinal obstruction due to a large
pseudopolyp is a rare event in CD. These types of pseudopolyps rarely regress with
medical management alone, often requiring surgical resection [4]. There have been two
different types of pseudopolyps described in adult CD, one form that in seen in the large
intestine in Crohn colitis and a second form which is the nodular lymphangiectasia
occurring in the small intestine [5]. There have not been any descriptions of small
intestine pseudopolyps in children before.

Lastly, genetic testing of our patient showed a deletion of p53 and ATM genes and the
presence of the rearrangement of BCL6 gene. This means that he is at high risk of
developing a cancerous disease and may also develop malignant lymphoma and many
other types of cancer and solid tumours [6].

Conclusion

The coexistence of an intestinal pseudopolyp with undiagnosed Crohn's disease may be

the cause of acute abdominal obstruction in children.

Competing interests

The author(s) declare that they have no competing interests.

Authors' contributions

EC, EM, DF operated on the patient, MC and SK performed the diagnostic and
histological examinations, MP, NP and SR participated in the follow up and the
diagnostic strategy. All authors participated in writing the case report and revising the
draft.

http://search.creativecommons.org/?
q=Intestinal+pseudoobstruction+ARTICLE&sourceid=Mozilla-search

Fred A: Crohn's disease. In Pediatric Surgery. Volume 2. 6th edition. Edited by:
Grosfeld JL, ONeill JA, Fonkalsrud EW, Coran AG. Philadelphia: Mosby Elsevier;
2006:1453-1461.