Inflammatory Bowel Disease.

Inflammatory Bowel Disease

Three categories of inflammatory bowel disease
are en-countered in childhood:
1 Crohn’s disease
2 Ulcerative colitis
3 Inflammatory bowel disease of indeterminate
pathology.
 Crohn’s disease
Crohn’s disease is a chronic inflammatory
disorder of unknown etiology, which can affect
any part of the gastrointestinal tract from the
mouth to the anus. It is a transmural inflammatory
process that most commonly occurs in the
terminal portion of the small intestine and colon.
There is a high incidence of involvement of the
large bowel and rectum in pediatric patients.
 Incidence
Crohn’s disease is by far the most common
category of inflammatory bowel disease. During
the past 25 years, there has been a dramatic
increase in the incidence of Crohn’s disease in
Australasia and in the Northern hemisphere. This
disease was almost unknown in childhood before
1980. In contrast, the incidence of ulcerative
colitis has remained relatively static.
 Age of onset of symptoms
Most paediatric Crohn disease presents in
adolescence but one third of the patients are
aged 18 years or less at the commencement of
symptoms.
 Symptoms and signs
There is a broad spectrum of symptoms and signs
associated with Crohn’s disease. Most common
symptoms include recurrent abdominal pain and bowel
disturbance, usually diarrhea together with rectal
bleeding. However, these symptoms may be relatively
mild and the patient may present with the long-term
effects of the disease such as weight loss, growth failure
and delay of onset of puberty.
Delay in diagnosis often occurs because of lack of
knowledge by medical practitioners concerning the
relatively high incidence of Crohn disease in childhood
and the variety of non-specific presenting symptoms.
 Perineal inflammation
One of the commonest modes of presentation is
perineal inflammation, occurring in one third of
pediatric patients with Crohn’s disease, and this
is invariably associated with rectal disease.
Accordingly, pediatric patients particularly
adolescents who present with a perianal abscess
and associated anal fistula should have a biopsy
of the abscess wall at the time of drainage, to
exclude underlying Crohn’s disease.
Extra-intestinal manifestations
of Crohn’s disease
Examples of extra-intestinal manifestations
include arthritis and erythema nodosum, which
may be presenting symptoms.
Unusual modes of presentation
of Crohn’s disease
Very occasionally, Crohn’s disease may present
with acute right-sided abdominal pain and
gastrointestinal disturbance, mimicking acute
appendicitis. The diagnosis is then made at
laparoscopy/laparotomy.
 Cheilosis
Uncommonly, the patient may present with
chronic inflammation of the oral cavity and lips,
so-called cheilosis, manifested by edema,
erythema and fissuring of the lips. Biopsy reveals
evidence of chronic inflammation including
granulomas consistent with Crohn’s disease.
Investigations
 Role of endoscopy
Endoscopy has a crucial role in diagnosis, initial evaluation and continuing
assessment of Crohn disease. Upper and lower (‘top and tail’) gastrointestinal
endoscopy with biopsies is the key investigation for the diagnosis and initial
assessment of the extent and severity of the disease. Colonoscopy together
with biopsy provide a good chance of diagnosing Crohn’s disease because
there is a high incidence of colonic involvement in pediatric patients with this
disease. Gastroscopy is performed as well as colonoscopy because
involvement of the upper intestinal tract is common. Endoscopy has an
important role in assessing the response to treatment and distribution of the
disease, and is performed at periodic intervals. In Crohn’s disease, the
inflammation is typically segmental, and the characteristic appearance is single
or multiple ulcers with the intervening mucosa appearing normal. In some
instances, the diagnosis may be made by serial biopsies even when the
macroscopic appearances are normal. Histological diagnosis depends on
demonstration of granulomas, in association with other chronic inflammatory
changes in the bowel wall.
 CT scan with oral contrast
This imaging is performed to assess the small intestine
that is not accessible to endoscopy. It also may provide
valuable information concerning disease in the colon.
Most commonly, abnormalities are demonstrated in the
terminal ileum, and often in the colon. Abnormal findings
include an irregular bowel contour, longitudinal ulcers
and fissures, narrowing of the lumen by edema and
separation of loops by mural thickening. There may be
evidence of stricture formation associated with dilatation
of the proximal bowel. Bowel loops may be displaced by
the presence of an inflammatory mass.
 MRI
This imaging is used to assess pelvic Crohn’s
disease. It provides information concerning
ischio-rectal and perianal suppuration. It also
demonstrates sphincter anatomy and distortion or
damage related to the inflammatory process.
 Laboratory tests
CBC is performed to detect evidence of anaemia,
and liver function tests are performed to exclude
associated liver disease.
Stool cultures are performed to rule out chronic
infection due to such enteric pathogens as
Salmonella, Shigella, Campylobacter and
Yersinia.
 Treatment
The aetiology of Crohn’s disease is unknown, so
treatment is directed at controlling the disease
rather than curing it.
 Medical treatment
High doses of oral steroids are used to induce a remission,
for example, Prednisolone, 2 mg/kg (maximum 60–75
mg/day) for 4 weeks, with gradual reduction to nil after 8
weeks. Sulphasalazine, to prevent relapses, is introduced
when the Prednisolone dose is down to 20 mg/day and is
built up to a dose of 50 mg/kg/day twice daily. It may be
necessary to continue low-dose Prednisolone (5 mg/day).
Immuran can be used in resistant cases. Metronidazole
may be helpful for perianal Crohn disease. Newer biological
therapies such as Infl iximab appear to be very
effective.
 Nutrition
Children with inflammatory bowel disease fail to
grow because of the disease, not its treatment,
and the reason the disease influences growth is
its effect on appetite and caloric intake. High
caloric dietary supplements, and occasionally
enteral tube feeding or parenteral nutrition, all
have their place in management.
 Surgical treatment
The indications for surgical treatment of Crohn’s
disease are as follows:
1 perianal disease;
2 intestinal complications;
3 acute abdomen – possible acute appendicitis.
 Perianal disease
The most common indication for surgical intervention in Crohn’s disease is
perianal disease. Perianal inflammation is invariably associated with rectal and
colonic Crohn disease. There may be extensive involvement of the soft tissues
of the perineum, scrotum, penis or vulval region. Skin-tags and anal fissures
are common and usually do not require surgical intervention. Perianal abscess
is also common and requires incision and drainage. There is usually an
associated anal fistula that may need to be laid open, but healing will often
subsequently be very slow. Occasionally, insertion of a seton suture along a
chronic fistula tract may be appropriate to facilitate drainage of an infection
associated with it and subsequent healing. More extensive suppuration may
occur with development of an ischio-rectal abscess. Appropriate surgical
management of this complication is drainage of the abscess and consideration
given to faecal diversion in the form of a colostomy or ileostomy to help control
the infection. Faecal diversion is usually effective in controlling the infection but
it does not necessarily influence the activity of the Crohn disease. The potential
end result of ischio-rectal sepsis is damage to the sphincters.
 Perineal disease
Occasionally, there may be extensive
involvement of the soft tissues of the perineum
extending into the scrotum, penis or vulval region.
This may be manifest by unsightly painful edema
and inflammation of the scrotal or vulval tissue.
The aim of surgical treatment for complicated
perineal and perianal Crohn disease is to control
infection and promote healing. In these regions,
healing is often delayed and chronic inflammation
may be protracted despite appropriate surgical
and medical treatment.
 Intestinal complications
These complications are due to transmural inflammation
and include the following:
• localised stricture formation;
• localised disease unresponsive to medical treatment;
• localised disease associated with growth delay and
often delay in pubertal development;
• inflammatory mass;
• intestinal fistulae;
• rectal stricture.
The aim of surgical treatment for these complications
is to preserve as much intestine as possible.
 Intestinal complications
Localised strictures may be treated by simple stricturoplasty (without loss of
bowel) or resection. An important role for surgery is resection of localised
disease associated with growth delay or delay in pubertal development, or
both, despite maximal medical treatment. In the majority of these patients, a
sustained remission can be expected, together with catch-up growth and
resumption of normal schooling. The best results can be anticipated with
resection of localised ileo-caecal disease before or at the onset of puberty.
Resection may be necessary for inflammatory masses and fistulas. There is a
high incidence of rectal strictures in pediatric Crohn’s disease. It is often
associated with perineal inflammation. The management of this complication
includes steroid therapy, both systemic (as previously mentioned) and local
steroid medication as administered in enema form. The surgery includes
regular dilatations of the stricture under general anesthesia, usually conducted
in association with endoscopic evaluation. The associated perineal
inflammation tends to resolve with control of the rectal stricture.
 Ulcerative colitis
Ulcerative colitis is a chronic inflammatory
disease of the rectal and colonic mucosa, the
etiology of which is unknown.
 Clinical features
The onset is usually insidious, but an acute onset similar to a Salmonella
infection can occur. The onset of the disease is usually after 5 years of age, but
can be as early as the first year of life.
The typical features are as follows:
1 unexplained bloody diarrhea, with mucus, lasting more than 2 weeks;
2 anemia;
3 fever;
4 weight loss.
All degrees of severity are encountered, and the predominating symptom varies
from one patient to another.
Perianal complications occur in between 10% and 20% of patients, and include
ulcers, abscesses and fistulas. As in Crohn’s disease, perianal complications
may be the presenting problem, but more usually the development of perianal
disease is preceded by a period of diarrhea.
Investigations
 Colonoscopy
Colonoscopy accurately assesses the extent of macroscopic
disease and biopsies taken at colonoscopy achieve the diagnosis.
In ulcerative colitis, infl ammatory changes are seen in the rectum
and extend for varying distances proximally in the colon. The
changes range in severity from loss of the normal mucosal sheen
and vascularity with associated mucosal friability to diffuse
ulceration with blood and pus in the lumen. Numerous biopsies
taken during colonoscopy will confirm the histological diagnosis
and indicate the severity of inflammation at various levels. The
histology can be reported, at best, as ‘consistent with’ ulcerative
colitis, for there is no pathognomonic lesion.
In some instances, even when macroscopic appearances at
endoscopy are normal, multiple biopsies will provide diagnostic
histolological changes.
 Contrast imaging
This investigation may show a ‘saw-tooth’ or
marked irregularities in the mucosa, with deep
ulceration. Later, the colon becomes narrow, rigid
and devoid of visible peristalsis or haustration.
Finally, there may be pseudopolyps or stenosis
due to the development of a fibrous stricture.
 Other tests
Other tests include an CBC, to demonstrate
anaemia.
Bacteriology tests should include a careful search
for enteric pathogens, including Salmonella,
Shigella, Campylobacter and Yersinia.
Blood for Yersinia antibodies should be collected.
 Course of the disease
Improvements in medical treatment, with more
aggressive use of steroids, have produced better
control of the disease. Many children have
remissions lasting several years, to the extent
that the diagnosis is subsequently questioned. A
small proportion continue to have recurrent
lapses, and may require colectomy.
 Risk of malignancy
The incidence of carcinoma is directly
proportional to the duration of the disease. The
risk in the first 10 years of disease is very low.
After 10 years of disease, the rate increases by
10% for each decade. Surveillance by regular
colonoscopy and detection of dysplasia by
biopsies as a predictor of potential malignancy is
an important component of management of
paediatric patients with ulcerative colitis.
Colectomy is recommended after 10 years of
proven disease.
Medical treatment of ulcerative
colitis
The principles of medical treatment are similar to
those for Crohn’s disease. These principles have
been enumerated earlier. However, many of the
drugs for ulcerative colitis can be given as
enemas.
 Surgical treatment of
ulcerative colitis
Procto-colectomy is curative, but other surgical procedures have a
place in the treatment of this disease. The indications for surgical
treatment are as follows:
1 Severe inflammation unresponsive to medical treatment;
2 Severe disease associated with growth delay and delay in
pubertal development;
3 Long-term risk of malignancy;
4 Acute haemorrhage;
5 Perforation;
6 Toxic megacolon.
It is most important to realize that absence of symptoms should
not be taken as evidence of quiescent or inactive disease, or of
healing. Surveillance by regular colonoscopy should continue in
proven cases of ulcerative colitis.
 Surgical options
Subtotal colectomy with ileo-rectal anastomosis: This
procedure may be considered in a situation where there
is minimal rectal inflammation, particularly in an
adolescent entering young adulthood. This procedure
may avoid the need for an ileostomy. However, this
option requires continuing endoscopy surveillance,
together with biopsies every 6 months. Removal of the
rectum will be necessary after 10 years of disease.
Procto-colectomy: This procedure may be achieved with
a number of techniques as follows:
• ileo-anal anastomosis with ileal reservoir;
• ileo-anal anastomosis without reservoir – Soave
pull-through procedure.
Chronic inflammatory bowel disease
of indeterminate pathology
In a small number of patients, the pathology is
uncertain, and a diagnostic dilemma arises as to
whether the patient has ulcerative colitis or
Crohn’s disease. The principles of medical
treatment are similar to those outlined earlier in
this chapter. The principles of surgical treatment
depend on what is considered to be the most
likely condition as judged on clinical, endoscopic
and histological evidence.
Sign Crohn's disease Ulcerative colitis

Terminal ileum involvement
Crohn’s disease
Commonly Seldom

Colon involvement Usually Always

Rectum involvement Seldom Usually

Involvement around
Common Seldom
the anus
No increase in rate of primary
Bile duct involvement Higher rate
sclerosing cholangitis
Patchy areas of inflammation Continuous area of
Distribution of Disease
(Skip lesions) inflammation
Deep geographic and serpiginous
Endoscopy Continuous ulcer
(snake-like) ulcers
May be transmural, deep into
Depth of inflammation Shallow, mucosal
tissues
Stenosis
Common Seldom

Granulomas on biopsy May have non-necrotizing non- Non-peri-intestinal

peri-intestinal crypt granulomas crypt granulomas not seen
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