Crohn disease

Dr Craig Hacking and Dr Frank Gaillard et al.

Crohn disease, like ulcerative colitis, is an idiopathic inflammatory bowel disease (IBD),
characterised by widespread gastrointestinal tract involvement typically with skip lesions, thereby its
synonym regional enteritis, and frequently systemic involvement.
Epidemiology
The diagnosis is typically made between the ages of 15 and 25 years of age, with no gender
predilection 5. There is a familial component and incidence also varies with geographical location.
Clinical presentation
Clinical presentation is typically with chronic diarrhoea and recurrent abdominal pain. Alternatively,
patients may present with one of the many complications or extraintestinal manifestations (see
below).
Pathology
Crohn disease remains idiopathic, although infective agents have been gaining in popularity as a
possible cause, including the measles virus and atypical mycobacterium. As there are definite
genetic factors at play, multiple factors are likely to contribute 1. Incidence is higher in people with
first relative having IBD and it reaches up to 10%, also there has been shown 30-50% chance of
developing disease in mono- or heterozygos twins.
Initially the disease is limited to the mucosa, with lymphoid hyperplasia, lymphoedema and shallow
aphthoid ulceration. As the disease progresses, the entire bowel wall becomes involved, with linear
longitudinal and circumferential ulcers extending deep into the bowel wall, predisposing to fistulae.
Inflammation also extends into the mesentery and over time leads to chronic fibrotic change, and
stricture formation 5.
Extraintestinal manifestations include 3,15-17:

anywhere along digestive tract, including mouth and oesophagus

o

mucogingivitis, mucosal tags, deep ulceration, cobblestoning, lip swelling and pyostomatitis
vegetans, esophageal ulcers and strictures to mention only a few of this Augian stable

skin
o

erythema nodosum

joints
o

arthritis (limb)

seronegative spondylarthritis

sacroiliitis (one of the most frequent extra-intestinal manifestation)

eyes
o

episcleritis

iritis

uveitis (acute anterior uveitis AAU)

liver and biliary system

o

pericholangitis

primary sclerosing cholangitis (PSC) (more common in ulcerative colitis)

autoimmune hepatitis

cirrhosis

gallstones: seen in 30-50% 8

hepatic abscess 8

pancreatitis

renal tract: renal calculi containing oxalate

o

pyoderma gangrenosum

poor fat absorption results in binding of calcium by fats, which in turn reduces the amount of
calcium that can bind to oxalate, therefore increasing the amount of unbound oxalate
available for resorption; this resorption occurs in the colon, and therefore patients with an
ileostomy do not have the same increased risk

pulmonary and thoracic associations

o

such as bronchiectasis, mosaic perfusion and air trapping just to name some of them

Radiographic features
The characteristic of Crohn disease is the presence of skip lesions. The frequency with which various
parts of the gastrointestinal tract are affected varies widely 5:

small bowel: 70-80% 5-6

small and large bowel: 50%

large bowel only: 15-20%

The choice of investigation modality depends on local expertise and availability. CT and MR
enteroclysis are similar in sensitivity for active inflammation (89% vs 83% respectively) and both are
somewhat better than small bowel follow-through (67-72%) 6. The lack of ionizing radiation from MRI
would make it a better option, however the availability of MRI is limited in many countries.
Barium small bowel follow-through

mucosal ulcers
o

aphthous ulcers initially

deeper transmural ulcers typically either longitudinal or circumferential in orientation

when severe leads to cobblestone appearance

may lead to sinus tracts and fistulae

2

widely separated loops of bowel due to fibro-fatty proliferation (creeping fat)

thickened folds due to oedema

pseudodiverticula formation: due to contraction at the site of ulcer with ballooning of the opposite site

string sign: tubular narrowing due to spasm or stricture depending on chronicity

partial obstruction

on control films presence of gall stones, renal oxalate stones, and sacroiliac joint or lumbosacral spine
changes should be sought

CT

CT examination can be carried out with both intravenous and intraluminal contrast (positive or
negative) 5:

fat halo sign

comb sign

bowel wall enhancement

bowel wall thickening (1-2 cm) which is most frequently seen in the terminal ileum (present in up to
83% of patients) 8.

strictures and fistulae

mesenteric/intra-abdominal abscess or phlegmon formation 8

abscesses are eventually seen in 15-20% of patients 8

CT is also able to give valuable information on:

perianal disease

hepatobiliary disease

MRI

MRI enterography has no ionising radiation and an ability to evaluate both mural and extramural
involvement. It has become an increasingly important part of management of patients with Crohn
disease. MRI enteroclysis may be attempted in select patients.
MRI enterography (MRE)

MR enterography can be a useful technique for evaluation of the bowel. Inflamed loops of bowel
demonstrate thickening and contrast enhancement.
Extramural disease is where MRI excels:

fibrofatty proliferation:
o

thickening of extramural fat, which separates bowel loops

equivalent to the fat halo sign on CT

vascular engorgement: comb sign

stenoses and strictures

Coronal cine sequences (bSSFP) can also be useful in diagnosis. Inflamed loops of bowel frequently
demonstrate decreased peristalsis.
MRI enteroclysis

MRI enteroclysis requires the placement of a nasojejunal catheter through which 1.5-2 L of contrast
solution (e.g. water with polyethylene glycol and electrolytes) are injected 2.
Spatial resolution is not as good as with conventional fluoroscopic enteroclysis, and thus minor
mucosal changes are not apparent. When disease is transmural, with cobblestone appearance, the

abnormalities are evident as high T2 signal linear regions, provided adequate distension is achieved
2

Routine MRI

Routine MRI can also give valuable information:

perianal disease

liver disease

sacroiliac joints and spine

Ultrasound

Ultrasound has a limited role, but due to it being cheap and available and not involving ionizing
radiation, it has been evaluated as an initial screening tool 4. Typically examination is limited to the
small bowel and wall thickness assessed:

bowel wall thickness should be <3 mm

The usefulness of this finding needs to be interpreted in the context of pretest probability (i.e.
thickness of less than 3 mm helps exclude the disease in a low risk patient, and a thickness of
greater than 4 mm helps establish the diagnosis in a high risk patient) 4. As it has difficulty examining
the whole bowel, it is not appropriate as a true diagnostic test.
On doppler evaluation Increased SMA flow volume and decreased SMA resistive index (SMA RI) also
correlate with disease activity. Successful treatment may result in normalization of these imaging
parameters 12.
Ultrasound does of course have a significant role to play in the assessment of:

perianal disease: rectal ultrasound

hepatobiliary disease

Treatment and prognosis

Management is complex as the condition is chronic with a relapsing-remitting course.
Medical management includes corticosteroids, 5-ASA preparations, immunomodulation (e.g.
azathioprine, cyclosporin, methotrexate) 7.
Surgical management is reserved for complications including:

strictures

adhesions and bowel obstructions

fistula

perianal disease

History and etymology

It is named after Burrill Bernard Crohn, American gastroenterologist 11.
Differential diagnosis
The differential diagnosis depends on the presenting symptom. When terminal ileitis is the main
presentation, then differentials (adjusted for patient's age) include 1:

acute appendicitis

Yersinia ileitis

mesenteric adenitis

ileocaecal tuberculosis 9

malignancy

When colonic involvement is the predominant feature then other considerations include:

ulcerative colitis

acute diverticulitis

acute epiploic appendagitis

ischaemic colitis

pseudomembranous colitis

infectious colitis

Crohns Disease
Findings
Changes of early/intermediate Crohns disease, with thickened folds, tending to asymmetry and
obliteration in places. There are small apthous ulcers, and nodules, with normal diameter bowel.
There is a linear mesenteric ulcer (arrowed, lower image).

Case 48 - Figure 1

Case 48 - Figure 3

Diagnosis
Small bowel Crohns disease
Discussion
Early disease
Radiology and Pathology correlate well.
1. Thickened folds Generally regular and more symmetrical than intermediate Crohns.
Produced by hyperplasia of lymphoid tissue and obstructive lymphoedema in submucosa.
2. Thickened villi Adhere to one another producing a coarse villous pattern (granular/lumpy
mucosal surface).
3. Apthoid ulcers Produced by hyperplasia of lymph follicles in lamina propria and overlying
shallow mucosal erosions 1 3mm.
4. Affected villi produce increased mucous secretions.
Above features are non-specific for Crohns but, if 2 or 3 of the above are present, then Crohns
should be suspected. Above features are best seen with enteroclysis.
Intermediate disease
1. Widening of base of folds to cause partial or total disappearance secondary to increasing
submucosal oedema. This process mimics thumbprinting seen in ischaemia.
2. Development of distorted folds Secondary to fibrosis. Shortening on mesenteric border in
particular.
3. Deepening of ulcers Enlarged, rose thorn shape.
4. Typical linear ulcer on mesenteric border separated from adjacent submucosa by oedema.
5. Inflammatory polyps Nodular pattern. Polyps more common in colonic disease. Usually
found in area denuded of folds. In profile, polyps appear as notches into barium. Diameter

of bowel is not reduced differentiate from ulceronodular cobblestone pattern of

advanced disease.
Intermediate disease changes are more obviously asymmetrical with skip areas.
Advanced disease
Transmural disease reaches serosa and beyond.
1. Ulcers deep linear clefts and fissures. Islands of surviving mucosa produce pseudopolyp
cobblestone appearance. This pattern is always associated with lumen reduction. Large flat
ulcers derived from enlargement of apthoid ulcers.
2. Linear mesenteric ulceration extends caudad. Antimesenteric redundancy and pleating
disappears as disease extends transaxially around bowel lumen.
3. Thickened bowel wall (now seen on CT with inflammatory changes extending into
mesentery). Thickening secondary to fibrosis.
4. Complications:
a. Strictures.
b. Abscesses.
c.

Fistulas.

d. Perforation.
e. Carcinoma Increased incidence with small bowel disease. Affects younger patients.
Distal ileum (76%) usually in areas of long standing disease. Very difficult to diagnose
pre-operatively.