simplified

1. Approach to child with
Inflammatory bowel disease
By Dr. Irum Siddiquie
PGR Pediatrics BBH

2. Inflammatory bowel disease (IBD),
idiopathic chronic intestinal inflammation
that encompasses two related but distinct
disorders of as yet unknown aetiology,
characterized by unpredictable
exacerbations and remissions.

3. Inflammatory bowel disease
Ulcerative colitis (UC)
A chronic idiopathic inflammation of the rectum
extending continuously over a variable length of
the colon from the distal to the proximal end.
Crohn’s disease (CD)
A chronic, idiopathic transmural inflammation
which can affect one or several segments of the
digestive tract
Indeterminate colitis (IC)
It is reserved for cases of colitis in which
findings are not sufficient to allow differentiation
between CD and UC.

4. Bimodal distribution:-
• An early onset at 10-20 yrs of age
• Second smaller peak at 50-80 yrs of
age

5. Classified according to age
 Pediatric onset (<17yrs)
 Early onset (<10yrs)
 Very early onset (<6yrs)
 Infant/toddler onset (0-2yrs)
 Neonatal onset IBD

6. Etiology
• Frequency as high as 40% in 1st degree relatives.
• Monozygotic twins have 37% concordance for chron’s disease and
10%for ulcerative colitis.
• The 2 disease can occur in same family.
Genetic predispositions
• Mutations in NOD2/CARD15 gene on chromosome 16 have been
implicated in CD ,this gene has an important role in activating
nuclear factor-k.
• Genetic disorders have been associated with IBD including turner,
hermansky-pudlak syndrome , glycogen storage disorder type 1 b and
various immunodeficiency disorders.
Immune system disturbances
• Smoking
• Breastfeeding
• Diet
• gut microbiota
Environmental triggers

7. Definitions

8. Ulcerative Collitis
• Localized to colon and spares upper gastrointestinal tract.
• Begins in rectum and extends proximally for variable distance.
UC is characterized by diffuse mucosal inflammation limited
to the colon.
• Disease extent:
 “Distal’’ disease refers to colitis confined to the rectum
(Proctitis) or rectum and sigmoid colon
(Proctosigmoiditis).
 “Extensive disease” includes left sided colitis (up to the
splenic flexure), extensive colitis (up to the hepatic
flexure), and pancolitis (affecting the whole colon)
• Approximately 50-80% of pediatric patients have extensive
collitis adults more commonly have distal disease.

10. Chron’s Disease
• (Regional enterits, Regional ileitis, Granulomatous collitis)
• CD is characterized by patchy, transmural inflammation,
which may affect any part of the gastrointestinal tract.
• It may be defined by:
 Location (terminal ileal, colonic, ileocolic,
uppergastrointestinal),
 Pattern of disease (inflammatory, fistulating, or
stricturing).
• Compared to adult onset pediatric chrons is more likely to
have extensive involvement.

11. At initial presentation:
• 50% have ileocolitis
• 20% have exclusive colonic disease
• 30% upper GI involvement
Isolated colonic disease is common in children
younger than 8 yrs of age and may be
indistinguishable from ulcerative colitis.
Anatomic involvement tends to extend over time
in children.

12. How to Approach?

13. History
A complete history should include:
• Detailed bowel history with stool frequency,
consistency, urgency, and presence of blood, mucus
or pus per rectum.
• Abdominal pain, malaise, fever, weight loss.
• Recent travel.
• Medication history.
• Dietary and family history.
• Symptoms of extra intestinal manifestations of IBD
(joint, cutaneous, and eye) should be sought.

14. Complete Physical Examination
General examination includes
• General Wellbeing
• Weight and height centiles
• Pubertal status using Tanner staging
• Pulse rate, bloodpressure, temperature
• Abdominal examination for tenderness,
distension, masses.
• Inspection of perianal area for skin tags,
fissures, ulcers and/or oedema suggesting CD.

15. Common symptoms
Symptoms related to
inflammation of GI tract
Diarrohea /constipation
(inflammatory diarrohea
blood, mucus and pus in
stools)
Abdominal pain (cramping)
Rectal bleeding
Urgent need of bowel
movement(urgency)
Sensation of incomplete
evacuation (tenesmus)
Nocturnal bowel movements.
General symptoms that may
be related to IBD
Fever
Loss of appetite
Weight loss/growth
failure/failure to thrive
Fatigue
Night sweats

16. Extra-Intestinal
Manifestation of IBD

17. Extra-
Intestinal
Manifestat
ion of IBD
Skin and mucous
membranes
Oral lesion, chelitis
Apthous stomatitis
Glossitis
Peristomatis vegitans
Endocrine and metabolic
Delayed growth
Delayed sexual maturation
Thyroiditis, pancreatitis
Osteoporosis
Osteomalacia
Cardiac
Pleuropericarditis
Cardiomyopathy
Endocarditis
Myocarditis
Dermatological
Erythema nodosum
Pyoderma gangrenosum
Metastatic chron disease
Psoriasis
Epidermolysis bullosa
Acquisita
Perianal skin tags
Bronchopulmonary
Chronic bronchitis
with bronchectasis
Fibrosing alveolitis
Pulmonary vasculitis
bronchiolitis
obliterans
Granulomatous lung
disease
Ocular
Conjunctivitis,uveitis,
iritis
Episcleritis, scleritis,
chorioretinitis
Retrobilbar neuritis
Retinal vascular
disease
Malnutrition
Loss of appetite
Dietary restriction
Malabsorption
Bowel resection
Bile salt depletion
Bacterial overgrowth
Intestinal losses of
electrolytes mineral
and nutrients
Increase calorie
needs

18. Mode of onset
Gradual progression
Acute
Flminant
Fulminant collitis: Fever, severe anemia,
hypoalbuminemia , leucocytosis and more than
5 bloody stools per day for 5 days .
 Chronicity is important part of
diagnosis , symptoms beyond 1-2 wks
duration often proves to be
secondary to IBD
 More common
extraintestinal menifestations
•Pyoderma gangrenosum
•Sclerosing cholangitis
•Chronic active hepatitis
•Ankylosing spondylitis
 Clinical course
Remission relapse
Prolonged remission
(longer than 3 yrs)
Flare up with
Enteric infections or use
of NSAIDS
 Risk of colon cancer
After 8-10 yrs of
disease
And inc. by 0.5 – 1 % per
yr.
More
common with
Ulcerative
Collitis

19. Montreal classification of extent and severity of
ulcerative collitis
E1 (proctitis) Inflammation limited to rectum
E2 (left sided;
distal)
Inflammation limited to splenic flexure
E3 (pancolitis) Inflammation extends to proximal splenic flexure.
S0 (remission) No symptoms
S1 (mild) 4 or less stools per day (with or without blood),
absence of systemic symptoms, normal inflammatory
markers
S2 (moderate) 4 stools per day,minimum signs of systemic symptoms
S3(severe) 6 or more bloody stools per day,pulse rate ≥90 beats
per min,temp≥37.5˚C(99.5˚F), hemoglobin
concentration < 10.5 g/dl,ESR ≥30mm/hr

20. Pediatric Ulcerative Collitis Activity Index
Item Points
Abdominal pain
No pain
Pain can be ignored
Pain cannot be ignored
0
5
10
Rectal bleeding
None
Small amount only, in < 50% of stools
Small amount with most stools
Large amount (>50% of stool content)
0
10
20
30
Stool consistency of most stools
Formed
Partially formed
Completely unformed
0
5
10
Number of stools per 24hours
0-2
3-5
6-8
>8
0
5
10
15

21. Item Points
Nocturnal stools
No
yes
0
10
Activity level
No limitation of activity
Ocassional limitation of activity
Severe restricted activity
0
5
10
Sum of index (0-85)
Remission
Mild
Moderate
Severe
<10
10-34
35-64
65-85

22. Characterized by inflammatory, stricturing and penetrating disease.More
likely to have an obstructive pattern.
Systemis s/s , growth
failure with delayed
bone maturation and
delayed sexual
development can
precede other
symptoms by 1-2 yrs.
Right lower quadrant
pain with or without
mass. d/d :
• Appendicitis
• Mesenteric Adenitis
• Meckel Diverticulum
etc
 Complications:
• Perianal disease.
• (tags,fistulas, deep fissures,
abscess)
• Partial small bowel obstruction.
• Penetrating disease with fistula
formation.
• Entero-enteric.
• Entero-colonic.
• Entero-vesical.
• Enterovaginal .
• Enterocutaneous.
• Perianal abscess (usuallu painful)
• Perianal fistula.
• Psoas abscess.
 Bileacid malasorption
• Secondary renal stones
• Cholelithiasis
More common
with Chron’s
Disease

23. The Lémann Score
Crohn's Disease Digestive Damage Score.
• Portrays a patient's disease course on a
double-axis graph, with time on the x-axis,
bowel damage severity on the y-axis, and
the slope of the line connecting data points
as a measure of disease progression.
• This instrument could be used to assess the
effect of various medical therapies on the
progression of bowel damage.

25. Differential diagnosis

26. Infectious agents mimicking IBD
Bacterial
Campylobacter jejuni
Yersinia enterocolitica
Clostridium deficile
E-COLI 0157:H7
Salmonella
Tuberculosis
Shigella
Common in adolescence,may relapse
Common in adolescense as fevre of
unknown origin,wt loss,abd pain
Toxic megacolon possible
Hemolytic uremic syndrome
Usually acute
Can mimic chron disease
Dysentry symptoms
Parasites
Entamoeba histolytica
Giardia lamblia
Travel to endemic areas(trophozoites
in stool,colonic mucosal flask
ulceratio,serological test)
May be chronic(“owl like” trophozoites
and cysts I stool
AIDS Associated enteropathy
Cryptosporidium
Cytomegalovirus
Differ in mucosal findings
With immunosuppression

27. Chronic Inflammatory-like intestinal disorders
Vascular-ischemic disorders
Systemic vasculitis(SLE,dermatomyositis)
Henoch-schonlein purpura
Hemolytic uremic syndrome
Granulomatosis with angitis
Immune-inflammatory
Severe combined immunodeficiency disease
Rheumatoid rthritis
Chronic granulaomatous disease
Wiskott-aldrich syndrome
Bechet disease
Autoimmune enteropathy
Microscopic colitis
Others
Hypopituitrism
Glycogen storage disease type 1b
Dystrophic epidermolysis bullosa
Radiation collitis
Neonatal necrotizing enterocollitis

28. Diagnosis
• Abscense of an identifiable cause.
• Chronicity of >2-3 wks when infection has
been excluded.
• Typical endoscopic and histological findings
provide confirmation of diagnosis.

29. Baseline investigations
• No laboratory test is specific enough to
adequately and definitively establish the
diagnosis of IBD.
• Laboratory values may be used as surrogate
markers for inflammation and nutritional
status.
• To look for deficiencies of necessary vitamins
and minerals.

30. Routine Blood and Stool Tests
Test Descriptive Name Helps to Diagnose
CRP C-reactive protein Inflammation (non-
specific)
ESR Erythrocyte
Sedimentation Rate
Inflammation (non-
specific)
CBC Complete Blood Count Anemia, infection,
inflammation
Electrolytes Sodium, Potassium,
Chloride, CO2
Dehydration
Liver Function Liver Enzymes Medication side effects,
PSC (primary sclerosing
cholangitis)
Vitamin B12 Anemia, nutritional
status
Vitamin D Bone mineral status
Calprotectin Stool protein Active intestinal
inflammation
Lactoferrin Stool protein Active intestinal
inflammation

31. Specialized Blood Tests
Test Descriptive Name Potential Usefulness
pANCA Perinuclear anti-
neutrophil antibody
Distinguishes UC from
CD
ASCA Anti-saccharomyces
cervisiae antibody
Distinguishes CD from
UC

32. Imaging Tests
Suspected IBD Location or Complication Possible Tests
Ileocolonic disease Colonoscopy,
SBFT/enteroclysis, CTE, MRE,
capsule endoscopy (CE)
Upper tract Crohn’s disease EGD-Upper GI Series (UGIS)
Perianal Crohn’s disease MRI-EUS
PSC (primary sclerosing cholangitis) ERCP
Pancreatic and bile ducts MRCP
Perforations, blockages, abscesses Plain X-ray and CT scan
Fistulography
(multiple biopsies should be obtained)

33. Abdominal Radiography
• If severe fulminant colitis
is present, edematous,
irregular colon with thumb
printing sign.
• Free air and evidence of
Toxic Megacolon, which
appears as a long
continuous segment of air-
filled colon greater than 6
cm in diameter, indicates a
surgical emergency.

34. Barium enema double-contrast
radiographic studies
• Segmental narrowing
• Fistula formation
• String sign (a narrow band of
barium flowing through an
inflamed or scarred area) in the
terminal ileum are typically
observed on radiographs
• Contraindicated in setting of
potential toxic megacolon

35. Severe colitis. The mucosa is
grossly denuded, with active
bleeding noted.
Narrowed lumen. Relatively
little active inflammation is
present, indicating that this
is a cicatrix stricture

36. Histopathology
• Terminal illeal biopsies.
• Help to exclude other diagnoses (e.g. TB, Behcet’s
syndrome, lymphoma, vasculitis).
• Assessing the extent of IBD.
In children from a population at high risk of TB, tissue
should be sent for TB culture

37. Treatment
• Medication is the first therapeutic option.
Main goals:
• To achieve remission.
• Maintain remission (prevent flare-ups of
symptoms).
• Improve quality of life.

38. Diet
• Ensure adequate caloric intake.
• A high-protein, high-carbohydrate diet with
normal amounts of fat is recommended.
• Decreased fiber may prevent symptoms during
active colitis or partial intestinal obstruction;
• Increased fiber may benefit mucosal health
via bacterial production of fatty acids when
disease is inactive.
• Lactose is poorly tolerated when disease is
active.
• Total parenteral nutrition for periods of 4–6
weeks may induce remission and promote linear
growth and sexual development.

39. Corticosteroids
• Moderate to severe CD and UC
• Prednisolone
• Effective first line therapy
• 1-2 mg per/kg/d (maximum 40-60mg a day)
• Full dose for 2-4 weeks until remission achieved
(until clinical remission)
• Thereafter gradual reduction of the dose over 4-8
weeks depending on the response.

40. • Other management strategies at induction
• Antibiotics
Metronidazole (7.5mg/kg/dose tds) +/- Ciprofloxacin
(5mg/kg/dose bd) for peri-anal disease.
• Aminosalicylates
Mesalazine 50-100mg/kg/d to maximum 3-4g/d
Sulphasalazine 40-60mg/kg/d may be effective in mild disease.
Topical Mesalazine is effective in mild to moderate left sided
colitis.
Regular 6 monthly blood monitoring of liver and renal function is
essential.
• Intravenous (iv) steroids in severe disease.
Hydrocortisone 2mg/kg qds (maximum 100mg qds) or
methylprednisolone 2mg/kg od (60mg/d maximum).

41. Immunomodulators
• Azathioprine
• 6-mercaptopurine
• Methotrexate concomitantly with folic acid.
Effective in those who have a poor response to
prednisolone or those who are steroid dependent.
A beneficial effect of drug may be delayed for 3-4
months after starting therapy, therefore is not helpful
acutely.

42. • Ensure adequate dietary intake of calcium
and vitamin D and if insufficient consider
supplement.
• Gastric acid suppression with Proton Pump
Inhibitors (e.g. Omeprazole) may be
required inthe presence of gastritis.

43. Infliximab
• Effective treatment for perianal and
enterocutaneous fistulae.
• Should be reserved for patients’ refractory
to other treatments.
• A pelvic MRI scan should be carried out to
exclude any abscess and to diagnose fistulae
before starting Infliximab.

44. Surgery – Abscess drainage, Fistulotomy,
Collectomy and Seton insertion may be
appropriate.
Indication:
Obstruction
Fistula
Heamorrhage/Perforation
Colitis
Neoplasia
Failed medical management

45. To summarize

47. Questions

48. A 12-year-old female child presents with an 8-month
history of bloody diarrhea, intermittent crampy
abdominal pain, persistent spasms of the bowel, and
stringy mucus in her stools. You suspect IBD, with the
likely diagnosis of UC. Which of the following will allow
you to differentiate it from Crohn’s disease?
• A. areas of affected and unaffected mucosa, “skip”
• lesions
• B. Transmural involvement on biopsy
• C. Involvement of the small bowel
• D. Pseudopolyps
• E. Fistula formation
• Answer D

49. You are assessing a patient diagnosed with ulcerative
colitis. The patient has an altered level of
consciousness, fever, and lower abdominal distension.
Which of these additional findings would confirm a
diagnosis of toxic megacolon?
• Bradycardia
• Splenomegaly
• Leukocytosis
• Constipation
• Answer c

50. A patient is prescribed a tumor necrosis factor
(TNF) blocking agent for the treatment of Crohn's
disease. Before beginning treatment, the you will
confirm the results of which of these diagnostic
tests?
• Stool culture
• Sigmoidoscopy
• Erythrocyte sedimentation rate
• Tuberculin skin test
• Answer D

51. Thank you!