PAEDIATRICS

CASE REPORT 2019

TITLE: A CASE STUDY OF CROHN’S DISEASE IN A CHILD

STUDENT ID: 18100753

CONSULTANT: DR FOWZY AL-DELAIMI

Statement of Originality:
This is to certify that to the best of my knowledge; the content of this case report is my own
work and that all sources used for my discussion have been acknowledged.
 PATIENT HISTORY
I. Chief Complaint

EM, a 12-year-old female, presented to the Emergency Department in University

Hospital Galway on 8th October 2019 on a background of Crohn’s Disease with a
2-week history of abdominal pain associated with per-rectal (PR) bleeding.

II. History of Presenting Complaint

 The abdominal pain started 2 weeks ago with a pain scale of 5/10 and
progressively got worse 2 days ago with a pain scale of 9/10. The pain is
cramping in nature, most severe at the umbilicus region and radiates to the
right iliac fossa. It is relieved by placing a bottle filled with warm water
and there are no aggravating factors.
 The PR bleeding started 2 days ago and lasted for only 1 day. There is a
total of 4-5 episodes with about 1 teaspoon volume of blood each. It was
dark red in colour however occasionally bright red. It is not associated
with her stools. There was no pain involved.
 She has soft and watery stools about 8 episodes a day.
 Fever of 39.2 oC 2 days ago.
 Weight loss of 2kg in 2 weeks.
 She had nausea, and one episode of vomiting which is red and bright
orange in colour.
 Painful mouth ulcers that has been there since young.
 2-day history of frontal pounding headache.
 Erythematous rashes on the medial aspects of both her thighs.
 Reduced appetite
 Sore lower back

III. Birth History

 Mother had carpal tunnel syndrome during pregnancy, but it has resolved
after pregnancy
 EM was delivered by a Lower Segment Caeserean Section (LSCS) on 39
weeks gestation because she had a breech presentation and on doctor’s
recommendation because mother underwent eye surgery prior to delivery.
 Birth weight of 3.6kg
 No admission to NICU or any other complications.

IV. Vaccination History

 EM is up to date on all her vaccinations.

V. Developmental History

 No delays in development since birth. Hit all milestones.

 She is doing well in school, currently in Sixth Class, with good social
circle and enjoys learning.
 She goes to cooking classes on weekends and shares the food with her
family has a good social relationship with her family.

VI. Past Medical History/Medication History

 Diagnosed with Crohn’s Disease in August 2016. Treated with 6-MP,

infliximab and Humira. Had to change medications because 6-MP and
infliximab was not working effectively. Humira was just started 4 months
ago but EM feels there is no significant improvement.
 Cold sores at age 2, treated with 6-MP.
 Calpol OTC
 IV Iron Transfusion 4-5 months ago, due to low iron levels and having
blurry vision.
 She had an infection of her skin 5 weeks ago and was treated with
flucloxacillin.
 Undergoes physiotherapy to straighten back and correct posture due to
lower back pain.
 NKDA

VII. Family History

 Grandfather on paternal side has Crohn’s disease.

 Father has some sort of irritable bowel during young age. Diagnosed and
had a cystectomy for kidney cancer 4 years ago.
 Mother is healthy.

VIII. Social History

 Eats healthy food, particularly berries.

 Loves spicy food but unable to eat them because of her Crohn’s disease.
 Loves sports but unable to do them without a toilet nearby, because any
sort of physical activity will cause an urge to defecate and she is unable to
hold it for longer than 1 minute.
 Loves dancing and can go for dancing classes because toilet is nearby.
 EM must miss class often because she gets exacerbations from her Crohn’s
disease. If she misses class, there will be a teacher who will come visit
EM’s home for a few hours to teach her the lessons she has missed.
  She has tremendous teacher support who understands her condition and
takes care of her, and bends rules for her.
 EM lives with her father and mother and is being taken care by her mother
(housewife) at home.
 There are no smokers in the household, and she has 2 cats.

PHYSICAL EXAMINATION
I. Vital Signs

 Temperature: 36.8 oC [CHARTS]

 Heart Rate: 96 beats per minute
 Blood Pressure: 112/78 mm Hg [CHARTS]
 Respiratory rate: 20 breaths per minute
 Capillary Refill Time: < 2s

II. Growth Parameters

 32kg – 9th centile

 143cm – 25th centile

III. General Inspection

 EM was alert, conscious, responsive and lying flat comfortably on one

pillow.
 She does not appear to be in any pain or respiratory distress.
 She appears pale in colour.
 There is no IV access.
 There are marked erythematous rashes throughout the limbs and face.
 There are no signs of dysmorphic features.
 There are no medications around the bed, and EM is not on oxygen or IV
fluids.

IV. Gastrointestinal Examination

 Hands/Arms: Red rashes of the hands and arms, not painful. There is
muscle wasting of both her thenar muscles. No leukonychia, no koilonychia,
no pallor of palmar creases, no clubbing, No scratch marks.
 Face: No yellow discolouration of the sclera, no conjunctival pallor. Red
circular lesions, 1cm diameter, around the lower mouth. Angular stomatitis
is present. Presence of 4 ulcers in the mouth.
 Abdomen: On inspection, no distension/no striae/no distended veins/
inverted umbilicus. On palpation, tenderness on all 9 quadrants with muscle
guarding, most felt in the right iliac fossa. There is rebound tenderness on
 the McBurney’s point. No palpable masses felt. On percussion, no shifting
dullness or organomegaly noted and abdomen is tympanic. On
auscultation, bowel sounds are present and normal. There are no bruits
heard.

V. Cardiovascular Examination
 S1 and S2 sounds are heard, no murmurs or added sounds.

VI. Respiratory Examination

 Air entry good and equal on both sides.
 Vesicular breath sounds heard.
 No wheeze, crepitations, stridor, or added sounds.

SUMMARY OF CASE
EM is a 12 year old female child who presented to the Paediatric Emergency Department
with complains of abdominal pain for the past 2 weeks, associated with PR bleeding, soft
watery stools, fever, weight loss, nausea and vomiting, painful mouth ulcers, erythematous
rashes on her thighs, reduced appetite, and sore lower back, on a background of Crohn’s
Disease. On examination, there was generalized erythematous rashes on her limbs and face,
muscle wasting of thenar muscles, red lesion below her lower lip, angular stomatitis and
mouth ulcers. Abdominal palpation yielded generalized abdominal pain and positive rebound
tenderness on McBurney’s point. Otherwise, all other examinations are unremarkable.
 WORKING DIAGNOSIS
Diagnosis: Acute Exacerbation of Crohn’s Disease

A study done by Andrew S Day et al. discussed Crohn’s disease in children and adolescents.
It is outlined that Crohn’s disease classically presents with abdominal pain, diarrhoea and
weight loss [1]. EM has complaints of abdominal pain in her history and tenderness was also
elicited during abdominal soft palpation. Children can also present with extra-intestinal
manifestations such as axial arthritis, skin changes such as erythema nodosum, eye disease
and liver disease [1]. EM had erythema nodosum on history and physical examination and a
history of axial arthritis which supports my working diagnosis. Besides that, EM had multiple
mouth ulcers elicited on both history and physical examination which are an example of an
oral manifestations of Crohn’s disease [2].

EM had been diagnosed with Crohn’s disease in 2016, and since then she was managed with
6-MP, infliximab, and Humira. Maintaining remission of Crohn’s disease is exceedingly
tough, especially in EM’s case whereby she has not found a drug that works well for her. A
study done by L. Peyrin-Biroulet et al. shows that the remission rates using adalimumab
(Humira) in Crohn’s disease ranges from 40-47%, which means that there is more than 50%
chance for an acute exacerbation of Crohn’s disease to occur despite treatment [3].

Table 1: Remission rates using medical treatments in luminal Crohn's Disease in maintenance randomised, controlled trials
[3]

DIFFERENTIAL DIAGNOSIS
I. Acute Appendicitis
Patients with acute appendicitis presents with abdominal pain in the umbilicus
region which radiates towards the right iliac fossa [4]. Not only that, anorexia,
nausea and vomiting are also prevalent [4]. Also, rebound tenderness in the right
iliac fossa at McBurney’s point is indicative of acute appendicitis. EM had all of
these. However, the timeline of acute appendicitis does not suit here very well.
The severity progression in EM’s abdominal pain from 5/10 to 9/10 should have
taken 1-2 days if it were acute appendicitis, not 12 days. Bloody diarrhoea is also
not indicated in acute appendicitis. Hence this is an unlikely although possible
diagnosis.
 II. Acute Gastroenteritis (AGE)
Patients with AGE presents with loose, watery stool of more than 3 episodes in 24
hours and lasts for 2-7 days depending on its aetiology [5]. There may be blood or
mucus in the stool [5]. AGE also presents with vomiting, nausea, abdominal pain,
headaches, and body temperatures of 38-39oC [5]. EM fits all the criteria above
except for the timeline because she has had this pain for 2 weeks. AGE does not
present with mouth ulcers and rarely presents with erythematous rashes [6].
Hence, this is also a far more unlikely diagnosis as compared to an acute
exacerbation of Crohn’s Disease.

MANAGEMENT PLAN
 EM was seen by Paediatric GP in the Emergency Department.
 Measure body temperature, weight, height, blood pressure, heart rate, respiratory rate
and SpO2.
 Take FBC, CRP, U&E, LFTs, Blood Gas, Serum Amylase.
 Admission to St Bridget’s ward.
 Input from physiotherapist, dermatologist and consultant paediatrician Dr Al-Delaimi.

PATIENT FOLLOW-UP
8 October 2019
Test Results Increased/Decreased Normal Range
Red Blood Cells 4.2 3.8 – 5.3 x 1012/L
White Blood Cells 6.9 5 – 17 x 109/L
Haemoglobin 13.4 11 – 15 g/dL
Platelets 490 Increased 150 – 450 x 109/L
CRP 54 Increased <3.0 mg/L
Sodium 142 136 – 145 mmol/L
Potassium 4.2 3.5 – 5.0 mmol/L
Calcium 1.26 Decreased 2.2 – 2.7 mmol/L
Chloride 102 95 – 105 mmol/L
Amylase 27 23 – 85 U/L
Table 2: Blood test results of EM. [CHARTS]
9 October 2019
 EM still has abdominal pain rated 4/10, along with nausea and fever. She does not
have PR bleeding. On examination, she still had right iliac fossa pain on palpation but
did not have any rebound tenderness. Otherwise, everything was the same as on 8th
October 2019.
 EM was seen by a dermatologist. She was prescribed the following:
a. Silcocks base topical to be applied twice a day.
b. Dermova to be applied OD for 2 weeks.
c. Hydrocortisone topical ointment for 2 weeks.
d. Betnovate RD OD
 She was given Odansetron 8 hourly PRN 4mg PO.

10 October 2019
 EM did not have abdominal pain, nausea and fever on history. On examination she
had slight tenderness on deep palpation over the umbilicus and right iliac fossa.
 Methotrexate 20mg once weekly.
 Forsitip Compact 125ml chocolate banana flavor (Patient’s favourite)
 Paraffin gel QDS topical
 Plan: Weekly FBC, U&E and LFTs in local GP to see tolerance towards
Methotrexate.

11 October 2019
 EM’s history of presenting illness was unremarkable. On examination, there was no
longer any pain elicited on palpation. Otherwise, examination was normal apart from
rashes which did not improve.
Discharge Arrangements
 Methotrexate increased dosage to 80mg. If tolerating well and normal FBC, U&E and
LFTs, then discharge with weekly visit to GP.
 DISCUSSION
I. Crohn’s Disease – Prevalence, Pathogenesis, Effect on growth, Severity,
Investigations, Management, and Other Complications.

Crohn’s disease is a subset of Inflammatory Bowel Disease (IBD) that affects

mainly adults and very rarely, children. Josef Sykora et al. in an article showed
that the highest annual incidence of Crohn’s disease in Europe is 12.3/100000
person-years [7].

Despite an immensely complex aetiology, the most widely recognized hypothesis

of Crohn’s Disease is the disease behaves as an immune-mediated condition in
genetically susceptible patients who were triggered by environmental factors,
resulting in mucosal barrier alterations, imbalance gut microbiota, and abnormally
stimulated gut immune response [8].

Crohn’s disease has a significant impact on the growth of the child. Weight loss is
the presenting complaint in 85% of children with Crohn’s disease and this results
from reduced oral intake from anorexia, painful ulcers in the mouth, odynophagia,
nausea or early satiety, which is seen in the case of EM [1]. The reduced oral
intake results in malnutrition and hence delayed pubertal development, which
results in delayed pubertal growth spurt and reduced potential height [1].
Micronutrient deficiencies such as iron, vitamin D and calcium are common in
children with Crohn’s disease. EM was deficient in Iron and she was subjected to
IV Iron infusion 4-5 months ago [1].

When managing Crohn’s disease, it is imperative to understand the severity of the

disease in the child. Therefore, the Crohn’s Disease Activity Index (CDAI) can be
used to quantify symptoms faced by patients to assess the current severity of the
disease [9]. EM’s CDAI at the Emergency Department would be under 150 so she
should be in the remission stage.
Figure 1: Crohn's Disease Activity Index (CDAI) [9]

According to the ECCO guidelines, diagnosis of Crohn’s disease is based on a

plethora of investigations, namely clinical, biochemical, stool, endoscopic and
histological investigations [10]. Laboratory investigations include FBC
(thrombocytosis, anaemia and leukocytosis), CRP (raised), and albumin
(decreased) [10]. The most sensitive marker of inflammation in Crohn’s disease is
fecal calprotectin which rises with disease activity [10]. If these investigations
yield any result that raises suspicion of Crohn’s disease, an ileocolonoscopy
should be performed [10]. In EM’s case, a large part of these investigations was
not done, probably because she has a background of Crohn’s disease.

Once the diagnosis of Crohn’s Disease has been confirmed, it can be managed by
first inducing remission, then maintaining remission. Drugs that are suitable for
inducing remission include monotherapy with a glucocorticosteroid
(prednisolone), adding on 6-MP if there are two or more inflammatory
exacerbations in 12 months [11]. This was done for EM in the past, however, it
was not effective in inducing remission. Hence, the next line in the NICE
guidelines mentions the use of infliximab, which did not work either, and now
adalimumab, which is currently being used [11]. Methotrexate was used in EM to
 induce remission in the ward because all other medication does not seem to be
working.

Some of the complications in Crohn’s disease are more detrimental if present in

children. Growth failure occurs in about 40% of children with Crohn’s disease
[16]. This is caused by decreased oral intake, malabsorption and raised metabolic
demands [16]. An adult height of less than 8cm of expected height is present in
19% of children with Crohn’s disease [16]. Hence, measuring height and plotting
the height to monitor centiles is extremely important. Apart from disruptions in
vertical bone growth, children with IBD may not have optimal bone mass when
they become adults. Hence, they are subjected to vertebral compression fractures
[16]. Treatments should be directed at restoring proper bone growth. In children
with complicated IBD such as growth impairment, delayed puberty or use of
corticosteroids, dual-energy x-ray absorptiometry of the whole body is
recommended [16]. 1000mg Calcium and 800 to 1000 IU of Vitamin D is
recommended for children [16].

Besides growth issues, children with IBD has an increased risk of developing
colorectal carcinoma. This is due to chronic inflammation and the cumulative
incidence of colorectal carcinoma in patients with IBD is 13 per 1000 patients
[16]. Therefore, Crohn’s disease causes very serious complications in children and
must be addressed adequately. So, after the 7th year of diagnosis, colonoscopy
with surveillance biopsy must be done every 1-2 years [16].

II. Psychological issues in IBD and Paediatric Crohn’s Disease on the child.

According to M. S. Sajadinejab et al., stress is an aggravating factor towards the

course and symptoms of IBD and is a precursor of disease relapse [12].

Figure 2: Direct and indirect mechanisms by which the course of IBD can be influenced by stress [12].
 IBD, once developed, can cause psychological concerns to the patients due to its
nature of being unpredictable, and its chronic course. Some of these psychological
concerns would include soiling due to loss of bowel control, impairment of body
image due to excessive weight loss and erythema nodosum. All of these would
result in social isolation and a loss of self-unworthiness [12].

A study done by G. van den Brink et al. on the prevalence of anxiety and
depression symptoms in 374 children with IBD showed anxiety, depression or
both, was found in 28.6%, 3.0% and 15.9% respectively [13]. The study
concluded that active disease was a significant risk factor for both anxiety and
depression, or either [13].

When I clerked EM, she mentioned that her pain was the most distressing thing
amongst all her symptoms. It disables her from concentrating in class, or at home.
The pain causes her to miss school often and she does not like missing school.

Paediatrics CDAI (PCDAI) used in a study showed that patients in a flare of IBD
reported higher levels of disability, bowel symptoms, anxiety, and reduced quality
of life as compared to those in remission [14]. As for the pain, patients who are in
remission from the disease who presents with abdominal pain claims higher
disability and lower quality of life [14].

III. Psychological issues in IBD and Paediatric Crohn’s disease on the parents.

The pain that EM experiences also worries her father immensely, particularly
when he just got cleared of his kidney cancer not too long ago. Her father
mentioned that it is very tough taking care of her mentally because of all the stress
he has got over the recent years, but he enjoys doing it.

It is evident that when a child gets diagnosed with a chronic disease like Crohn’s
disease, the entire family will be affected. The duties of dosing schedules and
administering multiple medications must be incorporated into the parents’ daily
routine. That is not an easy task. Since acute exacerbation of Crohn’s is common
and frequent, parents would have to reschedule appointments or take emergency
leave from work. The reason behind EM’s father having increased stress could be
because he is living in this state of increased uncertainty. On the other hand, he
could also be stressed because he worries about the role IBD will play in
moulding EM’s future [15].
 REFLECTION
I learnt a lot from when I started taking a history from EM and her father until now when I
am reflecting upon it.

When the Paediatrics GP in the Emergency Department told me to clerk this case as it was a
good one for a case report, I panicked because I knew very little about Crohn’s disease and
was not sure about the extra-intestinal manifestations that a patient could present with.
Nevertheless, I knew I had to act quick and talk to EM and would not be able to do a quick
search on my phone because I did not know at the time about the management plan for EM
and if she was going to be discharged. I felt afraid and nervous when talking to EM and I had
to ask her to repeat some details of her history just to clarify because I did not hear them very
well. During history taking, something struck me – I did not need to know the extra-intestinal
manifestations prior to taking a history, all I needed to know was to follow the structure of
systems review taught to us and I would have a good chance of nailing these associated
symptoms, and I did. I felt more confident and the rest of the history taking, and physical
examination went smooth. I will continue to be more confident in the future as my knowledge
widens. I learnt that even if I do not know a disease and how it presents, extracting these
signs and symptoms would be exceptionally helpful when presenting the case to a consultant
because they can give their input on the possible diagnosis. Also, I have learnt how Crohn’s
disease presents and how to diagnose and manage it in a child throughout this experience.

Besides that, when I was taking the history from EM, I learnt that it is best to go on a tangent
and talk about whatever she wants to for a short while. This is to maintain a good rapport and
to ensure that she is still interested talking to me. I realized that simple gestures such as
waving “hello” or smiling does a great deal in making the child more comfortable. In the
future, I will try my best to engage in what the child is interested in, while using simpler
grammar and vocabulary so that the child would understand better.

When talking to EM and her father about the impacts of this chronic disease on their lives
and how they are coping with it, I felt very sad and empathetic because they sounded really
stressed out and sad when discussing it when me. I learnt to console them about it and tried to
be more understanding by telling them that they are doing a great job so far. I think this is
effective in making the patients more comfortable talking to me and it will keep the session
going on longer. Therefore, there is no doubt that I will continue to be empathetic toward
patients in general because this encounter with EM strengthens the reason behind it.

To conclude, talking to EM and her family has widen my horizon not only on a medical
knowledge aspect, but also more on professionalism and approach to children. I will continue
to work hard and apply what I have learnt in my long journey towards being a successful
doctor.
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