2: GI - Duodenal Atresia, Coeliac Disease, Hernias, Functional Abdominal Pain, Hirschsprung's, IBD Flashcards
1
Q
What is duodenal atresia
A
Complete occlusion of the duodenum
2
Q
What is duodenal stenosis
A
Narrowing of duodenum
3
Q
What condition is duodenal atresia associated with
A
Down’s Syndrome (30%)
4
Q
How will an infant with duodenal atresia present in-utero
A
Polyhydramnios
5
Q
Why does a foetus with duodenal atresia have polyhydramnios
A
Unable to swallow and process amniotic fluid
6
Q
What are neonatal features of duodenal atresia
A
- Billous post-partum vomiting
- Meconium ileum
- Distended upper abdomen
- Scaphoid lower abdomen
7
Q
Why is vomiting bilious in duodenal atresia
A
Obstruction after major duodenal papilla
8
Q
Why is scaphoid lower abdomen in duodenal atresia
A
No gas in abdomen beyond the obstruction
9
Q
What is used to investigate duodenal atresia
A
AXR
10
Q
What is seen on AXR
A
Double-bubble sign
11
Q
What is the double-bubble sign
A
Accumulation of gas before the stenosis and after the stenosis - giving appearance of two bubbles
12
Q
How is duodenal atresia managed
A
- Gastric decompression
- Parental nutrition
- Duodenostomy
13
Q
What is coeliac disease
A
Enteropathy caused by sensitivity to gluten
14
Q
When does coeliac disease typically present in children
A
4-6 months
- Introduction of solid foods
15
Q
What gene is most associated with coeliac disease
A
HLADQ2 (95%)
16
Q
Aside from HLA DQ2, what other gene is associated with coeliac disease
A
HLADQ8 (10%)
17
Q
What happens in coeliac disease
A
Gliadin triggers an auto-immune reaction
18
Q
How does coeliac disease present clinically
A
- Diarrhoea
- FTT
- Distended abdomen
- Everted umbilicus
- Irritability
- ANAEMIA
19
Q
What is a late presentation of coeliac disease
A
Wasting of the buttocks
20
Q
What are very late signs of coeliac disease
A
- Hypotonia
- Apathy
- Delayed puberty
- Gross motor developmental delay
21
Q
How is coeliac disease stereotypically identified on growth chart
A
4-6m (gluten introduced) will be crossing-down of centiles
22
Q
What is first-line investigation for coeliac disease
A
anti-TTG (IgA)
23
Q
What is second-line investigation for coeliac disease
A
anti-EMA
24
Q
What is third-line for coeliac disease
A
Total IgA
25
When is serology for coeliac disease less reliable
Under 18 months
26
What imaging should be ordered in coeliac disease
Endoscopy (OGD)
27
Explain management of coeliac disease
Gluten-free diet
28
What is a complication of coeliac disease
Increases risk of small bowel lymphoma, even if asymptomatic
29
What are the three types of hernias that may occur in children
Umbilical
CDH
Inguinal
30
What is an umbilical hernia
Protrusion abdominal contents through umbilicus
31
What can cause umbilical hernia
Increase intra-abdominal pressure
32
Describe clinical features of umbilical hernia
- Mass protruding through umbilicus
- Increased on increasing intra-abdominal pressure
- Can be easily reduced
33
What are two differential diagnoses of umbilical hernia
- Omphalocele
| - Gastrochisis
34
What is an omphalocele
Organs herniate through midline abdominal wall
35
When do majority of umbilical hernias resolve by
2-3 years
36
What are the TWO indications for repair of umbilical hernia
1. >4-years
| 2. > 1.5cm
37
What is gastrochisis
Paraumbilical herniation intestine
38
Will inguinal hernias in paediatrics be direct or indirect and why
Indirect - due to patent processes vaginalis
39
Will indirect inguinal hernias be medial or lateral to the inferior epigastric vessels
Lateral to inferior epigastric vessels
40
Describe position of inguinal hernias relative to pubic tubercle
Superior-medial
41
When are inguinal hernias most common in paediatrics
Before 1-year
42
What causes inguinal hernias
Patent processes vaginalis
43
What are 4 risk factors for inguinal hernias
- FH
- Male
- Pre-Mature
- Raised intra-abdominal pressure
44
What can cause raised intra-abdominal pressure
Prolonged ventilation
45
What is the main presentation of inguinal hernia in children
Intermittent swelling inguinal-scrotal region
46
What is the main risk with inguinal hernias
Strangulation
47
What is strangulation
Occlusion blood supply resulting in necrosis
48
How will strangulation of an inguinal hernia
- Irritability
| - Absolute bowel obstruction (no defectation or flatulence)
49
What are two factors that increase risk strangulation inguinal hernia
- Smaller hernia
| - Younger child
50
What is the main differential diagnosis of inguinalscrotal swelling, aside from inguinal hernia
Hydrocele
51
What is the main differentiating feature of hydrocele and inguinal hernia
Can get above a hydrocele, not a inguinal hernia
52
What two conditions does a patent process vaginalis predispose to
Inguinal hernia
| Hydrocele
53
If asymptomatic, how are inguinal hernias managed
Elective repair within 1-month
54
If incarcerated, how are inguinal hernias managed
Repair in 24-hours
55
If strangulated, how are inguinal hernias managed
Urgent herniotomy
56
If a boy under 2-years or girl more than 5-years with inguinal hernias and what is required and why
Laparoscopy - to investigate for contralateral hernia
57
what is a congenital diaphragmatic hernia
when portion bowel protrudes through diaphragm
58
what is the most common type of CDH
bochdalek
59
what is a bochdalek CDH
posterior-lateral hernia
60
what is a morgagni hernia
anterior hernia
61
in which gender is CDH more common
males
62
what are two causes of CDH
Idiopathic
| Failure diaphragm to form
63
how does congenital diaphragmatic hernia present clinically
- Respiratory distress
- Barrel chest
- Scaphoid abdomen
64
what are symptoms of respiratory distress
- Breathlessness
- Cyanosis
- Grunting
- IC recessions
65
why does respiratory distress happen in CDH
as abdominal contents in the mediastinum - prevent growth of the lungs
66
what are two signs of CDH on auscultation
- absent breath sounds unilaterally
| - bowel sounds in the chest
67
which side is CDH more common
left
68
why is CDH of the left more common
as liver protects the right hemidiaphragm
69
explain CDH
failure of pneumoparietal membrane to fuse
70
what is bochdalek hernia
postero-lateral defect in diaphragm
71
what is morgagni hernia
anterior defect
72
when is CDH usually detected
antenatally on 20W anomaly scan
73
what investigation is ordered if postnatally suspect CDH
CXR - bowels present
74
what is antenatal management of CDH
corticosteroids: 2-doses 24h apart
75
explain management of CDH
Resp distress: resuscitation
Bowel decompression: NG tube decompression
Surgical repair: thoracotomy or laparotomy 24-48h after repair
76
what is functional abdominal pain
abdominal pain with no identifiable organic cause
77
what is the most common cause of chronic abdominal pain in children and adolescents
functional abdominal pain
78
what are risk factors for functional abdominal pain
Family history of: migraines, IBS
79
explain features of functional abdominal pain
- Central abdominal pain
- Erratic: pain for several days and then no pain for a prolonged period. May be related to anxiety (eg. school)
- Often school-age
- No change in growth
80
what is a key feature of functional abdominal pain
no change in growth
81
explain management of functional abdominal pain
- Explain to child there is no serious underlying disorder explaining their pain. Try to reassure and distract
Exclude following from diet:
- Sorbitol
- Fizzy drinks
- Caffeine
Keep a food diary to identify triggers
82
Define Hirschsprung's disease
Inherited disorder with aganglionic section colon due to failure Auerbach and meissner plexus
83
What does hirchspung's disease cause
impaired failure bowel to relax causing intestinal obstruction
84
In which gender is Hirschsprung's disease more common
Males
85
What two genetic conditions is Hirschsprung's associated with
Down syndrome
| MEN2
86
Explain 4 early-features of Hirschsprung's disease
1. Meconium Ileus (>48h)
2. Abdominal distention
3. FTT
4. Tight anal sphincter. Results in explosive release of stools on removing finger
87
Explain late-features of Hirschsprung's disease
- Chronic constipation
| - Inability to pass flatus
88
in 80% of cases, where does hirchsprung's disease affect
Rectosigmoid colon
89
what causes hirchsprungs
Failure caudal migration parasympathetic neuroblasts from neural crest to distal colon
90
explain histology of hirchsprungs
Absence myenteric and Auerbach's plexus. This causes un-co-ordinated peristalsis and slowed motility
91
what happens proximal to aganglionic segment
Dilation bowel segment proximal - this can lead to toxic megacolon
92
What is work-up of Hirschsprung's
AXR
Barium enema
Rectal biopsy
93
What is first-line for Hirschsprung's
AXR
94
What will be seen on AXR in Hirschsprungs
- No air in the rectum
| - Dilated segment proximal to aganglionic segment
95
Why is a barium enema performed
Determine length aganglionic segment prior to surgery
96
When is anal manometry used
Atypical presentations
97
What is the confirmatory test for Hirschsprung's
Rectal biopsy
98
What two findings will be seen on rectal biopsy
- Aganglionic
| - Increase acetylcholinesterase activity
99
What is surgical management of Hirschsprung's
1. Diverting colostomy - relieve dilated bowel
| 2. Resection aganglionic segment
100
What is a complication of Hirchsprung's
Toxic megacolon
101
What is the prevalence of IBD in children
Rare - only 1/4 presents in childhood
102
What age does IBD usually present in children
Adolescence
103
How does Crohn's disease present in children
- Abdominal pain
- Diarrhoea
- Weight loss
- FTT
104
What else may present in crohn's disease
Apthous ulcers
| Perianal disease: skin tags, fissures, abscesses
105
what is the most-specific symptom of crohns disease in children
Abdominal pain
106
what are symptoms of UC in children
- Blood in stool
- Diarrhoea
- Abdominal pain
- Arthropathy
107
what blood tests are ordered in IBD
- FBC
| - CRP and ESR
108
what stool test are ordered in IBD
Faecal calprotectin
109
what imaging is ordered in IBD
OGD and colonoscopy
110
why is a colonoscopy required, opposed to sigmoidoscopy
90% children with IBD will have pancolitis and hence require colonoscopy
111
list 5 findings crohn's on endoscopy
- Entire GI tract
- Skip lesions
- Transmural inflammation
- Fissures and fistulas
- Non-caesating granuloma
112
what investigation should all children with Crohn's disease have
barium enema with small-bowl follow through (SBFT)
113
why is barium enema with small bowl follow through (SBFT) ordered
to determine extent small bowel is involved in crohn's
114
how will barium enema with small bowel follow through present in Crohn's disease
string sign
115
what antibodies may be present in Crohn's disease
ASCA
116
what antibodies may be present in ulcerative colitis
p-ANCA
117
For Crohn's disease, what is first-line
oral corticosteroids and enteral nutrition (EEN)
118
What is used for maintenance in Crohn's disease
azathioprine
119
What are two indications for azathioprine
- More than two remissions per year
| - Remission in less than 6-months
120
What needs to be tested before giving azathioprine
TPMT levels
121
What is third-line for crohn's disease
Infliximab (Biologics)
122
What is used for mild-UC
- Left side colitis = topical mesalazine
| - Oral mesalazine
123
What is used for moderate-UC
Oral corticosteroids
124
What is used for acute-severe UC
IV hydrocortisone, IV Fluid, Colectomy and ileostomy
125
What are complications of UC
Toxic megacolon
126
What are complications of Crohn's
Strictures (Bowel Obstruction)
| Abscesses
