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