Gastroenterology Flashcards
(163 cards)
1
Q
Medical causes of abdominal pain in both boys and girls?
A
Constipation is also very common
Urinary tract infection
Coeliac disease
Inflammatory bowel disease
Irritable bowel syndrome
Mesenteric adenitis
Abdominal migraine
Pyelonephritis
Henoch-Schonlein purpura
Tonsilitis
Diabetic ketoacidosis
Infantile colic
2
Q
Additional causes of abdominal pain to consider in adolescent girls?
A
Dysmenorrhea (period pain)
Mittelschmerz (ovulation pain)
Ectopic pregnancy
Pelvic inflammatory disease
Ovarian torsion
Pregnancy
3
Q
Surgical causes of abdominal pain to consider in children?
A
Appendicitis
Intussusception
Bowel obstruction
Testicular torsion
4
Q
Where does pain present in appendicitis?
A
Central abdominal pain spreading to the right iliac fossa
5
Q
Characteristic features of intussception?
A
Intussusception causes colicky non-specific abdominal pain with redcurrant jelly stools
6
Q
Presentation of bowel obstruction?
A
Bowel obstruction causes pain, distention, absolute constipation and vomiting
7
Q
Presentation of testicular torsion?
A
Testicular torsion causes sudden onset, unilateral testicular pain, nausea and vomiting
8
Q
Red flags for serious causes of abdominal pain?
A
Persistent or bilious vomiting
Severe chronic diarrhoea
Fever
Rectal bleeding
Weight loss or faltering growth
Dysphagia (difficulty swallowing)
Nighttime pain
Abdominal tenderness
9
Q
Initial investigations that may indicate the pathology causing abdominal pain in children?
A
Anaemia can indicate inflammatory bowel disease or coeliac disease
Raised inflammatory markers (ESR and CRP) can indicate inflammatory bowel disease
Raised anti-TTG or anti-EMA antibodies indicates coeliac disease
Raised faecal calprotectin indicates inflammatory bowel disease
Positive urine dipstick indicates a urinary tract infection
10
Q
Management of recurrent abdominal pain in children?
A
A diagnosis of recurrent abdominal pain is made when a child presents with repeated episodes of abdominal pain without an identifiable underlying cause. The pain is described as non-organic or functional. This is common and can lead to psychosocial problems, such as missed days at school and parental anxiety. There is overlap between the diagnoses of recurrent abdominal pain, abdominal migraine, irritable bowel syndrome and functional abdominal pain.
Recurrent abdominal pain often corresponds to stressful life events, such as loss of a relative or bullying. The leading theory for the cause is increased sensitivity and inappropriate pain signals from the visceral nerves (the nerves in the gut) in response to normal stimuli.
Management involves careful explanation and reassurance. Measures that can help manage the pain are:
Distracting the child from the pain with other activities or interests
Encourage parents not to ask about or focus on the pain
Advice about sleep, regular meals, healthy balanced diet, staying hydrated, exercise and reducing stress
Probiotic supplements may help symptoms of irritable bowel syndrome
Avoid NSAIDs such as ibuprofen
Address psychosocial triggers and exacerbating factors
Support from a school counsellor or child psychologist
11
Q
What is an abdominal migraine and how does it present?
A
Children are more likely than adults to suffer with a condition called abdominal migraine. This may occur in young children before they develop traditional migraines as they get older. Abdominal migraine presents with episodes of central abdominal pain lasting more than 1 hour. Examination will be normal.
There may be associated:
Nausea and vomiting
Anorexia
Pallor
Headache
Photophobia
Aura
12
Q
Abdominal migraine: Management of the acute attack
A
Low stimulus environment (quiet, dark room)
Paracetamol
Ibuprofen
Sumatriptan
13
Q
Abdominal migraine: Preventative medications?
A
Pizotifen, a serotonin agonist
Propranolol, a non-selective beta blocker
Cyproheptadine, an antihistamine
Flunarazine, a calcium channel blocker
14
Q
Withdrawal of pizotifen?
A
It needs to be withdrawn slowly when stopping as it is associated with withdrawal symptoms such as depression, anxiety, poor sleep and tremor.
15
Q
Most cases of consipation in children can be described how?
A
Most cases of constipation can be described as idiopathic constipation or functional constipation, meaning there is not a significant underlying cause other than simple lifestyle factors.
16
Q
Important secondary causes of constipation in children?
A
Hirschsprung’s disease, cystic fibrosis or hypothyroidism.
17
Q
Typical features in history and examination that suggest constipation?
A
Less than 3 stools a week
Hard stools that are difficult to pass
Rabbit dropping stools
Straining and painful passages of stools
Abdominal pain
Holding an abnormal posture, referred to as retentive posturing
Rectal bleeding associated with hard stools
Faecal impaction causing overflow soiling, with incontinence of particularly loose smelly stools
Hard stools may be palpable in abdomen
Loss of the sensation of the need to open the bowels
18
Q
What is encopresis and when is it considered pathological?
A
Encopresis is the term for faecal incontinence. This is not considered pathological until 4 years of age.
19
Q
What is the most common cause of encopresis in children?
A
It is usually a sign of chronic constipation where the rectum becomes stretched and looses sensation.
Large hard stools remain in the rectum and only loose stools are able to bypass the blockage and leak out, causing soiling.
20
Q
Rarer but serious causes of encopresis in children?
A
Spina bifida
Hirschprung’s disease
Cerebral palsy
Learning disability
Psychosocial stress
Abuse
21
Q
What lifestyle factors may contribute to constipation?
A
Habitually not opening the bowels
Low fibre diet
Poor fluid intake and dehydration
Sedentary lifestyle
Psychosocial problems such as a difficult home or school environment (always keep safeguarding in mind)
22
Q
What is desensitisation of the rectum and why does it occur?
A
Often patients develop a habit of not opening their bowels when they need to and ignoring the sensation of a full rectum.
Over time they loose the sensation of needing to open their bowels, and they open their bowels even less frequently.
They start to retain faeces in their rectum.
This leads to faecal impaction, which is where a large, hard stool blocks the rectum.
Over time the rectum stretches as it fills with more and more faeces.
This leads to further desensitisation of the rectum.
The longer this goes on, the more difficult it is to treat the constipation and reverse the problem.
23
Q
Secondary causes of constipation in children?
A
Hirschsprung’s disease
Cystic fibrosis (particularly meconium ileus)
Hypothyroidism
Spinal cord lesions
Sexual abuse
Intestinal obstruction
Anal stenosis
Cows milk intolerance
24
Q
Constipation in children - red flags that should prompt further investigations and referral to a specialist:
A
Not passing meconium within 48 hours of birth (cystic fibrosis or Hirschsprung’s disease)
Neurological signs or symptoms, particularly in the lower limbs (cerebral palsy or spinal cord lesion)
Vomiting (intestinal obstruction or Hirschsprung’s disease)
Ribbon stool (anal stenosis)
Abnormal anus (anal stenosis, inflammatory bowel disease or sexual abuse)
Abnormal lower back or buttocks (spina bifida, spinal cord lesion or sacral agenesis)
Failure to thrive (coeliac disease, hypothyroidism or safeguarding)
Acute severe abdominal pain and bloating (obstruction or intussusception)
25
Within what time frame should a neonate pass meconium and what can failure to do so be suggestive of?
Not passing meconium within 48 hours of birth (cystic fibrosis or Hirschsprung’s disease)
26
Complications of constipation in children?
Pain
Reduced sensation
Anal fissures
Haemorrhoids
Overflow and soiling
Psychosocial morbidity
27
Management of constipation in children?
A diagnosis of idiopathic constipation can be made without investigations, provided red flags are considered. It is important to provide adequate explanation of the diagnosis and management as well as reassure parents about the absence of concerning underlying causes. Explain that treating constipation can be a prolonged process, potentially lasting months.
NICE clinical knowledge summaries recommend:
- Correct any reversible contributing factors, recommend a high fibre diet and good hydration
- Start laxatives (movicol is first line)
- Faecal impaction may require a disimpaction regimen with high doses of laxatives at first
- Encourage and praise visiting the toilet. This could involve scheduling visits, a bowel diary and star charts.
Laxatives should be continued long term and slowly weaned off as the child develops a normal, regular bowel habit.
28
First line laxative in paediatrics?
Movicol
29
When and why is GORD normal in children?
Gastro-oesophageal reflux is where contents from the stomach reflux through the lower oesophageal sphincter into the oesophagus, throat and mouth.
In babies there is immaturity of the lower oesophageal sphincter, allowing stomach contents to easily reflux into the oesophagus.
It is normal for a baby to reflux feeds, and provided there is normal growth and the baby is otherwise well this is not a problem, however it can be upsetting for parents.
30
By what age do 90% of infants stop having reflux feeds?
1 year
31
Presentation of GORD in children
It is normal for babies to have some reflux after larger feeds. It becomes more troublesome when this causes them to become distressed. Signs of problematic reflux include:
Chronic cough
Hoarse cry
Distress, crying or unsettled after feeding
Reluctance to feed
Pneumonia
Poor weight gain
Children over one year may experience similar symptoms to adults:
Heartburn
Acid regurgitation
Retrosternal or epigastric pain
Bloating
Nocturnal cough
32
Possible causes of vomiting in children
Overfeeding
Gastro-oesophageal reflux
Pyloric stenosis (projective vomiting)
Gastritis or gastroenteritis
Appendicitis
Infections such as UTI, tonsillitis or meningitis
Intestinal obstruction
Bulimia
33
What red flag features of vomiting may be suggestive of pyloric stenosis?
Not keeping down any feed
Projectile or forceful vomiting
34
What red flag features of vomiting may be suggestive of intestinal obstruction?
Not keeping down any feed
Projectile or forceful vomiting
Bile stained vomiting
Abdominal distention
35
What red flag features of vomiting may be suggestive of peptic ulcer, oesophagitis or varicies?
Haematemesis or melaena
36
Why are reduced consciousness, bulging fontanelle or neurological signs are considered red flags when a child presents with vomtiing?
Meningitis
Raised ICP
37
When a child presents with vomiting alongside respiratory symptoms what may be of concern?
Potential aspiration or infection
38
Why are signs of infection red flag features in a presentation of vomiting?
Consider:
Pneumonia
UTI
Tonsilitis
Otitis
Meningitis
39
When a child presents with vomiting, what might make you suspect a cows milk allergy?
Rash, angiodema and any other signs of allergey
40
Red flags in a presentation of vomiting
Not keeping down any feed (pyloric stenosis or intestinal obstruction)
Projectile or forceful vomiting (pyloric stenosis or intestinal obstruction)
Bile stained vomit (intestinal obstruction)
Haematemesis or melaena (peptic ulcer, oesophagitis or varices)
Abdominal distention (intestinal obstruction)
Reduced consciousness, bulging fontanelle or neurological signs (meningitis or raised intracranial pressure)
Respiratory symptoms (aspiration and infection)
Blood in the stools (gastroenteritis or cows milk protein allergy)
Signs of infection (pneumonia, UTI, tonsillitis, otitis or meningitis)
Rash, angioedema and other signs of allergy (cows milk protein allergy)
Apnoeas are a concerning feature and may indicate serious underlying pathology and need urgent assessment
41
Management of mild/simple GORD in children?
Small, frequent meals
Burping regularly to help milk settle
Not over-feeding
Keep the baby upright after feeding (i.e. not lying flat)
42
Management of problematic or severe GORD?
Gaviscon mixed with feeds
Thickened milk or formula (specific anti-reflux formulas are available)
Proton pump inhibitors (e.g., omeprazole) where other methods are inadequate
Rarely in severe cases they may need further investigation with a barium meal and endoscopy.
Surgical fundoplication can be considered in very severe cases, however this is very rarely required or performed.
43
What is Sandifer’s Syndrome?
This is a rare condition causing brief episodes of abnormal movements associated with gastro-oesophageal reflux in infants.
The infants are usually neurologically normal.
44
What are the key features of Sandifer's syndrome?
Torticollis: forceful contraction of the neck muscles causing twisting of the neck
Dystonia: abnormal muscle contractions causing twisting movements, arching of the back or unusual postures
45
What is the course of Sandifer's syndrome and why does it require specialist referal?
The condition tends to resolve as the reflux is treated or improves. Generally the outcome is good.
It is worth referring patients with these symptoms to a specialist for assessment, as the differential diagnosis includes more serious conditions such as infantile spasms (West syndrome) and seizures.
46
What is the pyloric spchincter?
The pyloric sphincter is a ring of smooth muscle the forms the canal between the stomach and the duodenum.
47
What is pyloric stenosis?
Hypertrophy (thickening) and therefore narrowing of the pylorus
48
How does pyloric stenosis cause projectile vomiting?
Hypertrophy (thickening) and therefore narrowing of the pylorus prevents food traveling from the stomach to the duodenum as normal.
After feeding, there is increasingly powerful peristalsis in the stomach as it tries to push food into the duodenum.
Eventually it becomes so powerful that it ejects the food into the oesophagus, out of the mouth and across the room.
49
When and how does pyloric stenosis present?
Typically presents in the first few weeks of life,
A hungry baby that is thin, pale and generally failing to thrive
Projectile vomiting
50
Pyloric stenosis - examination
If examined after feeding, often the peristalsis can be seen by observing the abdomen.
A firm, round mass can be felt in the upper abdomen that “feels like a large olive”.
This is caused by the hypertrophic muscle of the pylorus.
51
Blood gas analysis in pyloric stenosis
HYPOCHLORIC METABOLIC ALKALOSIS
Blood gas analysis will show a hypochloric (low chloride) metabolic alkalosis as the baby is vomiting the hydrochloric acid from the stomach.
52
How is pyloric stenosis diagnosed?
Diagnosis is made using an abdominal ultrasound to visualise the thickened pylorus
53
Definitive management of pyloric stenosis?
Laparoscopic pyloromyotomy (Ramstedt’s operation)
An incision is made in the smooth muscle of the pylorus to widen the canal allowing that food to pass from the stomach to the duodenum as normal.
Prognosis is excellent following the operation.
54
Acute gastritis vs enteritis vs gastroenteritis?
Acute gastritis is inflammation of the stomach and presents with nausea and vomiting.
Enteritis is inflammation of the intestines and presents with diarrhoea.
Gastroenteritis is inflammation all the way from the stomach to the intestines and presents with nausea, vomiting and diarrhoea.
55
Why is it important to isolate inpatients with ?gastroenteritis?
Gastroenteritis is a very common condition in children. The most common cause of gastroenteritis is viral. It is very easily spread and patients presenting with gastroenteritis often have an affected family member or contact.
It is essential to isolate the patient in any healthcare environment, such as a paediatric assessment unit or hospital ward, as they can easily spread it to other patients.
56
Management of gastroenteritis in children?
Dehydration is the main concern - ensure they remain hydrated whilst waiting for vomiting and diarrhoea to settle
Prevention of spread: barrier nursing and rigorous infection control if inpatient, children need to stay off school until 48 hours after the symptoms have completely resolved
Antibiotics are not generally recommended or required, however sample of the faeces can be tested with microscopy, culture and sensitivities to establish the causative organism and antibiotic sensitivities.
Antibiotics should only be given in patients that are at risk of complications once the causative organism is confirmed.
Most children make a full recovery with simple supportive management, but gastroenteritis can potentially be fatal, especially in very young or vulnerable children with other health conditions.
57
Causes of loose stool in children?
Infection (gastroenteritis)
Inflammatory bowel disease
Lactose intolerance
Coeliac disease
Cystic fibrosis
Toddler’s diarrhoea
Irritable bowel syndrome
Medications (e.g. antibiotics)
58
What is steatorrhoea and what does it suggest?
Steatorrhoea means greasy stools with excessive fat content. This suggests a problem with digesting fats, such as pancreatic insufficiency (think about cystic fibrosis).
59
Viral causes of gastroenteritis?
Rotavirus
Norovirus
Adenovirus is a less common cause and presents with a more subacute diarrhoea.
60
Bacterial causes of gastroenteritis?
Escherichia coli (E. coli 0157 strain)
Campylobacter Jejuni
Shigella
Salmonella
Bacillus Cereus
Yersinia Enterocolitica
Staphylococcus Aureus Toxin
61
Common parasitic cause of gastroenteritis?
Giardiasis
62
Possible post-gastroenteritis complications?
Lactose intolerance
Irritable bowel syndrome
Reactive arthritis
Guillain–Barré syndrome
63
Hydration in gastroenteritis?
Attempt a fluid challenge. It involves recording a small volume of fluid given orally every 5-10 minutes to ensure they can tolerate it. If they are able to tolerate oral fluid and are adequately hydrated they can usually be managed at home.
Rehydration solutions (e.g. dioralyte) can be used if tolerated.
Dehydrated children or those that fail the fluid challenge may require IV fluids.
64
Antidiarrhoeal medication such as loperamide and antiemetic medication such as metoclopramide are generally not recommended in management of gastroenteritis - when in particular?
Antidiarrhoeal medications are particularly avoided in e. coli 0157 and shigella infections, and where there is bloody diarrhoea or high fever.
65
Why should antibiotics be avoided if E. coli gastroenteritis is considered?
The use of antibiotics increases the risk of haemolytic uraemic syndrome, therefore antibiotics should be avoided if E. coli gastroenteritis is considered.
66
Which toxin leads to HUS and what organisms produce it?
E. coli 0157 and Shigella produce the Shiga toxin. This causes abdominal cramps, bloody diarrhoea and vomiting. The Shiga toxin destroys blood cells and leads to haemolytic uraemic syndrome (HUS).
67
Where is e. coli found and how does it spread?
Escherichia coli (E. coli) is a normal intestinal bacteria. Only certain strains cause gastroenteritis. It is spread through contact with infected faeces, unwashed salads or contaminated water.
68
What is Campylobacter Jejuni?
Campylobacter is a common cause of travellers diarrhoea. It is the most common bacterial cause of gastroenteritis worldwide. Campylobacter means “curved bacteria”.
It is a gram negative bacteria that has a curved or spiral shape. It is spread by:
Raw or improperly cooked poultry
Untreated water
Unpasteurised milk
69
Incubation and symptoms of campylobacter jejuni?
Incubation is usually 2 to 5 days. Symptoms resolve after 3 to 6 days. Symptoms are:
Abdominal cramps
Diarrhoea often with blood
Vomiting
Fever
70
Antibiotics in campylobacter jejuni gastoenteritis?
Antibiotics can be considered after isolating the organism where patients have severe symptoms or other risk factors such as HIV or heart failure.
Popular antibiotic choices are:
azithromycin
ciprofloxacin
71
Campylobacter jejuni gram stain?
Gram negative rods with gull-wing shape
72
Ecoli on gram stain
gram-negative bacillus
73
Symptoms and incubation of shigella
The incubation period is 1 to 2 days and symptoms usually resolve within 1 week without treatment.
It causes bloody diarrhoea, abdominal cramps and fever.
74
Treatment of severe cases of shigella?
Treatment of severe cases is with azithromycin or ciprofloxacin.
75
How is shigella spread?
Shigella is spread by faeces contaminating drinking water, swimming pools and food.
76
Shigella gram stain
Gram negative rod
77
How is Salmonella spread?
Salmonella is spread by eating raw eggs or poultry, or food contaminated with the infected faeces of small animals.
78
Salmonella symptoms and incubation?
Incubation is 12 hours to 3 days and symptoms usually resolve within 1 week. Symptoms are watery diarrhoea that can be associated with mucus or blood, abdominal pain and vomiting.
79
Salmonella management
Symptoms usually resolve within a week. Antibiotics are only necessary in severe cases and should be guided by stool culture and sensitivities.
80
How is Bacillus Cereus spread?
Through inadequately cooked food. It grows well on food not immediately refrigerated after cooking. The typical food is fried rice left out at room temperature.
81
Bacillus cereus course
The typical course is vomiting within 5 hours, then diarrhoea after 8 hours, then resolution within 24 hours.
82
What toxin does Bacillus cereus produce and what symtpoms does it cause?
Whilst growing on food it produces a toxin called cereulide. This toxin causes abdominal cramping and vomiting within 5 hours of ingestion. When it arrives in the intestines it produces different toxins that cause a watery diarrhoea. This occurs more than 8 hours after ingestion. All of the symptoms usually resolves within 24 hours.
83
Bacillus cereus gram stain
gram positive rod
84
Salmonella gram stain
rod-shaped (bacillus) Gram-negative bacteria
85
Yersinia Enterocolitica gram stain?
Yersinia is a gram negative bacillus.
86
How is Yersinia Enterocolitica spread?
Pigs are key carriers of Yersinia, and eating raw or undercooked pork can cause infection. It is also spread through contamination with the urine or faeces of other mammal such as rats and rabbits.
87
Yersinia Enterocolitica presentation
Yersinia most frequently affects children, causing watery or bloody diarrhoea, abdominal pain, fever and lymphadenopathy.
Older children or adults can present with right sided abdominal pain due mesenteric lymphadenitis (inflammation in the intestinal lymph nodes) and fever. This can give the impression of appendicitis.
Antibiotics are only necessary in severe cases and should be guided by stool culture and sensitivities.
88
Yersinia Enterocolitica course and incubation
Incubation is 4 to 7 days and the illness can last longer than other causes of enteritis with symptoms lasting 3 weeks or more.
89
How can staphylococcus aureus result in gastroenteritis?
Staphylococcus aureus can produce enterotoxins when growing on food such as eggs, dairy and meat. When eaten these toxins cause small intestine inflammation. This causes symptoms of diarrhoea, perfuse vomiting, abdominal cramps and fever. These symptoms start within hours of ingestion and settle within 12 to 24 hours. It is not actually the bacteria causing the enteritis but the staphylococcus enterotoxin.
90
What is giardia lambilla, how is it spread, how does it prevent and what is the treatment?
Giardia lamblia is a type of microscopic parasite. It lives in the small intestines of mammals. These mammals may be pets, farmyard animals or humans. It releases cysts in the stools of infected mammals. The cysts contaminate food or water and are eaten, infecting a new host. This is called faecal-oral transmission.
Infection may not cause any symptoms, or it may cause chronic diarrhoea. Diagnosis is made by stool microscopy. Treatment is with metronidazole.
91
What is coeliac disease and how does it devlope?
Coeliac disease is an autoimmune condition where exposure to gluten causes an immune reaction that creates inflammation in the small intestine. It usually develops in early childhood but can start at any age.
In coeliac disease autoantibodies are created in response to exposure to gluten. These autoantibodies target the epithelial cells of the intestine and lead to inflammation.
92
Coeliac disease autoantibodies?
Anti-tissue transglutaminase (anti-TTG)
Anti-endomysial (anti-EMA)
(Deaminated gliadin peptides antibodies (anti-DGPs))
93
How does coeliac disease cause symptoms?
In coeliac disease autoantibodies are created in response to exposure to gluten.
These autoantibodies target the epithelial cells of the intestine and lead to inflammation.
Inflammation affects the small bowel, particularly the jejunum.
It causes atrophy of the intestinal villi.
The intestinal cells have villi on them that help with absorbing nutrients from the food passing through the intestine.
The inflammation causes malabsorption of nutrients and disease related symptoms.
94
Which part of the small bowel is particularly affected in coeliac disease?
Atrophy of the intestinal villi of Jejunum
95
How might coeliac disease present?
Failure to thrive in young children
Diarrhoea
Fatigue
Weight loss
Mouth ulcers
Anaemia secondary to iron, B12 or folate deficiency
Dermatitis herpetiformis is an itchy blistering skin rash that typically appears on the abdomen
96
Rarely, coeliac disease may present with neurologcal symptoms - such as what?
Peripheral neuropathy
Cerebellar ataxia
Epilepsy
97
In what condition is coeliac disease tested for due to the fact they are often linked?
T1DM
98
Which genes are associated with coeliac disease?
HLA-DQ2 gene (90%)
HLA-DQ8 gene
99
What should you check to make sure autoantibody levels accurately reflect the presence/absence of coeliac disease?
Total IgA
Anti-TTG and anti-EMA antibodies are IgA. Some patients have an IgA deficiency. When you test for these antibodies, it is important to test for total Immunoglobulin A levels because if total IgA is low the coeliac test will be negative even when they have the condition. In this circumstance you can test for the IgG version of the anti-TTG or anti-EMA antibodies or do an endoscopy with biopsies.
100
How is coeliac disease investigated?
Investigations must be carried out whilst the patient remains on a diet containing gluten otherwise it may not be possible to detect the antibodies or inflammation in the bowel.
Check total immunoglobulin A levels to exclude IgA deficiency before checking for coeliac disease specific antibodies:
Raised anti-TTG antibodies (first choice)
Raised anti-endomysial antibodies
Endoscopy and intestinal biopsy
101
What does intestinal biopsy show in coeliac disease?
“Crypt hypertrophy”
“Villous atrophy”
102
What other conditions is coeliac disease associated with?
Type 1 diabetes
Thyroid disease
Autoimmune hepatitis
Primary biliary cirrhosis
Primary sclerosing cholangitis
Down’s syndrome
103
Potential complications of coeliac disease?
Vitamin deficiency
Anaemia
Osteoporosis
Ulcerative jejunitis
Enteropathy-associated T-cell lymphoma (EATL) of the intestine
Non-Hodgkin lymphoma (NHL)
Small bowel adenocarcinoma (rare)
104
Management/monitoring of coeliac disease?
A lifelong gluten free diet is essentially curative. Relapse will occur on consuming gluten again. Checking coeliac antibodies can be helpful in monitoring the disease.
105
Crohn's features
Crohn’s (crows NESTS)
N – No blood or mucus (these are less common in Crohns.)
E – Entire GI tract
S – “Skip lesions” on endoscopy
T – Terminal ileum most affected and Transmural (full thickness) inflammation
S – Smoking is a risk factor (don’t set the nest on fire)
Crohn’s is also associated with weight loss, strictures and fistulas.
106
UC features?
Ulcerative Colitis (remember U – C – CLOSEUP)
C – Continuous inflammation
L – Limited to colon and rectum
O – Only superficial mucosa affected
S – Smoking is protective
E – Excrete blood and mucus
U – Use aminosalicylates
P – Primary sclerosing cholangitis
107
When should you suspect IBD in peads?
Suspect inflammatory bowel disease in children and teenagers presenting with perfuse diarrhoea, abdominal pain, bleeding, weight loss or anaemia.
They may be systemically unwell during flares, with fevers, malaise and dehydration.
108
IBD extra-intestinal manifestations
Finger clubbing
Erythema nodosum
Pyoderma gangrenosum
Episcleritis and iritis
Inflammatory arthritis
Primary sclerosing cholangitis (ulcerative colitis)
109
Investigations in ?IBD
Blood tests for anaemia, infection, thyroid, kidney and liver function. A raised CRP indicates active inflammation.
Faecal calprotectin is released by the intestines when inflamed. It is a useful screening test and is more than 90% sensitive and specific for IBD in adults.
Endoscopy (OGD and colonoscopy) with biopsy is the gold standard investigation for diagnosis of IBD.
Imaging with ultrasound, CT and MRI can be used to look for complications such as fistulas, abscesses and strictures.
110
General management of IBD in peads?
Any child presenting with symptoms suggestive of inflammatory bowel disease should be referred to secondary care for specialist assessment and management. They will be closely followed up by the multi-disciplinary team. This team includes paediatricians, specialist nurses, pharmacists, dieticians and surgeons if necessary.
It is essential to monitor the growth and pubertal development in children with inflammatory bowel disease, particularly when they are having exacerbations or being treated with steroids.
Management involves a combination of inducing remission during flares and maintaining remission when well. It is essential to monitor and supporting growth and development, and patients are likely to need input from a dietician.
111
Crohn's - inducing remission
First line are steroids (e.g. oral prednisolone or IV hydrocortisone).
If steroids alone don’t work, consider adding immunosuppressant medication under specialist guidance:
Azathioprine
Mercaptopurine
Methotrexate
Infliximab
Adalimumab
112
Crohn's - maintaining remission?
Treatment is tailored to individual patients based on risks, side effects, nature of the disease and patient preference. It is reasonable not to take any medications whilst well.
First line:
Azathioprine
Mercaptopurine
Alternatives:
Methotrexate
Infliximab
Adalimumab
113
When might children be considered for surgical management or Crohn's?
When the disease only affects the distal ileum it is possible to surgically resect this area to prevent further flares. Crohn’s typically involves the entire GI tract.
Surgery can also be used to treat strictures and fistulas secondary to Crohn’s disease.
114
Inducing remission in UC - mild to moderate disease
Mild to moderate disease
First line: aminosalicylate (e.g. mesalazine oral or rectal)
Second line: corticosteroids (e.g. prednisolone)
115
Inducing remission in UC - severe disease
Severe disease
First line: IV corticosteroids (e.g. hydrocortisone)
Second line: IV ciclosporin
116
Maintaining remission in UC
Aminosalicylate (e.g. mesalazine oral or rectal)
Azathioprine
Mercaptopurine
117
Surgical management of UC?
Ulcerative colitis usually only affects the colon and rectum.
Therefore, removing the colon and rectum (panproctocolectomy) will remove the disease.
The patient is then left with either a permanent ileostomy or something called an ileo-anal anastomosis (J-pouch).
This is where the ileum is folded back on itself and fashioned into a larger pouch that functions like a rectum.
This “J-pouch” is then attached to the anus and collects stools prior to the person passing a motion.
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What is biliary atresia?
Biliary atresia is a congenital condition where a section of the bile duct is either narrowed or absent.
This results in cholestasis, where the bile cannot be transported from the liver to the bowel.
Conjugated bilirubin is excreted in the bile, therefore biliary atresia prevents the excretion of conjugated bilirubin.
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How does billiary atresia present?
Biliary atresia presents shortly after birth with significant jaundice due to high conjugated bilirubin levels.
There are many causes of jaundice in the neonate. The majority of cases are benign (e.g. breast milk jaundice), however more serious causes such as biliary atresia need to be excluded by measuring the conjugated bilirubin level.
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When should biliary atresia be suspected?
Suspect biliary atresia in babies with a persistent jaundice, lasting more than 14 days in term babies and 21 days in premature babies.
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What is the initial investigation for ?biliary atresia and what might it reveal?
The initial investigation for possible biliary atresia is conjugated and unconjugated bilirubin.
A high proportion of conjugated bilirubin suggests the liver is processing the bilirubin for excretion (by conjugating it), but it is not able to excrete the conjugated bilirubin because it cannot flow through the biliary duct into the bowel.
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How is biliary atresia managed?
Management of biliary atresia is with surgery.
The “Kasai portoenterostomy” involves attaching a section of the small intestine to the opening of the liver, where the bile duct normally attaches.
This is somewhat successful and can clear the jaundice and prolong survival.
Often patients require a full liver transplant to resolve the condition.
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Causes of intestinal obstruction
Meconium ileus
Hirschsprung’s disease
Oesophageal atresia
Duodenal atresia
Intussusception
Imperforate anus
Malrotation of the intestines with a volvulus
Strangulated hernia
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Presentation of bowel obstruction
Persistent vomiting. This may be bilious, containing bright green bile.
Abdominal pain and distention
Failure to pass stools or wind
Abnormal bowel sounds. These can be high pitched and “tinkling” early in the obstruction and absent later.
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Diagnosis of bowel obstruction?
The initial investigation of choice is an abdominal xray.
This may show dilated loops of bowel proximal to the obstruction and collapsed loops of bowel distal to the obstruction.
There will also be absence of air in the rectum.
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Management of bowel obstruction in children?
Patients presenting with intestinal obstruction need to be referred to a paediatric surgical unit as an emergency.
Initial management involves making them nil by mouth and inserting a nasogastric tube to help drain the stomach and stop the vomiting.
They will also require IV fluids to correct any dehydration and electrolyte imbalances, and keep them hydrated while waiting for definitive management of the underlying cause.
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What is Hirschspurng's disease
Hirschsprung’s disease is a congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum.
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Which nervous system is formed by the myenteric plexus?
The myenteric plexus, also known as Auerbach’s plexus, forms the enteric nervous system. It is the brain of the gut.
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Course and function of the myenteric plexus?
This nerve plexus runs all the way along the bowel in the bowel wall, and is a complex web of neurones, ganglion cells, receptors, synapses and neurotransmitters.
It is responsible for stimulating peristalsis of the large bowel.
The aganglionic section of colon does not relax, causing it to becomes constricted.
This leads to loss of movement of faeces and obstruction in the bowel.
Proximal to the obstruction the bowel becomes distended and full.
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Pathophysiology of Hirschsprung's disease?
Absence of parasympathetic ganglion cells:
- During fetal development these cells start higher in the GI tract and gradually migrate down to the distal colon and rectum.
- Hirschsprung’s occurs when the parasympathetic ganglion cells do not travel all the way down the colon, and a section of colon at the end is left without these parasympathetic ganglion cells.
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What is total colonic aganglionosis?
The length of colon without innervation varies between patients from a small area to the entire colon.
When the entire colon is affected this is called total colonic aganglionosis
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Hirschsprung's - genetics and associations
There are a number of genes on various chromosomes that may have a modifying effect to increase the risk of Hirschsprung’s. A family history of Hirschsprung’s greatly increases the risk.
Hirschsprung’s disease usually occurs in isolation, however it is associated with a number of other syndromes, including:
Downs syndrome
Neurofibromatosis
Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair)
Multiple endocrine neoplasia type II
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How does Hirschsprung's diseasepresent?
The severity of the presentation and the age at diagnosis varies significantly depending on the individual and the extent of the bowel that is affected.
It can present with acute intestinal obstruction shortly after birth or more gradually developing symptoms:
Delay in passing meconium (more than 24 hours)
Chronic constipation since birth
Abdominal pain and distention
Vomiting
Poor weight gain and failure to thrive
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What is HAEC and how does it present?
Hirschsprung-associated enterocolitis (HAEC) is inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung’s disease.
It typically presents within 2-4 weeks of birth with fever, abdominal distention, diarrhoea (often with blood) and features of sepsis.
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What urgent management does HAEC require and why?
It is life threatening and can lead to toxic megacolon and perforation of the bowel.
It requires urgent antibiotics,
fluid resuscitation and
decompression of the obstructed bowel.
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Investigating Hirschsprung's?
Abdominal xray can be helpful in diagnosing intestinal obstruction and demonstrating features of HAEC.
Rectal biopsy is used to confirm the diagnosis. The bowel histology will demonstrates an absence of ganglionic cells.
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Management of Hirschsprung's
Unwell children and those with enterocolitis will require initial fluid resuscitation and management of the intestinal obstruction. IV antibiotics are required in HAEC.
Definitive management is by surgical removal of the aganglionic section of bowel. Most patients will live a normal life after corrective surgery, although they can have long term disturbances in bowel function and may be left with some degree of incontinence.
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What is intussuception?
Intussusception is a condition where the bowel “invaginates” or “telescopes” into itself.
This thickens the overall size of the bowel and narrows the lumen at the folded area, leading to a palpable mass in the abdomen and obstruction to the passage of faeces through the bowel.
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In which patients does intussusception typically occur?
Infants 6 months to 2 years
More common in boys
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What conditions is intussusception associated with
Concurrent viral illness
Henoch-Schonlein purpura
Cystic fibrosis
Intestinal polyps
Meckel diverticulum
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Features of intussusception?
Severe, colicky abdominal pain
Pale, lethargic and unwell child
“Redcurrant jelly stool”
Right upper quadrant mass on palpation. This is described as “sausage-shaped”
Vomiting
Intestinal obstruction
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Typical presenting case of intussusception?
Redcurrant jelly stool
Sausage-shaped mass in the abdomen. T
he typical child in the exam will have had a viral upper respiratory tract infection preceding the illness and will have features of intestinal obstruction (vomiting, absolute constipation and abdominal distention)
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How can intussusception be diagnosed?
Diagnosis is made mainly by ultrasound scan or contrast enema.
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Management of intussusception?
Therapeutic enemas can be used to try to reduce the intussusception. Contrast, water or air are pumped into the colon to force the folded bowel out of the bowel and into the normal position.
Surgical reduction may be necessary if enemas do not work.
If the bowel becomes gangrenous (due to a disruption of the blood supply) or the bowel is perforated, then surgical resection is required.
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Complications of intussusception?
Obstruction
Gangrenous bowel
Perforation
Death
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What is the initial investigation of choice for ?intussusception?
USS
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What occurs in appendicitis?
Appendicitis is inflammation of the appendix - a small, thin tube sprouting from the caecum.
The appendix becomes inflamed due to infection trapped in the appendix by obstruction at the point where the appendix meets the bowel.
The inflammation can quickly proceed to gangrene and rupture.
The appendix can rupture and release faecal content and infective material into the abdomen. This leads to peritonitis, which is inflammation of the peritoneal contents.
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The peak incidence of appendicitis is when?
Ages 10 to 20 years
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Appendicitis signs and symptoms
The key presenting feature of appendicitis is abdominal pain. This typically starts as central abdominal pain, that moves down to the right iliac fossa (RIF) over time and eventually becomes localised in the RIF. On palpation of the abdomen there is tenderness in McBurney’s point. This is a localised area one third the distance from the anterior superior iliac spine (ASIS) to the umbilicus.
Other classic features are:
Loss of appetite (anorexia)
Nausea and vomiting
Rovsing’s sign (palpation of the left iliac fossa causes pain in the RIF)
Guarding on abdominal palpation
Rebound tenderness is increased pain when quickly releasing pressure on the right iliac fossa
Percussion tenderness is pain and tenderness when percussing the abdomen
Rebound tenderness and percussion tenderness suggest peritonitis, caused by a ruptured appendix.
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Diagnosis of appendictis?
Diagnosis is based on the clinical presentation and raised inflammatory markers. Performing a CT scan can be useful in confirming the diagnosis, particularly where another diagnosis is more likely.
An ultrasound scan is often used in female patients to exclude ovarian and gynaecological pathology.
When a patient has a clinical presentation suggestive of appendicitis but investigations are negative, the next step is to perform a diagnostic laparoscopy to visualise the appendix directly. The surgeon can then proceed to an appendicectomy during the same procedure if indicated.
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Key differential diagnoses of appendicitis?
- ECTOPIC PREGNANCY
Consider ectopic pregnancy in girls of childbearing age. This is a gynaecological emergency with a relatively high mortality if mismanaged. A serum or urine bHCG (pregnancy test) to exclude pregnancy is essential in adolescent girls.
OVARIAN CYSTS
- can cause pelvic and iliac fossa pain, particularly with rupture or torsion.
MECKEL'S DIVERTICULUM
- Meckel’s diverticulum is a malformation of the distal ileum that occurs in around 2% of the population. It is usually asymptomatic, however it can bleed, become inflamed, rupture or cause a volvulus or intussusception. They are often removed prophylactically if identified incidentally during other abdominal operations.
MESENTERIC ADENITIS
- Describes inflamed abdominal lymph nodes. This presents with abdominal pain, usually in younger children. This is often associated with tonsillitis or an upper respiratory tract infection. No specific treatment is required.
APPENDIX MASS
- occurs when the omentum surrounds and sticks to the inflamed appendix, forming a mass in the right iliac fossa. This is typically managed conservatively with supportive treatment and antibiotics, followed by appendicectomy once the acute condition has resolved.
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Management of appendicitis?
Patients with suspected appendicitis need emergency admission to hospital under the surgical team. Older children, for example those aged above 10 years, can often be managed by adult general surgical teams at local hospitals, provided there is a paediatric department in the hospital. Younger children will need to be admitted under paediatric surgeons.
Removal of the inflamed appendix (appendicectomy) is the definitive management for acute appendicitis. Laparoscopic surgery is associated with fewer risks and faster recovery compared to open surgery (laparotomy).
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Complications of appendectomy?
Bleeding, infection, pain and scars
Damage to bowel, bladder or other organs
Removal of a normal appendix
Anaesthetic risks
Venous thromboembolism (deep vein thrombosis or pulmonary embolism)
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An ultrasound scan shows concentric echogenic and hypoechogenic bands indicates what?
Intussuception
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Characteristics of vomiting in pyloric stenosis?
Non billious
Projectile
Straight after feeds
Not present at birth
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Acid base status in pyloric stenosis
Hypocholemic hypokalemic metabloic acidosis
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Bloods in Reye's syndrome
Blood tests showed a mild anaemia, a transaminitis, raised bilirubin, a prolonged international normalised ratio, raised lactate and a raised C-reactive protein
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What is Reye's syndrome and when does it occur?
Reye's syndrome is acute liver failure and non-inflammatory hepatic encephalopathy which occurs in children under 12 who are given aspirin during the acute phase of a viral infection. Liver biopsies show microvesicular steatosis and venous collapse.
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Management of Reye's syndrome?
Treatment is supportive, and often requires ITU admission.
Lactulose can be used to lower ammonia levels
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What is the number one cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years?
Meckels diverticulum
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The outcome or prognosis of a patient with CDH is largely dependent on which 2 factors?
(1) Liver position
(2) Lung-to-head ratio
If the liver has herniated into the chest, the disease is more severe and there is lower chance of survival.
The lung-to-head ratio is a numeric estimate of the size of the foetal lungs, dependent on the amount of lung visible. A ratio >1.0 reflects a better outcome.
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How does congenital diaphragmatic hernia present?
Congenital diaphragmatic hernia presents with scaphoid abdomen, due to herniation of the abdominal contents into the cleft
Displaced heart sounds to midline, reduced breath sounds bilaterally
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Imaging in NEC
The condition is usually diagnosed via abdominal X-ray, which will often demonstrate pneumatosis intestinalis; air within the intestinal wall, arising due to the ongoing intestinal ischaemia.
Gas within the portal venous system and visibly dilated intestinal loops are other commonly observed features on imaging.
