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EOY4 > Paediatric Surgery and GI > Flashcards

Paediatric Surgery and GI Flashcards

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1
Q

What is Hirschprung’s disease?

A

Congenital abnormality causing the ganglionic cells in myenteric and submucosal plexuses to be missing

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2
Q

What is the pathophysiology of Hirschprung’s disease?

A

Leads to a narrow and contracted segment

The normally innervated bowel that is proximal to the affected area becomes dilated

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3
Q

How does Hirschprung’s disease normally present?

A

Usually within the neonatal period

Don’t pass meconium within first 48 hours

Signs of bowel obstruction
Abdominal distension
Bilious vomiting

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4
Q

How does Hirschprung’s disease present on examination?

A

On PR there is significant passage of wind and liquid stool when finger is removed. This can also lead to a delay in diagnosis as bowel is temporarily dilated.

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5
Q

How does Hirschprung’s disease present if the initial diagnosis has been missed?

A

Profound, chronic constipation + distension + no soiling

? Growth failure

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6
Q

What is the investigation for Hirschprung’s disease?

A

Full thickness rectal biopsy is diagnostic

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7
Q

What is the management of Hirschprung’s disease?

A

Rectal washout

Anorectal pull through (cut out affected bit and form an anastomosis

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8
Q

What are the complications of Hirschprung’s disease?

A

Hirschprung’s enterocolitis within first few weeks of life

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9
Q

What is Intussusception?

A

The proximal bowel folds into a distal segment of bowel to form a pouch

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10
Q

What is the pathophysiology of Intussusception?

A

The ileum usually passes into the caecum through the ileocaecal valve

Disruption of peristalsis = colicky pain

Disruption of venous and lymphatic drainage = ischaemia

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11
Q

Which other two abnormalities may be present in Intussusception?

A

Meckel’s Diverticulum or polyp likely to be present

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12
Q

What is the presentation of Intussusception? (4)

A

Episodic crying that is inconsolable/ colicky pain. Becomes pale/pallor around mouth and draws up legs when pain is present with periods of recovery in between

Redcurrant jelly stools are characteristic (bloody mucus in poo) but usually appear later on

Sausy shaped mass in the tummy

Signs of bowel obstruction (Bilious vomiting and Abdominal distension)

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13
Q

Which bedside tests should be performed to diagnose intussusception?

A

Baseline obs - looking for signs of shock/deterioration

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14
Q

Which blood tests should be performed to diagnose intussusception?

A

Surgical pre-op bloods

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15
Q

What imaging should be done to diagnose intussusception?

A

Probably not a CT
Abdominal X-ray - showing distended small bowel + absence of gas in large bowel
Abdominal USS helpful - shows doughnut sign

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16
Q

What is the management of Intussusception?

A

A → E

No signs of peritonitis
Drip and suck - insert NG tube
Rectal air insufflation

Signs of peritonitis
Laparotomy for peritonitis, perforation, prolonged (>24 hrs) or pathological lead point

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17
Q

What are the complications of Intussusception? (4)

A

Bowel perforation

hypovolaemic shock

gut necrosis

sepsis

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18
Q

What is pyloric stenosis?

A

Hypertrophy of the pyloric muscle leading to a gastric outlet obstruction

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19
Q

When does pyloric stenosis usually present?

A

2-8 weeks old (even if premature)

More common in:
Boys
Firstborns
Family history (maternal)

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20
Q

What are the symptoms of pyloric stenosis?

A

Vomiting
Non-bilious (too high up for that)
↑ in frequency and force over time until it becomes projectile
Happens within minutes of feeding

No diarrhoea

Hunger - baby is alert and ?dehydrated

Weight loss (delay in presentation)

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21
Q

What are the signs of pyloric stenosis?

A

↓ urine output

Metabolic disturbances
Hypochloraemic metabolic alkalosis
Hyponatraemia
Hypokalaemia

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22
Q

What are the bedside tests to diagnose pyloric stenosis?

A

Examination

Palpable, olive sized pyloric mass in RUQ

Fluid challenge/give a feed - May see visible left to right peristalsis in LUQ whilst feeding

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23
Q

What are the blood tests to diagnose pyloric stenosis?

A

U&E - electrolyte imbalances

? Pre-ops

VBG - check acid-base balance

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24
Q

What imaging should be done to diagnose pyloric stenosis?

A

Abdominal USS

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25
What is the conservative/medical management of pyloric stenosis?
Insert NG tube if stomach needs decompressing | Correct electrolyte imbalances
26
What is the surgical management of pyloric stenosis?
Pyloromyotomy Can usually feed within 6 hours of surgery, and discharged within 2 days
27
What are the causes of vomiting in an infant?
Bilious = malrotation, meconium ileus, necrotising enterocolitis, duodenal atresia Non-bilious Acute = Pyloric stenosis Chronic = GORD, CMPA, allergies/intolerance
28
What is GORD in an infant?
The involuntary passage of gastric contents into the oesophagus due to inappropriate relaxation of the lower oesophageal sphincter. It is worsened by fluid diet, horizontal posture and short oesophagus
29
How would GORD present in an infant?
``` Recurrent vomiting Distress after feeding Failure to thrive Apnoea Aspiration ```
30
What are the risk factors for GORD in a baby?
NM = first born, boy, preterm, neurodevelopment disorder M = post surgical e.g. diaphragmatic hernia repair
31
How would GORD in a baby be managed?
C = reassurance. should resolve after 1 year. Avoid overfeeding - keep feeds to small and often. ? adding thickener to foods M = PPI/gaviscon/H2 antagonist S = only if really bad
32
What are the complications of GORD in a baby?
Oesophagitis - haematemesis and Fe deficiency anaemia Aspiration pneumonia Hyperchloraemic metabolic alkalosis
33
Define colic
When a baby cries for a long time but there is not an obvious cause Follows a rule of 3: Lasts for >3 hours a day Lasts for >3 days a week Lasts for >3 weeks
34
What are the risk factors for colic?
NM - preterm/SGA, CMPA M - mother smoked during pregnancy
35
What are the causes of colic? (7)
IT CRIES Infection Trauma ``` Cardiac (SVT) Reaction/Reflux Intussusception Eyes (corneal abrasion) Surgical (testicles) ```
36
What are the symptoms of colic?
High-pitched, inconsolable crying, usually during afternoon/evening Burping Pumping Drawing up knees Become red in the face
37
Define gastroenteritis
Sudden change to bowel movement leading to loose, watery stools, often with associated vomiting
38
What are the most likely causative agents for gastroenteritis?
Viral - rotavirus etc Bacterial - campylobacter jejuni (+ severe abdo pain and bloody stools), shigella, E. coli (lots of diarrhoea, really dehydrated)
39
What is the management of cow's milk protein allergy?
Conservative! Reassure - self limiting - usually stops within 4 months Feeding advice - Try without cow's milk or mam can try hypoallergenic diet if she is breast feeding
40
What is Coeliac disease?
A bowel disease that occurs due to the presence of Gliadin, which is a factor of gluten, barley, rye etc. Gliadin causes an immunological response within the proximal small bowel leading to inflammatory damage
41
What is the pathophysiology behind coeliac disease?
- Gliadin is not broken down in the small intenstine so IgA binds to it - Gliadin-IgA complex then binds to Transferrin receptors on enterocytes (coeliacs have more expressed) - Complex is then phagocytksed and presented by MHC2 cells - CD4 response = cytokine release and inflammatory damage - Also recruits B cells = more IgA produced = more damage
42
How may coeliac disease CLASSICALLY present in a child?
Classic presentation = profound failure to thrive within 18-24 months after introducing gluten into the diet Anaemia, osteomalacia, wasting of buttocks, bloating Change in stools - steatorrhoea, diarrhoea
43
How does coeliac disease COMMONLY present in a child?
Variable presentation - usually more asymptomatic and older Anaemia Incidental finding when investigating other HLA-DQ2/8 conditions e.g. T1DM, autoimmune thyroiditis, dermatitis herpetiformis
44
Which blood tests should be done to investigate a child presenting with coeliac?
tTGA (tissue transglutaminase), IGA Endomysal antibody FBC if anaemic
45
What is the gold standard imaging to diagnose coeliac?
Endoscopic Duodenal biopsy Have to eat normally for at least two weeks beforehand so can see the pathological changes
46
What are the findings on duodenal biopsy that signify coeliac disease?
Villous atrophy Crypt abscesses Intra-epithelial lymphocytic infiltration
47
What is the treatment of coeliac disease?
Remove gluten from the diet under dietician supervision
48
What are the complications of coeliac disease?
Osteoporosis, anaemia malabsorption failure to thrive Increased risk of small bowel cancer
49
What is the definition of failure to thrive?
Suboptimal weight gain in infants/children A sustained drop in 2 centile spaces leading to growth stunting and delayed development if severe
50
What are the two main causes of malabsorption?
Reduced food intake - environmental, pathological, retention Malabsorption - coeliac, IBD, CF, CMPA
51
What are the causes behind reduced food intake leading to malabsorption?
Environmental e.g. neglect, maternal depression Pathological - impaired suck e.g. cleft palate, Cerebral palsy Retention/Vomiting e.g. GORD
52
What questions could you ask to the parents of a child presenting with failure to thrive?
Feeding - breast or bottle? when were they weaned? Development Otherwise well? Birth hx Ask to keep a food diary for 3 days + behaviours
53
Which bedside and blood tests could be done to investigate a child presenting with failure to thrive?
Bed - urine MSC (rule out UTI or renal disease), pancreatic elastase if pancreatic insufficiency Blood - IgA + tTGA (coeliac), FBC (cancer), U&E (renal disease and metabolic disorders)
54
How would a moderately dehydrated child present?
Head to toe ``` Sunken fontanelles/eyes Altered consciousness Dry mucous membranes Reduced skin turgor Reduced urine output/fewer wet nappies ``` Baseline obs and skin colour still normal
55
How would a severely dehydrated/shocked child present?
As with moderately + mottled/pale skin + cool extremities + deranged baseline obs e.g. tachycardia, hypotension, tachypnoea, prolonged cap refill
56
What is the fluid requirement for resuscitation fluids in a child?
20mls/kg
57
What is the fluid requirement for maintenance fluids in a child?
1st 10 kg = 100mls/kg/day 2nd 10 kg = 50mls/kg/day The rest = 20mls/kg/day Remember to divide total amount by 24 to get requirement per hour
58
What are the 4 broad categories for the causes of constipation in children?
Neonatal Metabolic Autoimmune Functional
59
What are the neonatal causes of constipation?
Medical = meconium ileus secondary to cystic fibrosis Surgical = intussusception, hirschprung's, anal atresia/imperforate anus, lower spinal cord problem e.g. spina bifida
60
What are the metabolic causes of constipation?
Hypothyroidism Hypocalcaemia
61
What are the autoimmune causes of constipation?
Coeliac
62
What are the functional causes of constipation?
Poor toilet training Poor fluid intake Low exercise Hard poo = painful so scared to do it again and cycles round
63
What is the conservative management of constipation in children?
Use to gastrocolic reflex - sit them on the toilet after eating Increase fluid and fibre and encourage exercise(unlikely to make a difference) Refer to nurse led constipation clinic Star chart!
64
What is the medical management of constipation in children?
First line = osmotic laxative so Lactulose or Movicol (Draws water into bowel so softens stool) Second line = stimulant laxative e.g. Senna (irritates bowel wall so moves things along) Both are contraindicated in IBD and bowel obstruction. Osmotics are also contraindicated in bowel perforation and toxic megacolon and may cause hyper/hypokalaemia
65
How is the pre-existing deficit in dehydration calculated? When would you add potassium?
ml = % dehydration X weight (Kg) X 10 So 7.5% X 10kg X 10 = 750ml as the pre-existing deficit Replace the deficit over 48hours if over 5% dehydrated Add potassium (20mmol in a 500ml bag) once they have passed urine UNLESS IN DKA
66
What are the signs of 5% (mild), 10% (mod) and >10% (severe) dehydration?
5%/mild = dry mucous membranes, reduced urine output 10%/moderate = very dry MM, oliguric, tachycardic >10%/severe = shock signs, anuric, reduced consciousness
67
When would 0.45% saline and 0.9% saline be used as fluid replacement in severe dehydration? Why do you need to correct the deficits slowly?
0. 45% = if dehydration is isotonic or hyponatraemic e.g. excess fluid loss through vomiting/diarrhoea/DKA 0. 9% = if dehydration = hypernatraemic Correct slowly due to risk of cerebral oedema
68
What is sickle cell anaemia?
An autosomal recessive condition that results for synthesis of an abnormal haemoglobin chain termed HbS. Symptoms in homozygotes don't tend to develop until 4-6 months when the abnormal HbSS molecules take over from fetal haemoglobin.
69
What is the pathophysiology of sick cell anaemia?
polar amino acid glutamate is substituted by non-polar valine in each of the two beta chains (codon 6). This decreases the water solubility of deoxy-Hb in the deoxygenated state the HbS molecules polymerise and cause RBCs to sickle at a higher kPa sickle cells are fragile and haemolyse; they block small blood vessels and cause infarction
70
How would a meckels diverticulum normally present?
Abdominal pain Fresh blood in stool Nausea and vomiting Typically male and under 2
71
What is the pathophysiology of a meckels diverticulum?
Incomplete obliteration of the vitelline duct leads to an outpouching This can then tort and become inflamed/BO

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