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Paediatrics > Gastrointestinal Development > Flashcards

Gastrointestinal Development Flashcards

1
Q

What is the pathophysiology and anatomy of Hirschsprung’s disease?

A
  1. Lack of parasympathetic innervation of rectosigmoid colon, constant contraction.
  2. Obstruction and dilation of bowel proximal to affected segment.
  3. Internal anal sphincter always involved.
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2
Q

What is this a presentation of?
Newborn fails to pass meconium within 48 hours of birth, distended abdomen, vomiting, tight anal sphincter, explosive passage of stool on removal after PR.

A

Hirschsprung’s disease

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3
Q

How is Hirschsprung’s disease diagnosed?

A
  1. Rectal suction biopsy an aganglionic section gold standard.
  2. Rectal contrast enema is a good screening test.
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4
Q

What is the management of Hirschsprung’s disease?

A
  1. Resection of aganglionic segment is gold standard.
  2. Initial bridging management with rectal washouts.
  3. Colon anastomosed to anal canal or colostomy.
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5
Q

What are the complications of Hirschsprung’s disease?

A

Perforation, bleeding, ulcers, enterocolitis, incontinence.

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6
Q

What is this describing?

Blind-ending oesophagus and an abnormal connection between the oesophagus and the trachea.

A

Oesophageal atresia and tracheo-oesophageal fistula (most often occur together)

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7
Q

What is this a presentation of?

Saliva pooling in mouth, blowing bubbles, choking, cyanosed when feeding (milk spills into lung), cough.

A

Oesophageal atresia and tracheo-oesophageal fistula

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8
Q

What are the clinical features of prenatal oesophageal atresia and tracheo-oesophageal fistula?

A

Polyhydramnios, small stomach

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9
Q

How is oesophageal atresia and tracheo-oesophageal fistula diagnosed?

A
  1. Inability to pass NG tube into stomach, coiling of tube in oesophagus.
  2. In isolated OA - no gastric bubble on CXR
  3. If gastric bubble +ve - suggests distal TOF
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10
Q

What is the management for oesophageal atresia and tracheo-oesophageal fistula?

A
  1. NBM
  2. Suction of oropharynx to prevent aspiration of saliva.
  3. Reanastomosis surgery within 24 hours
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11
Q

What is the main complication a congenital diaphragmatic hernia?

A

Pulmonary hypoplasia

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12
Q

What is this a presentation of?
Neonate, severe respiratory distress, scaphoid (concave) abdomen, no breath sounds on one side, heart sounds displaced to one side, bowel sounds in one hemithorax.

A

Congenital diaphragmatic hernia

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13
Q

How is prenatal and postnatal congenital diaphragmatic hernia diagnosed?

A

Prenatal - USS

Postnatal - CXR, bowel loops in hemithorax, mediastinal shift

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14
Q

What is the management for a congenital diaphragmatic hernia?

A
  1. Immediate intubation and ventilation
  2. Large NG tube to enable air to escape to aid lung expansion
  3. Surgery - delay until respiratory status has been established
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15
Q

What is the prognosis of a congenital diaphragmatic hernia?

A

50%

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16
Q

What is this describing?

A paraumbilical defect in the abdominal wall with evisceration of the intestine.

A

Gastroschisis

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17
Q

How is gastroschisis identified?

A

Antenatally on USS

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18
Q

What is the management of gastroschisis?

A
  1. Cover exposed bowel in cling film

2. Close defect surgically

19
Q

What is this describing?
Defect in the umbilical ring with herniation of abdominal viscera, covered in peritoneum. Often associated with other congenital malformations.

A

Exomphalos

20
Q

How is exomphalos identified?

A

Antenatally on USS

21
Q

What is the management for exomphalos?

A

Surgery, reduce gradually with delayed closure to prevent respiratory insufficiency.

22
Q

What is the pathophysiology of malrotation (congenital bowel issue)?

A

Bowel fixed in abnormal position in abdomen, absent attachment of small intestine mesentery causes mid gut volvulus/obstruction.

23
Q

What is this a presentation of?

Bilious neonatal vomiting and bowel obstruction.

A

Malrotation of the bowel

24
Q

How is malrotation of the bowel in neonates diagnosed?

A

Upper GI contrast studies

25
Q

What is the management for malrotation of the bowel in neonates?

A
  1. IV fluids, NG tube

2. Surgical fixation of the bowel

26
Q

What is the complication of malrotation of the bowel in neonates?

A

Volvulus - surgical emergency, bowel can become necrotic due to occlusion of blood supply form mesentery.

27
Q

What is this describing?

Blind-ending passage of the duodenum.

A

Duodenal atresia (most common bowel atresia)

28
Q

What is this a presentation of?

Vomiting (can be bilious), failure to pass flatus and faeces.

A

Intestinal atresia

29
Q

How is intestinal atresia diagnosed?

A

AXR - dilated bowel loops proximal to atresia, ‘double bubble’ sign in duodenal atresia.

30
Q

What causes the ‘double bubble’ sign on the AXR in duodenal atresia?

A

Air distends the stomach and first part of duodenum forming two separate bubbles.

31
Q

What is the management of intestinal atresia?

A

Surgical anastomoses of proximal and distal ends.

32
Q

What is the pathophysiology of biliary atresia?

A
  1. Atresia of common bile duct/hepatic ducts.
  2. Bile produced but unable to be secreted into small bowel.
  3. Inflammation of the bile ducts and liver
33
Q

What is this a presentation of?

Presents in first few weeks of life with prolonged jaundice (>14 days), pale stools, dark urine, poor weight gain.

A

Biliary atresia

34
Q

How is biliary atresia diagnosed?

A
  1. Conjugated hyperbilirubinaemia, deranged LFTs
  2. USS to exclude other causes of cholestasis.
  3. Gold standard - radioisotope scans showing no excretion of radioisotope-labelled bile from liver.
35
Q

What is the management for biliary atresia?

A

Hepatoportoenterostomy (Kasai’s procedure)

36
Q

What is a hernia?

A

A protrusion of an internal organ/tissue through a weakness in its containing cavity into an abnormal position.

37
Q

What is the most common type of hernia in children and why?

A

Inguinal hernia due to patent processus vaginalis

38
Q

Which gender is more likely to get an inguinal hernia in children?

A

Male (M:F = 9:1)

39
Q

Which type of inguinal hernia are children most likely to get and at what age does it change?

A
  1. Almost always indirect

2. >25 years old direct is more likely

40
Q 
What is this a presentation of?
Painless swelling (may be intermittent in inguinal region/scrotum, exacerbated by crying), impalpable from above, can be reduced.
A

Inguinal hernia

41
Q

What is this a presentation of and how would you manage it?

Painful, firm mass in inguinal region/scrotum, intestinal obstruction, irreducible.

A

Incarcerated inguinal hernia - emergency surgery as there is a risk of strangulation

42
Q

How is an inguinal hernia diagnosed?

A
  1. Clinical examination - can you palpate above it? (to differentiate from hydrocele)
  2. USS if diagnostic uncertainty
43
Q

What is the management for an inguinal hernia in an infant?

A

Six/two rule:

  1. Baby <6 weeks - operate within 2 days
  2. <6 months - within 2 weeks
  3. <6 years - within 2 months
44
Q

What is the management for an umbilical hernia?

A
  1. 80% spontaneously close by age 5, observe.
  2. If small - elective repair at 4-5 years of age
  3. If large - elective repair at 2-3 years of age

Paediatrics (148 decks)

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