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Paediatrics > Neonatal Surgery (TOF; intussusception; CDH) > Flashcards

Neonatal Surgery (TOF; intussusception; CDH) Flashcards

1
Q

What is the incidence of TOF?

A

1:3000 - 1:4500

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2
Q

What are the anomalies often associated with TOF?

A

Associated anomalies in 50%: VACTERL association

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3
Q

What is VACTERL?

A 
Disorder that affects many body systems. Stands for:
- Vertebral defects
- Anal atresia
- Cardiac defects
- TOF
- Renal abnormalities
- Limb abnormalities
Generally 3+ for VACTERL diagnosis
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4
Q

What are the CFx of TOF?

A 
VARIABLE with fistula type.
KEY = FROTHY 
- Hx Maternal polyhydramnios
Several months (no atresia):
- Non - bilious vomiting
- Cyanosis with feeds
- Coughing
- Respiratory distress
- Recurrent pneumonia
- Frothy bubbles of mucous in mouth and nose that return after suctioning
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5
Q

Rx TOF?

A
  • Call PIPER
  • Transfer out with films
  • Check for other abnormalities (VACTERL)
  • Early repair with surgical ligation to prevent lung damage and maintain nutrition and growth
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6
Q

What are the complications of TOF?

A
  • Pneumonia
  • Sepsis
  • Reactive airways disease
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7
Q

What are the complications of TOF post repair?

A
  • Oesophageal stenosis and strictures at site
  • GORD
  • Poor swallowing (dysphagia, regurg)
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8
Q

What is the most common cause of bowel obstruction between 6-36mo?

A

Intussusception

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9
Q

What are the pathologic lead points of intussusception?

A
  • Enlarged Peyer’s patches due to viral infections of GIT
  • Polyps
  • Meckel’s diverticulum
  • Lymphoma
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10
Q

What is the pathophysiology of intusussception?

A

Usually idiopathic.

  • Often starts at ileocaecal junction
  • telescoping of bowel into itself causing obstruction and vascular compromise
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11
Q

CFx intussusception?

A
  • Acute onset abdo pain
  • Episodic, “colicky”
  • Vomiting +/- bilious
  • Abdo mass
  • Red currant jelly stools suggests mucosal necrosis and sloughing
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12
Q

PEx features intussusception?

A

Abdo exam:

  • palpate for masses (esp SAUSAGE shaped upper abdo mass) and tenderness
  • Signs BO: distension
  • Look for localised peritonitis = transmural ischaemia
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13
Q

Ix intussusception?

A
  • AXR for BO or perforation

- US if suspect pathology

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14
Q

Rx intussusception?

A
  • Peritonitis = operative

- No peritonitis = air enema reduction

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15
Q

Prongosis intussusception?

A
  • 10% recurrence (more likely non - idiopathic)
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16
Q

What is the incidence of intestinal atresia?

A

2 - 14%

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17
Q

What is the pathophysiology of duodenal atresia?

A

Failure of bowel to recanalize after endodermal epithelium proliferation (W8 - 10)

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18
Q

What is the pathophysiology of jejunal / ileal atresia?

A

Acquired as result of vascular disruption -> ischaemic necrosis -> resorption of necrotic tissue -> blind distal and proximal ends

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19
Q

CFx duodenal atresia?

A
  • Gastric distension and vomiting (usu Bilious)
  • VACTERL association
  • 24% have DS
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20
Q

PEx approach to intestinal atresia?

A
  • Complete physical (esp abdo, anus)
  • Evaluate for resp distress
  • Evaluate volume status
  • Congenital anomalies (VACTERL)
  • Jaudice
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21
Q

Ix intestinal atresia?

A
  • Contrast enema +/- UGI with small bowel follow through
  • Group and screen
  • INR and PTT if for surgery
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22
Q

Rx intestinal atresia?

A
  • NBM
  • NG tube decompression
  • Fluid resuscitation
  • TPN
  • Broad spec ABx
  • Surgery
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23
Q

What is the incidence of hypertrophic pyloric stenosis?

A

0.03 - 1% live births

24
Q

When do infants with hypertrophic pyloric stenosis generally present?

A

1 - 20w; most common = 6-8w

25
Q

What is the gender spread of hypertrophic pyloric stenosis?

A

4M:1F

26
Q

Which ABx represents a RFx for HPS?

A

Early erythromycin exposure (<13d)

27
Q

What is the pathophysiology of HPS?

A
  • Acquired pyloric circular muscle hypertrophy resulting in gastric outlet obstruction.
  • Hypovolemia due to vomiting of gastric contents ==> hypochloremic hypokalemic metabolic alkalosis
28
Q

CFx HPS?

A
  • Projectile non bilious vomiting 30 - 60min after feeds.
  • Hungry after vomiting
  • Dehydration
29
Q

PEx features HPS?

A

“Olive”: 1-2cm smooth palpable mass above umbilicus.

- visible L - R gastric contraction “waves” after feeding

30
Q

Ix HPS?

A

-UEC: ELECTROLYTES
- US: pyloric length 14mm+ and thickness 4mm+
Upper GI series only when US unavailable or not diagnostic: will show “string sign”

31
Q

Mx HPS?

A
  • Fluid resuscitation with NSal
  • Correct acid/base anomalies with D5, 1/2NSal + KCl maintenance
  • NG tube decompression not required
  • Pyloromyotomy (Ramstedt vs transumbilical or lap approach)
32
Q

What are the types of congenital diaphragmatic hernia?

A

3 types of CDH:

  • Posterolateral (Bochdalek; 85% on LHS)
  • Anterior (Morgagni)
  • Hiatus
33
Q

When does CDH present

A

Within hours of life

34
Q

What is the pathophysiology of LHS CDH?

A
  • SB and LB
  • Stomach
  • Solid viscera (spleen, L lobe of liver)
    herniate into thorax
    PLUS:
  • pulmonary hypoplasia
  • pulmonary HTN
35
Q

What is the pathophysiology of RHS CDH?

A

-Liver
- LB
Herniate into thorax
PLUS:
- pulmonary hypoplasia
- pulmonary HTN

36
Q

What are the CFx of CDH?

A
  • Early respiratory distress
  • Cyanosis
  • Scaphoid abdomen
  • Prenatal diagnosis
37
Q

PEx features of CDH?

A
  • Decreased air entry +/- bowel sounds in the chest

- Displaced heart sounds (R as most hernias on L)

38
Q

Ix in CDH?

A
  • Prenatal US / MRI
  • ABG
  • CXR (bowel loops in hemithorax, shifted heart)
  • Echo
  • Genetic consultation if warranted
39
Q

Mx CDH?

A
  • Intubate
  • Orogastric suction
  • KEY: tertiary transfer
  • Period of respiratory stabilisation due to associated pulmonary hypoplasia (may need ECMO)
  • Surgical repair when stable
40
Q

What is malrotation?

A

Failure of to normally rotate around superior mesenteric artery with associated abnormal intestinal attachments and anatomic positions. Spectrum of rotation abnormalities

41
Q

Age of presentation of malrotation?

A
  • 1/3 by 1w
  • 3/4 by 1mo
  • 90% by 1y
42
Q

What is the cardinal sign of malrotation?

A

Bilious vomiting (esp if non-distended abdomen)

43
Q

What are the PEx features of malrotation?

A
  • Bilious drainage from NGT
  • Tachycardic, pale
  • Diaphoretic
  • Flat abdomen
  • Tenderness
44
Q

AXR features of malrotation?

A
  • obstruction of proximal small bowel
  • “double bubble” sign
  • intestinal wall thickened
45
Q

Mx malrotation?

A
  • IV ABx
  • Fluid resuscitation
  • Emergent laparotomy:
    LADD procedure
46
Q

What is Ladd procedure?

A
  • Couterclockwise reduction of midgut volvulus
  • division of Ladd’s bands
  • division of peritoneal attachments b/w caecum and abdo wall that obstruct duodenum
  • broadening of mesentery
  • +/- appendicectomy
47
Q

What is gastroschisis?

A

Defect of abdominal wall with free extrusion of intestine in amniotic cavity

48
Q

mx gastroschisis?

A
  • NG decompression
  • IVF
  • IVABx
  • Call PIPER
  • Put in humidiheat
  • Keep viscera moist and protected (GLAD WRAP) until surgical reduction with primary abdo closure OR staged closure with silo
  • May have bowel dysmotility and require motility medications
49
Q

What is gastroschisis associated with?

A
  • younger maternal age

- IUGR

50
Q

What is an omphalocele?

A
  • defect of abdominal wall with extrusion of sac covered viscera
51
Q

What is the rule of 2s for Meckel’s diverticulum?

A
  • 2% of population
  • 2M : 1F
  • 2% symptomatic
  • Within 2ft of IC valve
  • 2 inches in diameter
  • 2 inches in length
  • 2 types of tissue (gastric, pancreatic)
  • Often presents by 2y of age
52
Q

What does bilious vomiting represent in an infant?

A

Life threatening emergency secondary to midgut volvulus until proven otherwise

53
Q

What is the pathophysiology of Hirschsprung’s disease?

A

Defect in migration of neurocrest cells to intestine resulting in:

  • aganglionic bowel that fails to peristalse and
  • internal sphincter that fails to relax (internal anal sphincter achalasia)
54
Q

CFx of Hirschsprung’s disease?

A
  • Failure to pass meconium in first 48h

- Sx of BO: distension, constipation, bilious emesis

55
Q

Ix Hirschsprung’s disease?

A
  • Rectal biopsy (aganglionosis and neural hypertrophy)
  • AXR
  • Contrast enema (narrow rectum and transition zone)
56
Q

What is the first Ix to perform in ?intussusception?

A

AXR to exclude perforation or obstruction

57
Q

What should be completed after AXR in intussusception work up?

A

US

Paediatrics (31 decks)

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