Neonatal GITGU Flashcards

1
Q

What is a tracheoesophageal fistula?
What is the most common type?
How would it present antenatally (3)
How would it present postnatally (3)
What is it typically associated with? (2)
How would you confirm this diagnosis?
How would you treat?

A

faulty separation between embryonic trachea and foregut (esophagus)
Most common type: Atresia with distal fistula

Antenatally: Polyhydramnios =>Absent gastric bubble and distended upper esophagus.

Postnatally: Infant unable to swallow saliva => choking and bubbles from mouth
Respiratory distress and abdominal distension (air into stomach from fistula)

Associated with T21/18, Di George’s Syndrome

Ix: Attempt to pass NG tube into stomach and X ray it

Tx: NPO until Surgical reconstruction
Replogle tube to drain saliva
Respiratory support

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2
Q

What are some long term complications if tracheoesophageal fistula?

A

Think logically
Anastomatic leak
Recurrent fistula
esophageal stricture
GORD
Abnormal motility
recurrent cough from lodged food

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3
Q

Congenital Diaphragmatic Hernia:
What is it due to?
Which side is most commonly affected?
What is the most common type of hernia?
What would be the typical presentation?
What would be a typical finding on examination to aid diagnosis?

How would you manage this patient

A

Failure of closure of pleuroperitoneal canal
Left sided 6:1
Bockdalek hernia (90%)

Typically present within first few hours of life with sx of respiratory distress

On examination the patient will have a Scaphoid abdomen (chest larger than abdomen) and there will be audible bowel sounds in the chest.

1) Immediate stabilization ABCD avoiding bag and mask.
a) Intubate and give CPAP (> Mechanical > iNO > HFOV (oscillation) > ECMO) - consider surfactant
b) Pass NG tube for gastric decompression
c) IV access with central and arterial line (ABG)
d) take Pre and Post-ductal saturations
e) Give fluids
2) Surgical repair: returning contents to abdomen and closing defect

Note: Later presentations often indicate better outcomes as they were able to survive and grow for longer before having symptoms

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4
Q

What do you see in this Xray
What is the most likely diagnosis?

A

Absence of visible diaphragm
Absence of bowel loops in chest and presence of bowel loops in chest
Contralateral mediastinal shift with small contralateral lung
Tip of NG tube is in the chest

=> Congenital Diaphragmatic Hernia

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5
Q

How would you deal with someone who is acidotic?

A

Respiratory stimulation: oxygen therapy, caffeine > high flow Nasal cannula > CPAP > Mechanical ventilation > HFOV > ECOM
Metabolic: Sodium bicarbonate

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6
Q

A Premature infant is born small for their gestational age. The mother reports oligohydramnios and the following US was performed. What is the diagnosis?

A

Gastroschisis

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7
Q

Gastroschisis
How would you diagnose this?
What is the typical location of this defect?
What is the main concern on delivery?
How would you manage this patient at delivery?
Surgery is performed, but the contents failed to be reduced back. What would you do next? What are the risks of that and how would you prevent it?

A

Antenatal US
Typically, the defect is located laterally to the right of the intact umbilicus

The main concern is heat and fluid loss => Wrap the abdomen with cling film.
Pass NG tube (wide bore!!)
IV fluids (100ml/kg/day)
Antibiotics
Immediate transfer to neonatal surgical unit to reduce contents back into abdomen

If surgery fails, use Silastic “Silo” bag. This may compromise renal perfusion and has a severe risk of necrotizing enterocolitis => TPN

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8
Q

How would you distinguish between Gastroschisis and Exomphalos?

A

In Exomphalos, the contents are enclosed in a sac or membrane. It may also contain organs such as the liver. Gastroschisis also is not associated with any syndromes unlike exomphalos

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9
Q

What is Exomphalos?
What trisomies is it associated with?

A

It is the failure of the gut to return to the abdominal cavity in the first trimester.
associated with trisomy 13/18/21

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10
Q

How is exomphalos classified?
How is it diagnosed?
How would you manage this?
Surgery could not be performed due to major cardiac abnormalities as well as the fact that the defect is too large to repair primarily. What will you do?

A

Based on size of defect where >5cm is major (<5 minor) as well as the presence of absence of the liver in the sac
It is diagnosed antenatally via US

Management: Similar to gastrorchisis
Cling film if not covered by membrane
NG tube (wide bore!!)
Surgery: Primary closure
If surgery could not be performed (reasons in question), then treat the sac with topical antiseptics (Silver Sulphadiazine) until the surface has epithelialised and close later by direct closure (instead of primary)

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11
Q

You note bile-stained vomit when taking a history from a mother and her child. Is this a surgical emergency?

You note that there are blood stains in the vomit as well. What do you suspect?

A

Yes

Malrotation and Volvulus

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12
Q

What is the expected time for a newborn baby to produce meconium?

A

12 hours for most but up to 48 maxx

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13
Q

What are the main indications/signs/findings for Intestinal obstruction in the newborn?

State 5 common reasons of intestinal obstruction

A

1) Bile-stained (green) vomiting (blood stained in volvulus)
2) Abdominal distention (more pronounced in jejunal/ileal atresia)
3) Failure to pass meconium (most will pass by 12 hours, all by 48 hours)

Causes:
1) Malrotation with volvulus
2) Duodenal/Jejunal atresia/stenosis
3) Meconium ileus/Meconium Plug Syndrome
4) Hirshprung disease
5) Necrotizing enterocolitis (and intestinal perforation)

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14
Q

Why does Malrotation with volvulus occur?
What is an indication of this when taking a history?
What is the biggest concern with this?
What do you expect to see on a PFA?
How would you diagnose this? What would you see?

A

Interference with normal retuen of the fetal intestine into the abdominal cavity
Blood in billious vomit
Biggest concern in the occlusion of venous THEN arterial blood flow => necrosis
Normal PFA
Dx: Upper GI contrast study with Corkscrew appearance and duodenojejunal junction fails to cross midline

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15
Q

A premature baby with down’s syndrome appears with bilious vomiting, abdominal distention and failure to pass meconium for the past 38 hours. What is your primary diagnosis?
How would you diagnose this? What would you see?
How would you manage this patient?

A

Duodenal atresia (associated with T21 and Premature births.
Diagnose via Xray - Double bubble appearance (basically showing it has distended the small part of the abdomen and stomach)
Management: NGT > Decompression > Surgery

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16
Q

While inspecting a baby, you were unable to visualize a patent anus. You refer them to surgery. How will the surgery team decide what to perform?

A

High anomalies: Colostomy
Low anomalies: Dilation

17
Q

What is the diagnosis?
What is the main finding in the X-ray
What are the clinical manifestations of the disease?
What might show up on an FBC?
How would you manage this patient? (8)

A

Necrotizing enterocolitis (esp preterm) without perforation
Pneumatosis or the train track/bubbly appearance on Xray

Distended abdomen (maybe red discoloration)
Billious vomit with gastric aspirates
Failure to feed
Blood stained stools
Hypotension/sepsis/Acidosis (blood gas)

FBC: Thrombocytopenia (because of DIC) and anemia? => always take coag screen

Management: ABC!
1) NPO, instead establish IV access for TPN
2) Gastric decompression via NG tube
3) Intubation + Ventilation
4) Circulatory support (inotropes)
5) Sepsis workup and triple IV therapy (Amoxicillin, gentamicin, metronidazole)
6) Analgesia (morphine)
7) Thrombocytopenia - Platelets, Fibrinogen, Vit K
8) Contact Surgery

18
Q

What is the diagnosis?
What would this add to your management?

A

Free air in abdomen => NEC with perforation
If small, unstable baby = Penrose Drain
If large, stable baby = Laparotomy and stoma

19
Q

What is Meconium Ileus?
90% of patients with meconium ileus also have another associated disease. What is it?
What are some complications?

A

Meconium ileus is the obstruction of the distal ileum due to thick concentrated mucus
80-90% neonates have cystic fibrosis (in exam if its not cystic fibrosis then basically go with the plug syndrome)
Complications: Intestinal obstruction, perforation and peritonitis (may occur antenatally as well)

20
Q

What is this procedure?

A

Penrose drain

21
Q

What is Meconium Plug syndrome
What are some RFs (3)
How is it diagnosed?

A

Delayed passage of meconium or intestinal obstruction.

RFs: Premature, infants of diabetics, use of magnesium sulfate (used antenatally to reduce cerebral palsy)

Diagnosis: Contrast Enema showing pebbles of meconium/stool

22
Q

Neonate with delayed passage of meconium > 48 hours and Enterocolitis. What is the most likely diagnosis

A

Hirschprung disease

23
Q

What is Hirschprung’s disease?
What areas of the bowel are most affected?
What is it associated with?

A

Absence of ganglionic cells, most commonly in the rectum and sigmoid colon. Associated with Trisomy 21

24
Q

Posterior urethral valves are the mucosal folds in the posterior urethra of the male infant. Whenever there is an obstruction, what would you expect to see on imaging? What would it be called in this case?

A

Dilatation and thickening of the (renal) tract proximal to the obstruction => in this case, it is bilateral hydroureteronephrosis

25
Q

Posterior urethral valves are the mucosal folds in the posterior urethra of the male infant. How would you diagnose this antenatally and what findings would you expect to see to confirm this?

What would you expect to see post-natal?

What are the consequences of this?

How would you manage this Pre and post-nataly?

A

Diagnosis is via antenatal US.
Oligohydramnios Keyhole appearance of ultrasound showing dilated and thick-walled bladder with poor emptying => bilateral hydroureteronephrosis

Abdominal masses due to bilateral hydroureteronephrosis (distension)

Consequences: Renal insufficiency/failure (=> unable to concentrate urine => diluted urine and polyuria
Vesicoureteral reflux
!Urosepsis!
Bladder dysfunction
Reduced lung function (if no fetal shunt)

Management:
Pre: Fetal shunt placement to correct oligohydramnios and improve lung development (has no effect on renal function)
Post: surgical correction

26
Q

What is hypospadias?
What findings are associated with hypospadius (2)

A

It is the failure/delay in midline fusion of urethral folds causing a lower urethral meatus and may also have Chordee which is the curvature of the penis

27
Q

You are examining a child with hypospadias, what should you advise the parents?

A

Not to circumcise their baby as the foreskin is used to surgically repair the midline ureteral folds

28
Q

How do you assess the severity of hypospadias?

A

It is based on the location of the urethral meatus (lower = worse) and can even be a scrotal meatus.
It is also based on the degree and location of the chordee

29
Q

When do the testes descend into the scrotum?
If they have not descended what is it called?
When palpating a newborn infant, you notice that their left testicle has not descended but ruggae is present bilaterally. What is your management plan?

A

38-40 weeks
Cryptorchidism

The vast majority of these cases will have spontaneous descent by 9 months. Advise parents to return if it hasnt returned by then and conduct surgical intervention if not descended by 12 months.

30
Q

Why is surgery performed on infants with cryptorchidism at 12 months and not at 9?

A

Around the start of the second year of life, changes in the undescended seminiferous tubule occur on US

31
Q

What is the cause of an inguinal hernia in newborns?

This is often confused with a hydrocele. How can you tell if it is an inguinal hernia? (4)

How does the management differ?

A

Persistence of the patent processus vaginalis

No Transillumination
Cant reach under and over swelling
Can be reduced
Bowel sounds over area

Inguinal hernias need to be surgically repaired whereas a hydrocele usually resolves itself by 1 year

32
Q

What is congenital adrenal hyperplasia (CAH)
What is the most common type?
What are the biochemical consequences of that type?
How would this affect girls vs boys?

A

It is the deficiency of one of the enzymes in the biosynthetic pathway in the adrenal cortex.
The most common type is the deficiency of 21-hydroxylase causing the increased production of androgens and progesterone (leading to virilization) and block in cortisol and aldosterone

Girls: Ambiguous genitalia
Boys: Hyperpigmentation of scrotum, penile enlargement, early puberty
Both: premature adrenarche
Note: with hyperplasia comes tumors!!!

33
Q

Overtreatment of Congenital adrenal hyperplasia can lead to?

A

Cushing Syndrome
This is the overproduction of cortisol

34
Q

How do you ask about colicky pain in infants
Where is this seen?

A

Do they tend to draw up their legs when they feel this pain?

Intussusception
Acute appendicitis
Colic (crying child)