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1.02 Developmental GI Anomalies Flashcards

(49 cards)

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1
Q

What is the most common kind of TEF/EA?

A

Esophageal atresia + tracheoesophageal fistula

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2
Q

The most common TEF/EA complications will appear as what clinically?

A

Feeding intolerance = choking
Bronchitis/pneumonia = aspiration
GERD = spitting up
Strictures = increased secretions

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3
Q

What condition is associated with a 50% increased likelihood of additional abnormalities?

A

TEF/EA

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4
Q

If you clinically suspect TEF/EA what are the next steps?

A

Insert a NG/OG tube an order radiograph
(+) if tube in upper thorax

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5
Q

What prenatal finding hints at a possible duodenal or esophageal atresia?

A

Polyhydraminos

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6
Q

Down Syndrome is associated with what condition?

A

Duodenal atresia

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7
Q

What differentiates a duodenal atresia from an esophageal one on X-RAY?

A

Double bubble sign

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8
Q

What is the clinical presentation of newborn with duodenal atresia?

A

Bilious emesis in first two days
Distended abdomen

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9
Q

You notice a newborn with an umbilical hernia, next steps?

A

Watch, wait, restraint
Surgery IF still present at 5 years old

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10
Q

Where does an omphalocele originate?

A

Umbilical base/midline stalk

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11
Q

What should you consider in a child born with omphalocele that plays a part in prognosis?

A

Associated anomalies are common (50%) - prognosis based on it

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12
Q

What are risk factors for omphalocele?

A

Beckwith-Weidemann
Trisomy 13 & 18

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13
Q

How can you tell an omphalocele and gastroschisis apart?

A

Gastroschisis has no sac (no amnion, no jelly, no peritoneum)

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14
Q

Where does gastroschisis defect originate?

A

Right of umbilicus

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15
Q

What are the risk factors and prognosis related to gastroschisis?

A

Volvulus risk, intestinal atresia, and necrotizing enterocolitis (NEC)**

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16
Q

An omphalocele and gastroschisis are due to failure of what in embryonic development?

A

Lateral folding closure and return of gut to abdominal cavity

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17
Q

What is the plan if a child is born with omphalocele or gastroschisis?

A
  1. Cover exposed area with saline soaked dressing
  2. Gastric decompress, bowel bag, surgery
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18
Q

TEF/EA is due to a defect at what stage (when) in development?

A

Week 4-6

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19
Q

Duodenal atresia is due to a defect at what stage (when) in development?

A

Week 8-10 recanalization

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20
Q

Duodenal atresia is associated with what anomalies?

Study These Flashcards
A

Trisomy 21, malrotation, congenital heart disease, renal anomaly

21
Q

What is the preferred what to visualize a duodenal atresia?

Study These Flashcards
A

Upper GI contrast radiography

22
Q

Duodenal atresia plan?

Study These Flashcards
A
  1. Nasogastric decompress
  2. Ped surgery consult
  3. IV hydrate +stabilize
  4. Surgery
23
Q

What happens during intestinal malrotation?

Study These Flashcards
A

It will rotate at most 180 (vs. 270), could wrap around the SMA, and be held back by Ladd bands

24
Q

During a upper GI contrast series, what indicates a midgut volvulus?

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A

“Corkscrew sign” of dilated bowel loops and duodenum doesn’t cross vertebrae

25
What causes a midgut volvulus?
Blood flow at SMA compromised leading to ischemia — bilious vomiting (clinical sign)
26
What is the plan for a midgut volvulus?
Urgent Surgery!! Delay = necrosis, morbidity, mortality
27
What is the treatment plan for patient with intestinal malrotation and symptomatic/asymptomatic for less than 12months?
Ladd procedure
28
What is the treatment plan for a patient with intestinal malrotation if asymptomatic and OVER 12 months?
Surgical consult (+/-)
29
Heterotaxy syndrome present in 1% of the population leads to a high occurrence of what condition?
Intestinal malrotation; isomerism (left/right) vs. situ solitude (normal)
30
When do 95% of infants express meconium?
Within first 24 hours
31
Next step if newborn goes over 24 hours without meconium?
Plain abdominal radiograph
32
What is considered diagnostic and therapeutic for meconium ileus and meconium plug syndrome?
Contrast enema (barium)
33
What often causes meconium plug syndrome?
Maternal treatment of preeclampsia with magnesium-sulfate
34
What are indications for meconium plug syndrome?
No abdominal findings or gradual increase in girth with no other signs of illness, did not pass 24hr meconium, plain radiograph is nonspecific
35
Hirschsprung disease is a congenital defect where?
Parasympathetic postganglionic neuron cell bodies are not present (aganglionic section) leading to a proximal megacolon
36
The sensory neurons and Schwann cells of para nervous system and all enteric/autonomic ganglia are derived from what embryonic tissue?
Ectoderm - neural crest cells
37
What are some anomalies associated with Hirschsprung Disease?
RET protooncogene, trisomy 21, Waardenburg Syndrome
38
What section of the colon is most commonly aganglionic in Hirschsprung?
Rectosigmoid colon
39
What are the clinical features that differentiate Hirschsprung from a meconium plug?
Feeding intolerance, bilious vomit, severe constipation/obstruction, abdominal distention**
40
What is the gold standard diagnosis for Hirschsprung?
Suction rectal biopsy 2 cm above dentate line
41
You suspect Hirschsprung, next step?
Digital exam & evacuation -> large explosive could smelling bowel movement
42
What will you find in Hirschsprung histology?
Axon hyperplasia
43
What is the plan for Hirschsprung?
Surgical repair If not.. toxic megacolon risk
44
What are possible complications following surgical repair of Hirschsprung disease?
Constipation, fecal soiling, prolapse, and stricture
45
Meconium ileus over 95% of teh time associated with what condition?
cystic fibrosis
46
What are signs that differentiate meconium ileus from some other conditions in assessment?
Rectal exam has narrow vault, radiograph has dilated bowel oops, and barium enema shows “soap bubble” in ilium and the entirety of colon = micro
47
What causes the meconium in meconium ileus to be thick, dry, and smelly?
CF = decreased pancreatic enzymes = not processed correctly Malabsorption of carbs, fat, and protein
48
What increases the risk and is associated with neonatal small left colon syndrome?
Diabetic mothers
49
Small left colon syndrome affects what part of the colon?
Descending colon ONLY

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