1.02 Developmental GI Anomalies Flashcards

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?

A

Trisomy 21, malrotation, congenital heart disease, renal anomaly

21
Q

What is the preferred what to visualize a duodenal atresia?

A

Upper GI contrast radiography

22
Q

Duodenal atresia plan?

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

What happens during intestinal malrotation?

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?

A

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

25
Q

What causes a midgut volvulus?

A

Blood flow at SMA compromised leading to ischemia — bilious vomiting (clinical sign)

26
Q

What is the plan for a midgut volvulus?

A

Urgent Surgery!!
Delay = necrosis, morbidity, mortality

27
Q

What is the treatment plan for patient with intestinal malrotation and symptomatic/asymptomatic for less than 12months?

A

Ladd procedure

28
Q

What is the treatment plan for a patient with intestinal malrotation if asymptomatic and OVER 12 months?

A

Surgical consult (+/-)

29
Q

Heterotaxy syndrome present in 1% of the population leads to a high occurrence of what condition?

A

Intestinal malrotation; isomerism (left/right) vs. situ solitude (normal)

30
Q

When do 95% of infants express meconium?

A

Within first 24 hours

31
Q

Next step if newborn goes over 24 hours without meconium?

A

Plain abdominal radiograph

32
Q

What is considered diagnostic and therapeutic for meconium ileus and meconium plug syndrome?

A

Contrast enema (barium)

33
Q

What often causes meconium plug syndrome?

A

Maternal treatment of preeclampsia with magnesium-sulfate

34
Q

What are indications for meconium plug syndrome?

A

No abdominal findings or gradual increase in girth with no other signs of illness, did not pass 24hr meconium, plain radiograph is nonspecific

35
Q

Hirschsprung disease is a congenital defect where?

A

Parasympathetic postganglionic neuron cell bodies are not present (aganglionic section) leading to a proximal megacolon

36
Q

The sensory neurons and Schwann cells of para nervous system and all enteric/autonomic ganglia are derived from what embryonic tissue?

A

Ectoderm - neural crest cells

37
Q

What are some anomalies associated with Hirschsprung Disease?

A

RET protooncogene, trisomy 21, Waardenburg Syndrome

38
Q

What section of the colon is most commonly aganglionic in Hirschsprung?

A

Rectosigmoid colon

39
Q

What are the clinical features that differentiate Hirschsprung from a meconium plug?

A

Feeding intolerance, bilious vomit, severe constipation/obstruction, abdominal distention**

40
Q

What is the gold standard diagnosis for Hirschsprung?

A

Suction rectal biopsy 2 cm above dentate line

41
Q

You suspect Hirschsprung, next step?

A

Digital exam & evacuation -> large explosive could smelling bowel movement

42
Q

What will you find in Hirschsprung histology?

A

Axon hyperplasia

43
Q

What is the plan for Hirschsprung?

A

Surgical repair
If not.. toxic megacolon risk

44
Q

What are possible complications following surgical repair of Hirschsprung disease?

A

Constipation, fecal soiling, prolapse, and stricture

45
Q

Meconium ileus over 95% of teh time associated with what condition?

A

cystic fibrosis

46
Q

What are signs that differentiate meconium ileus from some other conditions in assessment?

A

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
Q

What causes the meconium in meconium ileus to be thick, dry, and smelly?

A

CF = decreased pancreatic enzymes = not processed correctly
Malabsorption of carbs, fat, and protein

48
Q

What increases the risk and is associated with neonatal small left colon syndrome?

A

Diabetic mothers

49
Q

Small left colon syndrome affects what part of the colon?

A

Descending colon ONLY