GI Malformations/Jaundice Flashcards

1
Q

A Tracheoesophageal Fistula is associated with what anomalies?

A

VACTERL
- Vertebral
- Anal
- Cardiac
- Tracheal
- Esophageal
- Renal
- Limb

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2
Q

What are the signs of a Tracheoesophageal Fistula?

A

Polyhydramnios in utero
Increased oral secretions
Inability to feed/gagging
Aspiration

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3
Q

If Esophageal Atresia is present, what will be a diagnostic on a CXR?

A

If an NG tube is coiled in the esophagus

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4
Q

With a Tracheoesophageal Fistula, what will be seen on XR?

A

Air in the GI tract

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5
Q

What occurs with a Diaphragmatic Hernia?

A

Bowel segments protrude through the left side of the diaphragm into the thorax

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6
Q

What are the signs of a Diaphragmatic Hernia?

A

Sunken abdomen with bowel sounds present over the left chest
– Pulmonary hypoplasia/HTN causing respiratory distress!

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7
Q

What is Gastroschisis?

A

Herniation of red, matted bowel through the abdominal wall NOT midline and NOT covered in peritoneum

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8
Q

What are 2 treatment options for Gastroschisis?

A

Surgery
Silo bag can gradually reduce bowel into abdomen

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9
Q

What is an Omphalocele?

A

Herniation of intestine through the abdominal wall at the level of the umbilicus and IS covered by peritoneum/amniotic membrane

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10
Q

Omphaloceles are associated with what syndrome?

A

Beckwith-Wiedemann

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11
Q

Duodenal Atresia involves failure of the duodenum to recanalize during gestation. What 2 conditions are often present?

A

Down Syndrome
Annular pancreas

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12
Q

What is the main symptom of Duodenal Atresia?

A

Bilious emesis after birth

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13
Q

What will be seen on XR with Duodenal Atresia?

A

Double bubble

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14
Q

What can cause Jejunal Atresia?

A

Cocaine or other vasoconstrictive substances that alter vasculature in utero

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15
Q

What will be seen on XR with Jejunal Atresia?

A

Triple bubble

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16
Q

GI malformations will often have what presenting sign in utero?

A

Polyhydramnios due to defective swallowing

17
Q

What type of bilirubin is always pathologic?

A

Conjugated (direct)

18
Q

What is Kernicterus and at what level of UNconjugated bilirubin can it occur?

A

Deposition of unconjugated bilirubin in the basal ganglia, pons, cerebellum
– > 25

19
Q

What are the signs of Kernicterus? (4)

A

Lethargy
Hypertonia
High pitched screaming cry
Seizures

20
Q

Unconjugated bilirubin may be physiologic in nature. What are the features of physiologic jaundice?

A
  • Not present at birth and resolves in 1-2 weeks
  • Levels increase by < 5/day
  • Levels peak at < 14
  • LOW Conjugated bilirubin
21
Q

What are the features of pathologic jaundice?

A
  • Present at birth and does not resolve
  • Levels increase by > 5/day
  • Levels peak at > 14
  • HIGH conjugated bilirubin
22
Q

What are 3 causes of high Unconjugated Bilirubin?

A

Hemolysis of RBCs
Impaired conjugation
Increased enterohepatic circulation

23
Q

What are examples of RBC hemolysis that will cause elevated Unconjugated bilirubin?

A

ABO/Rh incompatibility
G6PD deficiency
RBC structure

24
Q

What are 3 causes of impaired conjugation that will cause elevated Unconjugated bilirubin?

A

Physiologic jaundice of the newborn
Gilbert Syndrome
Criggler-Najjar Syndrome

25
Q

What are 3 examples of increased enterohepatic circulation that will cause elevated Unconjugated bilirubin?

A

Poor feeding
Breast milk jaundice
Pyloric stenosis

26
Q

What are the 2 treatment options for Unconjugated hyperbilirubinemia?

A

Phototherapy
Exchange transfusion (> 20)

27
Q

What workup should be done for elevated Unconjugated bilirubin?

A

CBC with peripheral blood smear
Coombs test
Blood typing

28
Q

What workup should be done for elevated Conjugated bilirubin?

A

LFTs and bile acids
Blood cultures
Metabolic disorders
US and HIDA scan

29
Q

What are 2 causes of high Conjugated Bilirubin?

A

Impaired secretion from the liver
Obstruction of biliary tree

30
Q

What are 2 syndromes that cause elevated Conjugated bilirubin by impaired secretion from the liver?

A

Dubin Johnson
Rotor