Congenital Anomalies

Question 0

Symptoms of Esophageal Atresia/TEF

Answer 0

Respiratory distress, coughing fits, copious oral secretions, aspiration
NG/OG coiled in esoph pouch on xray

• 50% have h/o maternal polyhydramnios
• 50% have associated anomalies
• 33% have VACTERL

Question 1

Types of Esophageal Atresia/TEF

Answer 1

Most EA have associated TEF
- most common proximal esoph ends in blind pouch
distal esoph has fistula to trachea
- less common proximal esoph has fistula to trachea
distal esoph blind pouch or also fistula to trachea
- can have EA w/o TEF
proximal and distal esoph end in blind pouches
NO AIR in intestines
- can have TEF w/o EA
full esoph but also fistula to trachea

Question 2

Meckel Diverticulum

Answer 2

remnant of embryonic yolk sac
omphalomesenteric/vitelline duct doesn’t involute
presents w/lower GI bleed or intussusception/volvulus
contains gastric mucosa which secretes acid&raquo_space; ulceration of
nearby intestine

Rule of 2’s:

• 2% infants
• 2 inches long
• 2 feet from ileocecal valve
• presents <2yo

Question 3

Biliary Atresia

Answer 3

atresia/hypoplasia of any part of extrahepatic biliary tree
hepatobiliary scan: tracer doesn’t leave liver and enter intestines
liver bx: bile duct proliferation, portal system dilation
Kasai procedure- portoenterostomy
transect intestine
connect distal part to liver to directly drain bile
connect proximal part to side of distal part
80% eventually need liver transplant
*most common cholestasis in non-premie 0-3mosj

Question 4

Hypertrophic Pyloric Stenosis

Answer 4

Emesis > loss of HCl
Alkalosis > kidneys retain H and lose K
Decreased intravascular volume > relative increased HCO3
(Contraction alkalosis)
Alkalosis > K moves into cells in exchange for H
*HPS classically&raquo_space; hypokalemic hypochloremic metabolic alkalosis