Development Clinical Correlates

Question 1

esophageal atresia

Answer 1

Causes:

• EA w/ Tracheoesophageal Fissure –> deviation of the tracheoesophageal septum in a POSTERIOR direction, which results in incomplete separation of the esophagus from the laryngotracheal tube.
• Isolated EA: Failure of recanalization

Clinical Presentation: polyhydraminos in utero; excessive drooling; inability to feed without immediate regurgitation & coughing;
Inability to pass a catheter through the esophagus into the stomach

Diagnosis/Treatment: Surgical repair of esophageal atresia now results in survival rates > 85%.

Question 2

Esophageal stenosis

Answer 2

Causes: usually occurs in the distal third of the esophagus

• Incomplete recanalization of esophagus during 8th week
• Failure of esophageal blood vessels to develop, resulting in atrophy of a segment of the esophageal wall.

Clinical Presentation: swallowing problems once weaning off milk (liquid to semi-solid)

Diagnosis/Treatment: surgical dilation of esophagus

Question 3

Pyloric stenosis

Answer 3

Causes: hypertrophy of smooth muscle in wall of the pylorus resulting in severe stenosis of the pyloric canal

Clinical Presentation: Stomach distention; olive-shaped palpable mass; projectile vomiting (w/out bile!!)

Diagnosis/Treatment: Abdominal X-ray w/ dye –> see “string sign.” Pyloromyotomy–> cut through muscle to widen pylorus.

Question 4

Duodenal stenosis

Answer 4

Causes: Defective vacuolation (incomplete recanalization. Usually occurring in horizontal and/or ascending parts of duodenum.

Clinical Presentation: Vomiting (most likely bilious); variable presentation in age & symptoms.

Diagnosis/Treatment: Abdominal X-Ray shows air that has made its way to small intestines

Question 5

Congenital Duodenal atresia

Answer 5

Causes: Duodenum lumen fails to recanalize. Subsequent block usually occurs around the hepatopancreatic ampulla, but may occur in the horizontal part of the duodenum.

Clinical Presentation: polyhydraminos in utero; bilious vomit seen shortly after birth; May see distention of epigastrium.

Diagnosis/Treatment: Abdominal X-ray or prenatal US showing DOUBLE BUBBLE sign. Treated with duodenoduodenostomy.

**associated with other congenital anomalies, esp. Down syndrome, and premature birth!

Question 6

Extrahepatic biliary atresia

Answer 6

Causes: Obliteration or absence of all or part of bile ducts at or superior to the porta hepatis (a deep transverse fissure on the visceral surface of the liver). Likely due to an acquired inflammatory lesion which causes fibrosis.

Clinical Presentation: jaundice, dark urine, acholic stools (lacking color)

Diagnosis/Treatment: Kasai Procedure!! Liver transplant if procedure unavailable.

Question 7

Annular pancreas

Answer 7

Band of pancreatic tissue surrounding 2nd part of duodenum causing constriction.

Causes: growth of bifid ventral pancreatic bud around duodenum that fuses with the dorsal bud to form a rung

Clinical Presentation: can be asymptomatic; complete/partial bowel obstruction, especially if there is pancreatitis.

Diagnosis/Treatment: surgery to bypass annular pancreas; could show on abdominal X-ray as double bubble.

Question 8

When does hematopoiesis occur? Where does it move to?

Answer 8

week 6; Liver –> Spleen –> Bone marrow

Question 9

When does bile formation and secretion occur?

Answer 9

Forms week 12 and enters duodenum week 13

Question 10

omphalocoele

Answer 10

Failure of regression back into abdomen around week 10

Question 11

“vitelline” means

Answer 11

yolk sac

Question 12

“cloacal” means

Answer 12

anus

Question 13

ductus venosus

Answer 13

shunt bypassing 1/2 of blood from the liver. Connects the umbilical vein to the Inferior Vena Cava

Question 14

ductus venosus

Answer 14

shunt bypassing 1/2 of blood from the liver. Connects the umbilical vein to the Inferior Vena Cava

Question 15

Transposition of the viscera

Answer 15

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Question 16

Nonrotation of gut

Answer 16

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Question 17

Malrotation of Gut

Answer 17

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Question 18

Midgut volvulus

Answer 18

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Question 19

Meckel’s diverticulum

Answer 19

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Question 20

Omphalocele

Answer 20

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Question 21

Gastroschisis

Answer 21

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Question 22

Intestinal stenosis

Answer 22

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Question 23

Intestinal atresia

Answer 23

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Question 24

Umbilical Hernia

Answer 24

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Question 25

Imperforate anus

Answer 25

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Question 26

Anorectal agenesis

Answer 26

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Question 27

Anorectal atresia

Answer 27

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Question 28

Anal agenesis

Answer 28

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Question 29

Anal Stenosis

Answer 29

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Question 30

Cloacal fistulas

Answer 30

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Question 31

Persistant cloaca

Answer 31

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Question 32

Congenital megacolon (Hirschsprung Disease)

Answer 32

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