Path: Pediatric GI

Question 0

What is the clinical presentation of esophageal atresia with tracheoesophageal fistula?

Answer 0

Aspiration, regurgitation and respiratory distress with initial feeds.

Question 1

What is the most common variant of esophageal atresia?

Answer 1

A proximal segment of the esophagus that is blinded and a fistula joining the trachea with the distal end of the esophagus.

Question 2

What karyotypic anomalies are frequently associated with esophageal atresia?

Answer 2

Trisomy 13, 18, 21.

Question 3

What is the clinical presentation of duodenal stenosis?

Answer 3

Vomiting at birth; bilious vomiting if the stenosis is distal to the ampulla of Vater.

Question 4

What is the typical cause of jejuno-ileal atresia?

Answer 4

Intrauterine vascular accidents or other vascular insults (volvulus, hernias, necrotizing enterocolitis).

Question 5

What is the clinical presentation of jejuno-ileal atresia?

Answer 5

Proximal: vomiting
Distal: abdominal distension

Question 6

How are anorectal atresias classified?

Answer 6

(1) low: below the levator sling and associated perineal fistulae
(2) high: above the levator sling and associated with fistulae to the genito-urinary tract

Question 7

What are the consequences of short bowel syndrome?

Answer 7

(1) decreased fluid and electrolyte reabsorption
(2) nutrient and salt deficiency
(3) chronic diarrhea

Question 8

How is short bowel syndrome treated?

Answer 8

(1) total parenteral nutrition

(2) bowel transplant

Question 9

On what side of the bowel are GI duplications typically found? GI diverticula?

Answer 9

Duplications: mesenteric
Diverticula: anti-mesenteric

Question 10

From what are diverticula derived?

Answer 10

Remnants of the vitelline duct.

Question 11

Describe the formation of neurenteric remnants or cysts.

Answer 11

(1) They originate from the dorsal midline of the GI tract.

(2) They attach or pass through vertebrae and spinal cord, typically at the cervical or lumbar level.

Question 12

Besides GI obstruction, what are frequent symptoms of neurenteric remnants or cysts?

Answer 12

(1) respiratory distress
(2) hypertrichosis or hyperpigmentation on dorsal cutaneous area
(3) paralysis
(4) infectious or chemical meningitis

Question 13

What are the 4 main classes of congenital GI structural anomalies?

Answer 13

(1) atresia and stenosis
(2) abdominal wall defects
(3) duplications and diverticula
(4) malrotation

Question 14

What are the 2 general classes of primary pseudoobstructions?

Answer 14

(1) enteric neuropathy

(2) visceral myopathy

Question 15

What is the primary function of ganglion cells in the enteric nervous system?

Answer 15

Controlling relaxation of intestinal smooth muscle.

Question 16

What are the 2 classes of plexuses in the GI tract?

Answer 16

(1) submucosal or Meissner’s plexus

(2) myenteric or Auerbach’s plexus

Question 17

What is the typical clinical presentation of Hirschsprung’s disease?

Answer 17

Failure to pass meconium and/or abdominal distension.

Question 18

What is a more technical name for Hirschsprung’s disease?

Answer 18

Aganglionosis.

Question 19

What is the most common GI emergency in newborns?

Answer 19

Necrotizing enterocolitis.

Question 20

What are the 4 most important contributing factors for necrotizing enterocolitis?

Answer 20

(1) intestinal ischemia
(2) intestinal immaturity
(3) bacterial colonization of gut
(4) enteral feeding

Question 21

What is the typical clinical presentation for necrotizing enterocolitis?

Answer 21

Abdominal distension, bloody stools and apnea in the first 2 weeks of life. It may affect any segment of the bowel, but the distal ileum, cecum and ascending colon are most frequent.

Question 22

J: This refers to the presence of gas in the wall of the bowel.

Answer 22

What is pneumotosis?

Question 23

What is a notable bad outcome of necrotizing enterocolitis?

Answer 23

Fibrous strictures or atresia that require resection of the bowel, resulting in short bowel syndrome.

Question 24

What is the most common cause of intestinal obstruction in childhood?

Answer 24

Intussusception.

Question 25

What is intussusception?

Answer 25

The invagination of one intestinal segment into another.

Question 26

What is the primary concern in the event of intussusception?

Answer 26

Ischemia of the intussuscepted segment due to compression of the mesentery.

Question 27

How can cystic fibrosis affect the intestines?

Answer 27

(1) meconium ileus
(2) distal intestinal obstruction syndrome
(3) rectal prolapse

Question 28

How can cystic fibrosis affect the pancreas?

Answer 28

(1) obstruction of ducts by viscid secretions
(2) acinar destruction, fibrosis, diffuse fatty replacement
(3) loss of exocrine function

Question 29

How can cystic fibrosis affect the liver?

Answer 29

Chronic hepatic disease manifested by focal biliary cirrhosis or multilobular cirrhosis.