Congenital GI Diseases

Question 1

How does malrotation develop?

Answer 1

improper rotation drawing the intestines back in to the abdominal cavity

Question 2

What is the ligament of Treitz?

Answer 2

fixed at duodenal jejunum and colon, keeps intestines in LUQ

should be locates LEFT of spine

Question 3

What occurs with malrotation?

Answer 3

the narrow mesenteric base permits abnormal mobility of the small bowel, allowing the mesentery to twise–>midgut volvulus (entire midgut can die)
ladd’s bands (cross the duodenum), can lead to obstruction

Question 4

What are the sx of malrotation?

Answer 4

tachycardic, tachypneic, crying/grunting
lungs clear
abdoment tender

Question 5

What is done to correct malrotation?

Answer 5

surgery-Ladd’s procedure

Question 6

What is the key imaging finding of duodenal atresia?

Answer 6

double bubble sign

Question 7

What causes duodenal atresia?

Answer 7

failure of recanalization

Question 8

Besides the double bubble sign, what are signs of duodenal atresia?

Answer 8

polyhydramnios from high intestinal obstruction

Question 9

What are other features of duodenal atresia?

Answer 9

asociated with trisomy 21 (&cardiac, genitourinary & anorectal abnormalities)

Question 10

How is duodenal atresia treated?

Answer 10

NG tube

operative repair

Question 11

What are signs of jejunoileal atresia?

Answer 11

doesn’t pass meconium

bilious emesis

Question 12

What causes jejunoileal atresia?

Answer 12

in utero vascular disruption leading to ischemic necrosis of the fetal intestine
may be linked to inherited thrombophilia & spontaneous thrombosis may play a role in some cases
maternal use of vasoconstrictive medications may contribute to interruption of mesenteric blood flow (pseudophedrine, cocaine, nicotine)
rare familial inheritance

Question 13

How is jejunoileal atresia treated?

Answer 13

surgery

Question 14

What is a potential long term issue of jejunoileal atresia?

Answer 14

short gut syndromes, may need SI transplant

Question 15

What is hirschspurng’s disease?

Answer 15

congenital defect in intestinal innervation (both meissners and auerbachs plexi)
more common in males
associated w/trisomy 21
possible assosciated w/ RET mutation

Question 16

What does is the lack of peristalsis in hirschspurng’s disease called?

Answer 16

fuctional obstruction

Question 17

Where is hirschspurngs most commonly located?

Answer 17

rectosigmoid

Question 18

What are symptoms of hirschspurng’s?

Answer 18

failure to pass meconium
functional obstruction/bilious emesis
enterocolitis

Question 19

What is the tx ofr Hirschspurng’s?

Answer 19

surgical resectin of aganglionated segment, anastomosis of normal colon to recturm

Question 20

How is necrotizing enterocolitis?

Answer 20

abdominal distention
feeding intolerance/emesis
rectal bleeding
abdominal wall erythema
temperature instability/apnea/bradycardia
peumatosis (gas within the bowell wall) on XRay

Question 21

What is protective against necrotizing enterocolitis?

Answer 21

breast milk

probiotics

Question 22

What causes pneumatosis?

Answer 22

gas produced by bacteria which is seen within serosal and muscularis layers of bowel

Question 23

What are some potential causes of necrotizing enterocolitis?

Answer 23

prematuritiy
formula feeding
indomethacin (other medications)

Question 24

How is necrotizing enterocolitis treated?

Answer 24

abx-broad spectrum
-ampicillin (gram +)
-gentamycin of ceftriaxone (gram -)
-metronidazole (anaerobe)
bowel rest (NPO, NG tube)
TPN
Surgery

Question 25

What are indications for surgery for necrotizing enterocolitis?

Answer 25

pneumoperitoneum free air

clinical deterioration

Question 26

What is a common imaging finding of anorectal malformation?

Answer 26

no air in rectom

Question 27

What are anorectal malformations associated with?

Answer 27

trisomy 21

VACTERL complex

Question 28

How do anorectal malformations occur?

Answer 28

abnormality of caudal descent of urorectum

• failure of descent of hindgut to anus
• fusion of urorectal ridges to walls of cloaca

Question 29

Which types of anorectal malformations are more common in males? females?

Answer 29

males-“high” rectovesicular or rectourethral fistula

females-“low” malformations to perineum or vestibule

Question 30

How is anorectal malformation treated?

Answer 30

colostomy with later pullthrough operation