4.6

Question 1

Hirschsprung disease is also called

Answer 1

congenital ganglionic megacolon

Question 2

megacolon in Hirschsprung disease is due to

Answer 2

absence of ganglion cells in the mucosal and muscular layers of the colon –> functional obstruction (failure of colonic muscles to relax)

Question 3

most common Hirschsprung disease

Answer 3

rectum and sigmoid colon involvement

Question 4

risk factors for Hirschsprung disease

Answer 4

most common in males (4:1)

Question 5

pathophys of Hirschsprung disease

Answer 5

failure of neural crest cells to migrate completely –> absence of enteric ganglion cells

Question 6

what plexuses are affected in Hirschsprung disease

Answer 6

Auerbach and meissner

Question 7

clinical manifestations of Hirschsprung disease

Answer 7

large bowel obstruction –> failure of meconium passage in first 48 H
Bilious vomiting
Progressive abdominal distention
Poor weight gain
FTT

Question 8

Hirschsprung disease may also present with

Answer 8

enterocolitis –> sepsis like symptoms

Question 9

PE in Hirschsprung disease

Answer 9

no stool in rectal vault : tight anal sphincter and the anal canal and rectum are devoid of fecal material
abdominal distention

Question 10

what disease is associated with squirt/blast sign

Answer 10

Hirschsprung disease

Question 11

what is the squirt/blast sign

Answer 11

explosive gush of flatus and liquid stool as the finger is withdrawn during rectal exam –> may relieve obstruction temporarily

Question 12

what is the best initial test for Hirschsprung disease

Answer 12

contrast enema –> funnel shaped transition zone between normal and aganglionic bowel

Question 13

when should you avoid contrast enema for Hirschsprung disease

Answer 13

if enterocolitis suspected

Question 14

definitive diagnosis of Hirschsprung disease

Answer 14

rectal suction biopsy –> absence of ganglion cells in mucosal and submucosal layers of involved bowel

Question 15

management for Hirschsprung disease

Answer 15

surgical resection of affected bowel

Question 16

rectus abdominis muscles separate

Answer 16

diastasis recti

Question 17

complete absence of closure of portion of the esophagus

Answer 17

esophageal atresia

Question 18

esophageal atresia most commonly presents with

Answer 18

tracheoesophageal fistula
polyhydramnios

Question 19

clinical manifestations of esophageal atresia

Answer 19

presents immediately after birth
excessive oral secretions
choking
drooling
inability to feed
respiratory distress
coughing

Question 20

common complication/illness associated with esophageal atresia

Answer 20

aspiration pneumonia

Question 21

diagnosis of esophageal atresia

Answer 21

inability to pass a nasogastric tube further than 10-15 cm
water-soluble contrast may reveal it in fluoroscopy

Question 22

what type of contrast should be avoided in esophageal atresia and why?

Answer 22

barium; risk of aspiration pneumonia

Question 23

management of esophageal atresia

Answer 23

surgical ligation with primary anastomosis of esophageal segments

Question 24

complete absence or closure of a portion of the duodenum leading to gastric outlet obstruction

Answer 24

duodenal atresia

Question 25

risk factors for duodenal atresia

Answer 25

polyhydramnios down syndrome

Question 26

clinical manifestation of duodenal atresia

Answer 26

presents shortly after birth (24-48 h) bilious emesis abdominal distention

Question 27

what will you see on abdominal radiographs for duodenal atresia

Answer 27

double bubble sign

Question 28

what is used preoperatively for duodenal atresia

Answer 28

upper GI series

Question 29

definitive management of duodenal atresia

Answer 29

duodenoduodenostomy

Question 30

twisting of any part of the bowel about its mesenteric attachment site and axis of its blood supply

Answer 30

volvulus

Question 31

what is involved in up to 90% of cases of volvulus

Answer 31

sigmoid colon

Question 32

in what type of patients does volvulus commonly present

Answer 32

patients who are less mobile, bed bound, and institutionalized usually with a background of chronic constipation

Question 33

clinical manifestations of neonates with volvulus

Answer 33

bilious vomiting within the first week of life colicky pain

Question 34

what imaging will you get first for volvulus

Answer 34

abdominal X-ray with NG tube --> bent inner tube or coffee bean sign

Question 35

what is the gold standard for diagnosing midgut volvulus

Answer 35

upper GI series with barium enema

Question 36

treatment for midgut volvulus

Answer 36

emergency surgery --> counterclockwise unwinding Ladd procedure possible appendectomy

Question 37

telescoping of an intestinal segment into the adjoining distal intestinal lumen, leading to bowel obstruction

Answer 37

intussusception

Question 38

epidemiology of intussusception

Answer 38

most common cause of bowel obstruction in children 6 most - 4 years of age

Question 39

when is intussusception commonly seen

Answer 39

after viral infection

Question 40

classic triad of intussusception

Answer 40

vomiting - may be bilious abdominal pain passage of blood per rectum (currant jelly stools)

Question 41

PE for intussusception

Answer 41

sausage shaped mass in the mid abdomen or RUQ + emptiness in RLQ (Dance's sign)

Question 42

emptiness in RLQ in intussusception

Answer 42

Dance's sign

Question 43

first line imaging for intussusception

Answer 43

US -- will show donut, target, or bull's eye sign

Question 44

What type of enema is preferred for intussusception

Answer 44

air > barium both diagnostic and therapeutic

Question 45

treatment for intussusception

Answer 45

air enema is curative observation for 48 hours after

Question 46

when should you consider surgical intervention for intussusception

Answer 46

failed reduction lead point presentation