Ch. 332.4 - Congenital Aganglionic Megacolon (Hirschsprung Disease) Flashcards Preview

Nelson - The Digestive System > Ch. 332.4 - Congenital Aganglionic Megacolon (Hirschsprung Disease) > Flashcards

Flashcards in Ch. 332.4 - Congenital Aganglionic Megacolon (Hirschsprung Disease) Deck (24):
1

MCC of lower intestinal obstruction in neonates

Hirschsprung disease

2

Hirschsprung disease, gender

Male

3

Hirschsprung disease is the result of absence of

Ganglion cells in the submucosal and myenteric plexus

4

T/F Hirschsprung disease is usually sporadic

T

5

In Hirschsprung disease, the aganglionic segment is limited to the ___ in 80% of patients

Rectosigmoid

6

Long-segment Hirschsprung disease is defined as

Proximal to the sigmoid colon

7

Enzyme found in high concentrations between muscular layers and in the submucosa seen in Hirschsprung disease

Acetylcholinesterase

8

Hirschsprung disease is usually diagnosed in what period

Neonatal

9

In 99% of healthy full-term infants, meconium is passed within ___

48 hrs of birth

10

T/F Hirschsprung disease is unusual in preterm infants

T

11

Hirschsprung disease is seen on barium enema as

Delayed evacuation of contrast (>24 hrs), and transition zone

12

T/F In Hirschsprung disease, the rectum is usually empty of feces

T

13

T/F In Hirschprung disease, stools are usually large

F, small pellets, ribbon-like, fluid consistency

14

In older infants, the Currarino triad must be considered as a differential diagnosis for Hirschprung disease; the triad consists of

1) Anorectal malformations 2) Sacral bone anomalies 3) Presacral anomaly

15

Gold standard for diagnosing Hirschsprung disease

Rectal suction biopsy

16

In rectal biopsy for Hirschsprung disease, biopsy material should contain adequate amount of ___ to evaluate for the presence of ganglion cells

Submucosa

17

Rectal suction biopsy should be obtained no closer than ___ above the dentate line to avoid obtaining biopsies from normal area of hypoganglionosis

2 cm

18

In anorectal manometry, a balloon is distended in the rectum and the normal response is

Relaxation of the internal sphincter

19

In the absence of classic findings of Hirschsprung disease with the use of contrast enema, it is imperative to compare the rectal diameter with the sigmoid diameter. ___ suggests Hirschprung disease

Rectal diameter ≤ sigmoid diameter

20

T/F A normal contrast enema rules out Hirschsprung disease

F, normal in 10% of affected patients

21

Hirschsprung disease where the aganglionic segment is limited to the internal sphincter

Ultra-short segment Hirschsprung disease

22

Hirschprung disease where ganglion cells are present on rectal suction biopsy but anorectal manometry is abnormal

Ultra-short segment Hirschsprung disease

23

Long-segment Hirschsprung disease involves what segments of the bowel

Entire colon and at times part of the small bowel

24

Treatment of choice for long-segment Hirschsprung disease

Ileal-anal anastomosis, preserving part of the aganglionic colon to facilitate water absorption