Motility Disorders and Hirschsprung Disease Flashcards
MCC of lower intestinal obstruction in neonates
Hirschsprung disease
Hirschsprung disease, gender
Male
Hirschsprung disease is the result of absence of
Ganglion cells in the submucosal and myenteric plexus
T/F Hirschsprung disease is usually sporadic
T
In Hirschsprung disease, the aganglionic segment is limited to the ___ in 80% of patients
Rectosigmoid
Long-segment Hirschsprung disease is defined as
Proximal to the sigmoid colon
Enzyme found in high concentrations between muscular layers and in the submucosa seen in Hirschsprung disease
Acetylcholinesterase
Hirschsprung disease is usually diagnosed in what period
Neonatal
In 99% of healthy full-term infants, meconium is passed within ___
48 hrs of birth
T/F Hirschsprung disease is unusual in preterm infants
T
Hirschsprung disease is seen on barium enema as
Delayed evacuation of contrast (>24 hrs), and transition zone
T/F In Hirschsprung disease, the rectum is usually empty of feces
T
T/F In Hirschprung disease, stools are usually large
F
small pellets, ribbon-like, fluid consistency
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
Gold standard for diagnosing Hirschsprung disease
Rectal suction biopsy
In rectal biopsy for Hirschsprung disease, biopsy material should contain adequate amount of ___ to evaluate for the presence of ganglion cells
Submucosa
Rectal suction biopsy should be obtained no closer than ___ above the dentate line to avoid obtaining biopsies from normal area of hypoganglionosis
2 cm
In anorectal manometry, a balloon is distended in the rectum and the normal response is
Relaxation of the internal sphincter
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
T/F A normal contrast enema rules out Hirschsprung disease
F, normal in 10% of affected patients
Hirschsprung disease where the aganglionic segment is limited to the internal sphincter
Ultra-short segment Hirschsprung disease
Hirschsprung disease where ganglion cells are present on rectal suction biopsy but anorectal manometry is abnormal
Ultra-short segment Hirschsprung disease
Long-segment Hirschsprung disease involves what segments of the bowel
Entire colon and at times part of the small bowel
Treatment of choice for long-segment Hirschsprung disease
Ileal-anal anastomosis, preserving part of the aganglionic colon to facilitate water absorption
The most common cause of acute pseudoobstruction is……
Ogilvie syndrome (acute pseudoobstruction of the colon)
