Hirschsprungs Disease

Which category of Hirschsprung's disease has the highest risk of enterocolitis?
1. Long segment disease
2. Rectosigmoid disease
3. Ultrashort segment disease
Hirschsprung's disease is the result of arrested fetal development of the ___ nervous system?
1. Sympathetic
2. Parasympathetic
3. Myenteric
4. Pre-synaptic
Neuroblasts derived from neural cell precursors are able to be found in the foregut by what gestational week?
1. 3rd week
2. 5th week
3. 8th week
4. 10th week
Neuroblasts migrate from cranial to caudal via what nerve fibers to complete the myenteric nervous system?
1. Sympathetic
2. Vagal
3. Phrenic
Normal ganglion cells are recognized in the esophagus at ___?
1. 2 weeks
2. 4 weeks
3. 6 weeks
4. 8 weeks
What happens once the neuroblasts migrate to their destination?
1. Transdifferentiation
2. Intramural dispersion
3. Dedifferentiation
T or F: maturation of neuroblasts into ganglion cells normally occurs well into infancy?
1. True
2. False
What percent of patients with Hirschsprung's disease have small bowel involvement?
1. 1%
2. 5%
3. 7 - 10%
4. 10 - 15%
Pathologically, in what layer of the bowel are ganglion cells missing with Hirschsprung's disease?
1. Intramuscular plexus
2. Internal sphincter
3. Submucosal plexus
4. All of the above
Which is true regarding the large, thickened, nonmyelinated nerve fibers in muscularis mucosa, lamina propria, submucosa, and Auerbach's plexus of HD patients.
1. Some are postganglionic from normal proximal ganglion cells
2. Some are preganglionic parasympathetic fibers
3. They are usually cholinergic
4. All of the above
What happens to adrenergic nerve fibers in aganglionic segments of bowel?
1. Increased
2. Decreased
3. Normal
What are the nonmyelinated neurons like in the transition zone of HD?
1. Diminished in number progressively
2. Dysplastic nuclei are present
3. Thickened
Which is true regarding neuronal intestinal dysplasia?
1. Absent ganglion cells
2. Dysplasia of the myenteric nervous system
3. Parasympathetic nerve hyperplasia
True or false: the cholinergic system helps to assist in propulsion through the intestine?
1. True
2. False
What reflex is missing in patients with Hirschsprung's disease?
1. Failure of internal anal sphincter to relax
2. Hyperactive internal anal sphincter
3. Persistently elevated pressure of the external anal sphincter
4. High amplitude pressure waves in internal sphincter
What is the incidence of Hirschsprung's disease?
1. 1 in 10,000
2. 1 in 4,000 - 5,000
3. 1 in 1,000
What percent of patients with Hirschsprung's disease have other congenital anomalies?
1. 1%
2. 5%
3. 10%
4. 15 - 25%
If a family has a child with Hirschsprung's disease, what is the incidence of the disease in other children?
1. Less than 5%
2. 5 - 10%
3. 15 - 25%
4. 50%
What is the incidence of chromosomal abnormalities in siblings of the patient with long segment Hirschsprung's?
1. Less than 0.5%
2. 2%
3. 5%
4. 10%
What disease is NOT associated with Hirschsprung's disease?
1. Trisomy 18
2. Smith-Lemli-Opitz syndrome
3. Down's syndrome
4. Waardenberg's syndrome
5. Ondine's curse
What percentage of patients with Hirschsprung's disease are identified in the neonatal period?
1. 25%
2. Less than 50%
3. 95%
What is the incidence of HD in children with meconium plug syndrome?
1. 50 - 75%
2. 25%
3. 10%
4. Less than 5%
What is the general accuracy rate of a barium enema for Hirschsprung's disease?
1. 90%
2. 70 - 80%
3. 50%
4. 25%
Does suction rectal biopsy or a full thickness rectal biopsy interfere with a subsequent pull through procedure?
1. Yes
2. No
What is the accuracy of suction rectal biopsy with adequate tissue?
1. 98 - 99%
2. 85 - 90%
3. 75%
The content of acetylcholinesterase is _____ in the nerve fibers of the lamina propria and muscularis mucosa in Hirschsprung's disease?
1. Increased
2. Decreased
3. Normal
T or F: ACH activity is normally absent or low proximal to the splenic flexure.
1. True
2. False
T or F: In total colonic HD, there are no ganglion cells in the appendix; and all sigmoid level HD has appendiceal ganglion cells?
1. True
2. False
In SwensonÂ’s 25 year series, what was the incidence of requiring a permanent colostomy?
1. 10%
2. 5%
3. 1 - 2%
4. Less than 0.5%
T or F: The Duhamel procedure is a good salvage operation for a failed Swenson or failed Soave?
1. True
2. False

Hirschsprungs Disease

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