In: Anatomy and Physiology
Find a digestive disease that you don't know about and write a report describing this disease.
Make sure this report is in your own words.
Please add any personal comments/experiences.
Remember to cite where you got your information.
-Submit the report and post for discussion
HIRSCHSPRUNG'S DISEASE
- Congenital defect in colonic innervation, producing congenital aganglionic megacolon
- more common in males but severe in females
- 1:5000 live births
PATHOGENESIS:
• Failure of migration of neural crest cells into the bowel.
• Leads to absence of ganglion cells particularly nitric oxide releasing cells in bowel.
• Affected part of bowel is not able to relax.
• Proximal part of affected bowel gets dilated.
MORPHOLOGY:
Short Segment Hirschsprung's Disease: when part of large intestine is involved.
Long Segment Hirschsprung's Disease: When entire colon is involved.
MOST COMMON SITE: Rectum
CLINICAL FEATURES:
• Failure of passage of meconium.
• Abdominal Distension
• Constipation
• Dilation causes thinning if bowel wall which may lead to rupture (especially in colon).
DIAGNOSIS:
Confirmed by Rectal Suction Biopsy.
It reveals:
Special stains are used.
• AchE: heavy staining (Normally, no staining)
• Calretinin: no staining (Normally, staining is present)
Reference: Robins Pathological Basis of Disease