congenital megacolon
| Noun | 1. | congenital megacolon - congenital condition in which the colon does not have the normal network of nerves; there is little urge to defecate so the feces accumulate and cause megacolon |
congenital megacolon
megacolon
[meg″ah-ko´lon]
con·gen·i·tal meg·a·co·lon
, megacolon congenitumHirschsprung disease
A condition of infant onset characterised by the absence of myenteric nerves in the distal colon, extending proximally from the anus for a variable distance. It affects an estimated 1:5000 live births, and is more common in males (male:female ratio, 4:1); up to 10% have Down syndrome.Clinical findings
Failure to pass meconium or stool in first 24 hours post-partum; abdominal distention; vomiting; constipation at birth. Peristalsis is absent in aganglionic segment, causing proximal pseudo-obstruction and gross dilatation, enterocolitis and perforation. In older children, Hirschsprung disease is characterised by chronic constipation, abdominal distention and stunted growth.
Diagnosis
By rectal suction biopsy; must include submucosa, taken at least 2 cm above pectinate line.
Management
Resection of aganglionic segment, guided by intraoperative frozen sections from myenteric plexus.
congenital megacolon
con·gen·i·tal meg·a·co·lon
, megacolon congenitum (kŏn-jen'i-tăl meg'ă-kō-lŏn, kon-jen'i-tŭm)Synonym(s): Hirschsprung disease.
Hirschsprung,
Harald, Danish physician, 1830-1916.congenital megacolon
- noun
Synonyms for congenital megacolon
noun congenital condition in which the colon does not have the normal network of nerves
Synonyms
- Hirschsprung's disease
Related Words
- congenital disease
- genetic abnormality
- genetic defect
- genetic disease
- genetic disorder
- hereditary condition
- hereditary disease
- inherited disease
- inherited disorder
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