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Congenital Malformations

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Congenital Malformations
Tuesday, 21 January 2014

Systemic congenital malformations!
GIT

- aglossia- without mouth!
- lingua plicata- fissured tongue!
- micrognathia- small jaw!
- progenia- premature ageing!
- fordyce spots- raised sebaceous (sweat) glands!
- dens in dente- tooth within tooth ( coronal more common)!
- amelogenesis imperfecta- AR disorder of enamel—>small, discolored, pitted or grooved, and prone to rapid wear and breakage!

- dentinogenesis imperfecta- dentin dysplasia that causes teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent giving teeth an opalescent sheen! - aplasia- congenital absence of … eg salivary glands!
- achalasia- can occur at many places along the GIT, it is the failure of smooth muscle to relax—> sphincter constriction—> regurgitation, pain. also referred to as
CARDIOSPASM!

- stenosis- abnormal narrowing in a blood vessel or other tubular organ or structure. It is also sometimes called a stricture (as in urethral stricture).[3] The term coarctation is a synonym!

- esophageal stricture is a narrowing or tightening of the esophagus that causes swallowing difficulties!

- tracheoesophageal fistula- is an abnormal connection (fistula) between the esophagus and the trachea.!

- omfalocele-is a type of abdominal wall defect in which the intestines, liver, and occasionally other organs remain outside of the abdomen in a sac because of a defect in the development of the muscles of the abdominal wall (exomphalos)!

- atresia- is were body passage or orifice is closed or absent, in this case - Intestines!
- meckels diverticulum- true congenital malformation, (bulge in small intestine wall) remains of yolk sac or omphalomesenteric duct!

1

Tuesday, 21 January 2014

- cogenital megacolon (hirschprung disease) when the COLON has no developed nervous supply—> unable to function!

- hypogangliosis- fewer than normal ganglion cells in the COLON—> megacolon!
- accessory pancreas-

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