Wednesday, January 24, 2018

Neural crest (clinical aspect)

Hello Awesomites! :D

Let us go back to the contribution of neural crest cells to many different systems (neural, skin, teeth, head, face, heart, adrenal glands, and gastrointestinal tract).

Neural crest forms neural and non-neural population.
-Cranial neural crest
-cardiac neural crest
-trunk neural crest
-Vagal neural crest

Malformations of NC origin
-Facial clefts, ear malformations, and other Facial defects 
-Branchial fistulae and anomalies of pharyngeal arch derivatives
-Cardiovascular malformations 
-Pigmentary disorders
-Abnormal enteric innervation 
-Hemangiomas and vascular malformations

1. Facial clefts, ear malformations, and other facial defects-
 The shaping of the face is therefore patterned under the influence of the NC.
The more common malformations of the region
-preauricular tags
-cleft lip, and cleft palate
-CHARGE association
-Treacher Collins, or Goldenhar syndrome.

2. Branchial fistulae and anomalies of pharyngeal arch derivatives 
-Malformations of the endocrine glands that are derived from the pharyngeal arches and pouches have the same origin. That is:-
The paired thymus which originate from the third pharyngeal pouch on each side,
The parathyroids, derived from the third (lower glands) and fourth pharyngeal pouches (upper glands) ,
C-cells of the thyroid (derived from the ultimobranchial body in the fifth pouch)
-Branchial cyst

3. (IMPORTANT) Cardiovascular malformations
TA, DORV, Tetralogy of Fallot (TOF), narrow outflow pulmonary tract (NOPT), transposition of the great vessels, perimembranous ventricular septal defect (VSD), and other heart defects are the result of defective NC influence on the region.

4. Pigmentary disorders 
-Neurocutaneous melanoses 
-neurofibromatosis of Von Recklinghausen 
-Tuberous sclerosis and all the gastrointestinal polyposes in which there are mucosal or cutaneous pigmentary spots (Peutz Jeghers, Cowden, Cronkhite- Canada etc.)
-neurofibromatosis albinism

5. Abnormal enteric innervation
-Hirschsprung disease
- Neuronal intestinal dysplasia

6. Tumors
Due to abnormal proliferation of NC cells
-Peripheral neuroectodermal tumor

7. Hemangiomas and vascular malformations 
The concept that hemangiomas and vascular malformations are derived from the NC is relatively new, but accepted. The flat vascular malformations that are located in the areas corresponding to the cutaneous innervation of the cranial nerves may have intracranial extension (Sturge-Weber syndrome) and are of this origin.

In next post, I will discuss NEUROCUTANEOUS SYNDROME as an extension of this post.
Feel connected: D

-Upasana Y. :)

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