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Summary Guest lecture AS (09-03-18)

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Guest lecture AS (09-03-18)

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  • August 14, 2019
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  • 2017/2018
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Guest lecture 09-03-18 – dag 2 (Malformations of orofacial structures and head)
Annelieke Schepens

*Dag 1 gemist!  terugkijken!

Normal development of orofacial structures:

Nasal placode  development of nose and smell.
1-4 in picture are pharyngeal arches  kieuwbogen?
They are numbered: 1,2,3,4 + 6.
The 5th is not present in humans.

There are several prominences:
Nasal pit  for closure of nasal placode
Maxilarry (upper jaw) + mandibular (lower jaw) prominence  surround the …..
Philtrum (‘snotgootje’) from medial nasal processes
….  from lateral masal processes

In the beginning, oral + nasal cavity are one joined cavity, but eventually they will
split.

Orofacial swellings: closure of the open connection between nasal and oral
cavities.
1. Intermaxillary segment; primairy palatum
2. Formation of secundairy palatum  inscicior teeth?
3. Formation of bone etc. in soft tissues  ‘premaxilla’.

The tongue is in between the palatine shells, but in order for them to close, the
tongue has to move down?

Schisis: fusion defects.
Results in opening between structures,
e.g. harelip = hazelip


*Babies born with schisis have problems with drinking.

Holoprosencephaly: spectrum of mid-facial malformations 
defects of the frontonasal prominence  nasal placodes, etc.

Most frequent malformation of the forebrain.
1 ventricle instead of 2 lateral ventricles.
Mostly defects of the olfactory nerves, etc. (midline structure defects).

Most defects are due to defects in cells, receiving the SHH-signal (Sonic Hedgehog).

Holoprosencephaly:
Midline structures are missing + lateral parts are connected. (cake splitting in the
middle example)
Cyclopia: only one eye is formed.

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