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.
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.
