Exam 2 Flashcards
Central face development begins at week ___
4
What happens in week 4 of orofacial development ?
Nasal placodes develop
Proliferation of ectomesenchyme on both sides of each placode results in medial and lateral nasal processes
The upper lip forms at week ___ - ___
6-7
The formation of the upper lip involves the primary palate and secondary palate. What fuses in each?
Primary palate - merging of the medial nasal process
Secondary palate - merging of the maxillary processes of the first pharyngeal arch
The secondary palate makes up ___% of the hard and soft palate
90
Developmental conditions can be ____, which means anomaly of development or hereditary/genetic. The two types of genetic conditions are ___ which means runs in families, or ___ which means present at birth
Developmental
Familial
Congenital
True or false… the palatal shelves are part of the maxillary processes
True
Cleft lip is due to defective fusion of the ___ process with the ____ process
Medial nasal
Maxillary
True or false… most cases of cleft lip are bilateral
False, 80% are unilateral
What conditions should be met in order to fix a cleft lip?
The infant should be 10 weeks, 10 lbs, and 10gm%/HM
Cleft palate failure of fusion of…
The palatal shelves
True or false… cleft lip is usually due to developmental issues, not genetic
True
The minimal manifestation of cleft palate is…
Bifid uvula
At what age do you treat a patient with cleft palate?
1.5 years
____ % of cases are CL+CP
____% of cases are only CP
____% of cases are only CL
45
30
25
____ % of syndromic patients have CL+CP
30%
____% of syndromic patients have cleft palate only
50
Name three symptoms of pierre robin sequence
Cleft palate
Mandibular micrognathia
Glossoptosis (downward displacement of tongue)
Note that pierre robin sequence is mostly developmental, not really genetic in nature
Name four non syndromic environmental factors that can lead to clefting
Maternal alcohol/cigarette use
Folic acid deficiency**
Corticosteroid use
Anticonvulsant therapy
Describe the lateral facial cleft
Lack of fusion of the maxillary and mandibular processes. Results in a hole in the cheek
Occurs in less than .5% of all facial clefts
Describe the oblique facial cleft.
Failure of fusion of the lateral nasal process with the maxillary process.
Can stretch from upper lip to eye
Patients with oblique facial clefts are usually associated with cleft palate
Describe the median cleft of the upper lip
Very rare
Failure of fusion of the medial nasal processes
Results in what looks like two noses
True or false… orofacial clefts are one of the most common major congential defects
True
Describe the prevalence of orofacial clefting in the following populations... Native americans Asians Caucasians African americans
Native americans: 1/250
Asians: 1/300
Caucasians: 1/700
African Americans: 1/1500
True or false… cleft palate only is more common in males
False.. it is more common in females
True or false… CP + CL is more common in males
True
Describe what a submucous palatal cleft is
Soft palate is intact due to epithelium, but the underlying musculature is absent.
This results in a bluish midline discoloration (because it is fluid filled.. blue is reflected due to the depth (tyndall effect))
A bone notch will be present on the posterior portion of the hard palate margin
Where are commissural lip pits found? Are the associated with facial or palate clefting?
They are mucosal invagination at the corners of the mouth on the vermilion border
They are not associated with facial or palatal clefting
Which is involved with syndromes, paramedian or commissural lip pits?
Paramedian
What are paramedian lip pits?
Congenital invaginations of the lower lip. Usually bilateral.
There is no treatment for this
Which syndrome has great association with paramedian lip pits?
Van der woude syndrome
What syndrome is the most common form of syndromic clefting? Is it autosomal dominant or recessive?
Van der woude syndrome (2% of all CL + CP)
Dominant
What is the difference between microglossia and aglossia?
Microglossia - abnormally small tongue
Aglossia - entirely missing tongue
True or false.. microglossia is usually syndromic
True
What are some other issues that are often associated with microglossia?
Micrognathia
Lower incisors missing
What is ankyloglossia? How common is it? Is it more common in males or females? What is the treatment?
Tongue tied. Tongue has abnormally short, thick lingual frenum.
Prevalent in up to 4% neonate
4:1 M:F
No treatment required (can be asymptomatic or self-correcting). Frenotomy (cutting frenum) at age 4-5
During week ___ the thyroid bud normally descends from the apex of the _____ of the tongue (forming the ____) into the neck. ___% of ectopic thyroid are found between the base of the tongue and the epiglottis
7
Sulcus terminalis
Foramen cecum
90
What is the stafne defect?
AKA Lingual mandibular salivary gland depression.
Asymptomatic (no treatment required)
Located between the molars and the angle of the mandible
True or false… lingual thyroid is more common in females than in males
True. It is 7 times more common
True or false… most of the time, in a patient with lingual thyroid, the lingual thyroid is their only thyroid tissue
True. In 70% of patients
Lingual thyroid can cause symptoms such as ___, ___, and ____, and these symptoms arise during puberty, adolescence, pregnancy, or menopause
Dysphagia (difficulty swallowing) , dysphonia (hoarse voice), dyspnea (difficulty breathing)
True or false.. 1/3 of patients with lingual thyroid have hyperthyroidism, which results in lingual thyroid enlargement
False… 1/3 have HYPOthyroidism. The thyroid is enlarged to compensate for hypofunction
How is lingual thyroid usually diagnosed?
Thyroid scan with radioactive iodine
Biopsy is avoided to avoid risk of hemorrhage
Describe thyroglossal duct. ____% are diagnosed before age 20.
Develop from the epithelial remnants of the descending thyroid. Will always result in a swelling at or near the MIDLINE. Usually inferior to the hyoid bone.
50
1% of the time, it results in carcinoma
What is hemihyperplasia and what are some associated syndromes?
Rare developmental anomaly
Asymmetric overgrowth of one or more body parts
Beckwith-wiedemann syndrome, neurofibromatosis, proteus syndrome
What is crouzon syndrome (cranial dysostosis)
Characterized by craniosynostosis. (Brachyecephaly - short head. Scaphocephaly - boat shaped head)
Most severe cases demonstrate a cloverleaf skull (kleeblatt-schadel deformity).
Associated with ocular proptosis (underdeveloped maxilla)
Due to a mutation of the fibroblast growth factor receptor 2
What is apert syndrome (acrocephalocyndactyly)
Ocular proptosis Hypertelorism Syndactyly Mental retardation Trapezoid shaped lips
What is tooth agenesis and cleidocranial dysplasia?
Tooth agenesis - failure of teeth to form
Cleidocranial dysplasia - third layer of teeth form
Developmental events are reiterated during ___
Repair
What week in development does tooth morphogenesis start?
5
All organs develop from ___ and ___ layers
Epithelium
Mesenchyme
What is intracrine signaling?
The signaling factor never leaves the cell but is able to affect the cell’s activity within the cell
Name some things that the neural crest cells form
Ganglia Adrenal medulla Dentin Pulp Cementum PDL Bones in face Ectomesenchyme
What is ectomesenchyme?
Embryonic connective tissue
What structure will give rise to the dental lamina?
Primary epithelial band
What is the dental lamina?
Thickening of the oral epithelium in a band around the arches
Where is meckel’s cartilage in relation to the dental lamina?
Directly inferior
How does signaling from the dental lamina initiate tooth development?
The dental lamina signals the underlying mesenchyme to condense. The condensing mesenchyme signals adjacent mesenchyme to suppress tooth development genes. This ensures that tooth generation only occurs where it is supposed to
What causes the thickening of the dental lamina and underlying mesenchyme?
Cell proliferation
What are dental placodes
Specific portions in the dental lamina where the individual tooth actually forms
What signal is the dental lamina producing, and what signal is the underlying mesenchyme producing to begin tooth formation?
Dental lamina - LEF 1
Mesenchyme - MSX 1
What are the six stages in crown development?
Initiation-induction
Bud stage-proliferation
Cap stage-proliferation, differentiation, morphogenesis
Bell stage-proliferation, differentiation, morphogenesis
Apposition stage- induction and proliferation (laying down of dentin and enamel)
Maturation stage - maturation
What forms during the initiation stage of tooth development?
Lamina
Bud
What forms during the morphogenesis stage of tooth development?
Cap
Early bell
In which stage is tooth shape determined?
In the morphological stage which includes cap and early bell
During what structure does cell termination/differentiation occur?
Late bell
The condensed mesenchyme underlying the dental bud is called the _____
Condensed papilla mesenchyme
How many buds form in each arch along the dental lamina?
10 (10 primary teeth)
Each bud is the precursor of the ____ for each deciduous tooth
Enamel organ
During the bug stage, ___ acts along with ___, ____, and ____ to stimulate the mesenchyme to express ___, ____, ____, early growth response 1 (___), and the ECM molecules ____ and ____ and more ____
BMP
FGF-1
LEF-1
Shh
Msx-1
Msx-2
Egr-1
Pax-9
Syndecan
Tenascin
BMP-4
During the initiation stage, ____ stimulates the pre-dental ectoderm to produce ____, which in turn induces the underlying matrix to express ____ and ____ beneath the thickened dental lamina. ____ and ____ inhibit ____
Lef-1
Fgf-8
Pax-9 and Msx-1
BMP 2 and BMP 4 inhibit Fgf-8
What is the dental follicle?
Capsule that encases the developing tooth
The dental papilla is made up of ___ cells
Mesenchymal
During the ___ stage, ____ dental lamina branches off ____ which will form the permanent tooth
Cap
Successional
Lingually
_____ cells condense around the developing cap. These will become the dental papilla and dental ____
Ectomesenchyme
Sac (follicle)
What comprises the tooth germ?
Enamel organ
Dental papilla
Dental sac/follicle
The second primary molars have three permanent buds that run ___ to form ____
Posteriorly
The permanent molars
What is the epithelium called that connects the oral epithelium to the tooth germ?
Outer dental epithelium
Also called enamel epithelium
The ____ is the star shaped cell tissue located in the enamel organ. It is filled with liquid, separating the cells and sequestering growth factors
Stellate reticulum
What are enamel niches?
Cross sectioning artifacts found in the enamel organ
What is the enamel knot and what is its function?
Signaling center located in the enamel organ that determines how many cusps will form.
The influence of shape and number of cusps goes from epithelium to mesenchyme
What signaling factor is strongly expressed in the enamel knot?
Fgf-4