Developmental Defects Flashcards
When does central face development begin?
At week 4 of development
Upper lip formation at week 6-7
The merger of the medial nasal process forms what?
Primary Palate
The merger of the maxillary processes forms what?
Secondary palate
A defective fusion of the medial nasal process with the maxillary process results in what?
Cleft lip
*80% are unilateral. Treat with the rule of 10 (10 lbs, 10 weeks, 10 gm %HM)
Failure of the palatal shelves to fuse results in what?
Cleft palate
What is the minimal manifestation of cleft palate?
Bifid uvula
______% of Cleft palate are CL and CP, _____% are only CP and ____% are CL only
45
30
25
What three things make up Pierre Robin sequence?
1-Cleft Palate
2-Mandibular Micrognathia
3-Glossoptosis (downward displacement)
Lack of fusion of the maxillary and mandibular processes results in what?
Lateral facial cleft
Failure of fusion of the lateral nasal process with the maxillary process results in what?
Oblique facial cleft
*upper lip to the eye, almost always with CP
Failure of fusion of the medial nasal process results in?
Median cleft of upper lip
What is the prevalence of orofacial clefts across races?
Native americans 1:250
Asians 1:300
Caucasian 1:700
African Americans 1:1500
When the surface is intact but there is a defect in the underlying musculature of the soft palate, what is it called?
Submucous palatal cleft
*appears as a bluish midline discoloration
Mucosal invaginations that occur at the corners of the mouth on the vermillion border that are not associated with clefts are called what?
Commissural Lip pits
Congenital invaginations of the lower lip, usually bilaterally located are called what?
Paramedian lip pits
What are 4 things to know about Van der Woude syndrome?
1-Greatest significance of paramedic lip pits
2-Autosomal dominant
3-CL + CP
4-Most common form of syndromic clefting
A redundant fold of tissue on mucosal side of lip is typical of ascher syndrome and called what?
Double Lip
What are the 3 characteristics of Ascher syndrome?
1-Double lip
2-Blepharochalasis (eyelid edema)
3-Nontoxic Thyroid enlargement
Ectopic sebaceous glands found in 80 % of the population appearing as yellow papular lesions on the buccal mucosa are called what?
Fordyce Granules
*More common in adults
Diffuse, gray-white, milky opalescent lesions found bilaterally on buccal mucosa that do not rub off and disappear when the cheek is stretched are is called what?
Leukoedema
Abnormally small tongue is usually syndromic and called what?
Microglossia
*often associated with limb features such as hypodactylia and hypomelia
Abnormally large tongue is called what and is most frequently cause by what 3 things?
Macroglossia
1-Vascular malformations
2-Muscular hypertrophy
3-Other (down syndrome, tumors etc.)
What feature is characteristic of Beckwith-Wiedmann syndrome?
Macroglossia