Developmental defects - Funny Looking Things Flashcards
What causes orofaccial clefts?
Secondary to lack of or improper merging & fusion of developmental process. Environmental and genetic factors.
Lateral palatine process also known as
Secondary palate/hard palate
What happens if secondary palate doesnt zip up?
Bifid uvula
List the various cleft
Cleft lip, cleft palate, Lateral facial cleft, Oblique facial cleft
Median cleft of the upper lip & Median maxillary anterior alveolar cleft
Environmental factors that cause orofacial clefts.
Maternal alcohol & cigarettes, folic acid deficiency, corticosteroid use, Anticonvulsants,
What is a syndrome?
3 or more clinical problems that occur together more frequently than statistically they should occur.
Cleft lip and or palate is?
failure of medial nasal process (primary palate) & maxillary process to fuse
What percent do cleft lip and palate occur together?
45%
30% CP alone
25% CL alone
3 - 8% associated with syndromes.
Complete and incomplete cleft
Complete goes all the way up to the nares. Incomplete does not.
Cleft lip more common in?
Males
Asians higher occurrence and Blacks lower
Cleft palate is?
failure of the palatal shelves to come together and fuse.
Cleft plate is more common in?
Females
ranges from severe to mild
What is minimal cleft palate?
Bifid uvula also mild cleft palate.
Bifid uvula is has high frequency in which race?
Asian and Native American.
Less common in Blacks
Pierre Robin Syndrome
Cleft Palate + Mandibular Micrognathia + Glossoptosis + Airway obstruction
Problems with Pierre Robin Syndrome?
Feeding, speech and malocclusion.
Tx: Surgical repair
Commussural Lip pits
blind invaginations 1-4mm deep located at the commissures. Painless
Commussural Lip pits are common in?
Males . No Tx necessaryy
Paramedian Lip Pits
This are pits on the lip like deep fingernail prints
Van der Woude syndrome
Paramedian Lip pits + cleft lip & or palate
Double lip
Upper lip showing a redundant fold on mucosal side. Drops down & out when smiling.
Congenital & isolated or found with Ascher syndrome
Ascher syndrome
Double lip + Blespharochalasis (double eyelid) + Nontoxic thyroid enlargement
Fordyce granules
Ectopic sebaceous gland on oral mucosa. Yello or yellowish-white papules.
Most common location of fordyce granules
buccal mucosa & vermilion border of lip.
LESS COMMON on retromolar area & tonsillar pillar
Leukoedema
Diffuse, grayish-white milky opalesccent appearance
Common in blacks
Unkown cause. It disappears when the cheek is stretched.
Microglossia
ranges from totally missing(aglossia) to a slightly small tongue.
UNCOMMON
What is associated to microglia
often associated with a syndrome or malocclusion
Macroglossia
large tongue
uncommon causes drooling, difficult speech& eating, open bite, airway obstruction
Reasons for Macroglossia
congenital/hereditary: Lymphangioma, cretinism, Down syndrome, MEN2b, Neurofibromatosis
Acquired: edentulous mandible, amyloidosis, Acromegaly, Myxedema, Acromegaly etc
Apergnathia
open bite caused by macroglossia
Ankyloglossia
“tongue tied.” A short thick lingual frenum causes limited protrusion of the tongue. Causes speech impediments.
More COMMON in MALES
Lingual thyroid
Caused by embryonic failure of the primitive thyroid gland to migrate from the foramen cecum to the normal position in the midline neck
Lingual thyroid most common in
Females
Hairy Tongue
Elongation of the FILIFORM papillae with increased keratin production or decrease desquamation & debris.
Varicosities
Abnormally dilated & tortuous veins. Multiple red-blue-purple nodules.
Caliber-persistent Artery
A main thick walled arterial branch in submucosal tissue.
OLDER adults
Usually on LIPS
Linear & pulsating
Eagle Syndrome (stylohyoid syndrome)
elongation & calcification of the stylohyoid ligament. Pain on swallowing, turning head & mouth wide opening due to impingement of nerves or blood vessels.
Stafne Defect
Lingual cortical concavity filled with normal salivary gland tissue. Lingual Mandibular Salivary Gland Depression
Location of Stafne Defect
Mostly submandibular gland in the posterior Mn below IAN.
RARE: below bicuspids &associated with sublingual gland.
MALE predilection
Bohn’s nodules
palatal cyst on newborn 1-2mm white or yellow-white papules on jxn of hard & soft palate
Epstein’s pearls
palatal cyst on newborn 1-2mm white or yellow-white papules near the midline.
Most COMMON nonodontogenic cyst of the oral cavity
Nasopalatine duct cyst aka incisive canal cyst
Arises from epithelial remnants if the nasopalatine duct.
Heart shaped radiolucency greater than 6mm
Nasopalatine duct cyst
Most cysts are lined by squamous epi but which cysts are lined by pseudostrat columnar epi (respiratory epi)
Nasolabial cyst (NASOALVEOLAR CYST)
Median palatal cyst
RARE swelling in midline of posterior hard palate
Epidermoid cyst common in
young adults, male
Dermoid cyst is
epidermoid ccyst with addition of aadnexal structures (sebaceous glands, hair follicles or sweat glands).
Most common location of dermoid cyst orally
floor of mouth
Painless epithelial remnants of the thryoglossal tract anywhere from foramen cecum to thyroid gland
Thyroglossal ducct ccyst
Four yellow lesions
oral lymphoepithelial cyst
Fordyce granules
Abscess
Synonym for Crouzon Syndrome
Craniofacial Dysostosis
CROUZON SYNDROME (Craniofacial Dysostosis)
sutures in your calvarium and face close prematurely and have tight face, birds beak nose, micrognathia of Mn
Pointed head and syndactyly (fingers connected)
Apert Syndrome: Acrocephalosyndactyly
Mandibulofacial dysostosis
Aka Teacher collins syndrome. Autosomal dominant
Hypophosphatasia
Hereditary decrease in serum alkaline phosphatase. Lack of cementum. DOES NOT affect decidious molars & permanent teeth
Bilateral facial swelling mostly affecting Mandible?
Cherubism
X-ray:Bilateral “soap bubble” multilocular radiolucencies of the premolar-molar and ramus areas of the mandible
Cleidocranial Dysplasia
Multiple unerupted teeth
Lack of clavicles
Short stature and large heads
Hypertelorism and frontal bossing
What syndrome? Osteomas (jaw), colon polyps, multiple odontomas
Gardners Syndrome
Nevoid Basal cell carcinoma syndrome
Hypertelorism
basal cell carcinoma skin
multiple OKC
skeletal abnormalities
Defect in wall of small blood vessels allowing dilation and visible on skin and mucous membrane ?
Hereditary Hemorrhagic Telangiectasia
