High yield Flashcards
Peutz-Jeghers Syndrome
Melanotic Macules
* hyperpigment macular lesions (Freckles)
* Lips & Mouth
Intestinal polyps
Increased risk of GI Adenocarcinoma
Behcet’s Syndrome
Multisystem vasculitis:
* Oral and genital Aphthous ulcers
* Eye inflammation
Tx: Corticosteroids
Cleft Lip
4-6 weeks in utero
No fusion b/w medial nasal process and maxillary process anteriorly
Usually Left, Unilteral
More common in Males
Tx: Surgically repaired at 3-6 months old
Cleft Palate
Females
6-8 weeks in utero
No fusion b/w palatal shelves
Primary Palate: carries lateral incisor to lateral incisor. Why lateral incisors are missing
Complete CP: No fusion of Both Primary & Secondary palates
Surgical Repair: 6-12 months old
Lip Pits
Invagination at commissures or midline
Commisural Lip Pits: at corner of mouth
Paramedian Lip Pits: Bilateral midline lips
Tx; None
* or Excised for cosmetics
Van der Woude Syndrome
Most common genetic syndrome associated w/Cleft Lip & Palate
Cleft (Lip, palate, or both) + (Paramedial) Lip Pits
Lingual Thyroid
Thyroid TIssue mass at midline base of tongue
- most common location of ectopic thyroid
located along embryonic path of thyroid descent
Tx: None:
* avoid biopsy
Melkerson-Rosenthal Syndrome
=Fissured Tongue + Granulomatous Cheilitis + Facial Paralysis
Think of it as MELS BELLS
* Bells Palsy=another type of facial paralysis w/facial nerve
Rosy Red
* red affecting lips-cheilitis
Sturge-Weber Syndrome
=Angiomas of leptomeninges (Arachnoid & pia Mater) + Skin w/CN V distribution
Neurofibromatosis type I
Aka Von Recklinghausens Disease (Von FRECKLINGhuasen Disease)
=Multiple Neurofibromas
+ Multiple skin freckles (Cafe au lait spots)
+ Axillary Freckles (Crowe’s sign)
+ Iris freckles (Lisch spots/nodules)
neurofibromas can transform to neurofibrosarcomas
Sjogren’s Syndrome
Autoimmune & lymphocyte mediated
* Affects salivary & tear glands
Types:
Primary:
* Keratoconjunctivitis sicca (Dry eyes)
* + Xereostomia
Seconary:
* Primary + another autoimmune disease (Rheumatoid arthritis)
Tx: Symptomatic
Gorlin Syndrome
=Multiple KCOTs (Keratocystic odontogenic tumor)
+ Multiple BCCs (Basal Cell Carcinomas)
+ Calcified Falx Cerebri
+ Fatal Disease
Aka Nevoid Basal Cell Carcinoma
Gardner Syndrome
=Multiple Odontomas
+ Intestinal Polyps
Cherubism
Autosomal Dominant
* Bone replaced with cystic tissue during growth
* begins at 3 y.o.
* after puberty=Reverse–>Bone fill cystic tissue
Cheeks Enlarge
Symmetrical Bilateral Swelling
Radiographically:
* expansile bilateral multilocular RL on ramus
* (histologically identical to CGCG)
Stops growing after puberty
Hereditary Hemorrhagic Telangiectasia (HHT)
AkA Olser-Weber-Rendu Syndrome
Abnormal Capillary Formation on skin, mucosa, and viscera
associated with:
* iron deficiency anemia
* Espistaxis (Nose bleeds)=frequent sign
Telangiectasia=red macule or papule from diluted or broken capillaries
Cleidocranial Dysplasia
Autosomal Dominant
* Affects differentiation of osteoblasts
Common Sign:
* Missing/poorly developed clavicles–shoulders appear hunched in towards midline
* Supernumery teeth
* prominent skull with wormian bones
Tx: Alot of Extractoins & Dentures
Ectodermal Dysplasia
X-Linked Recessive
Common Signs:
* Missing Teeth
* Multiple sharp pointed teeth
* Hypoplastic hair or nails
Amelogenesis Imperfecta
Intrinsic alteration of the ENAMEL
* Normal Dentin and Pulp
Teeth can be:
* discolored (yellow/brown)
* pitted
* thin
Type 1: Hypoplastic
*. Affects quantity of enamel
Type 2: Hypomaturation
* affects quality and quantity of enamel
Type 3: Hypocalcified
* affects quality of enamel
Tx: Full coverage crowns for cosmetics
Dentinogenesis Imperfecta
Autosomal Dominant
Intrinsic alteration of DENTIN in primary & permanent teeth
* SHOrt roots, bell shaped crowns, & obliterated pulps
* Bulbous crowns in radiographs (Bc constricted DEJ)
* Blue sclera
Tx: Full Coverage crowns
Regional Odontodysplasia
Quadrant of teeth exhibiting:
* short roots
* open apices
* enlarged pulp chambers
Radiographic:
* Ghost teeth: pulps are so huge they make the teeth look almost completely RL
Tx: Ext affected teeth
Dentin Dysplasia
Intrinsic alteration of dentin in permanent & primary teeth
Autosomal Dominant
2 types:
* Type 1: Chevron Pulp w/short roots
* Type 2: Thistle shaped Pulp w/normal roots; Primary teeth=blue or amber color
Tx: Not good candidates for restorations
Dentin Dysplasia
Autosomal dominant
* abnormal dental and pulp morphology
Type 1(radicular): Chevron pulp, minimal root development
Type 2: (Coronal): Thistle tube shaped pulps, normal root
* primary teeth= bluee/amber discoloration
Tx: Endo to keep primary teeth
Pierre-Robin Sequence
Micrognathia: Small mandible
Glossoptosis (Tongue displaced posteriorly)
Difficulty breathing & Feeding (Airway Obsttruction)
DiGeorge Syndrome
3rd and 4th pharyngeal pouches
CATCH22
Congenital Heart Defects (Tetralogy of Fallot)
Abnormal facies (Small mandible, hypertelorism-eyes far apart, Short philtrum, long face, small teth, broad nose)
Thymic hypoplasia
Cleft Palate
Hypoparathyroidism
22 Deletion (Chromosome 22)
Crouzon Syndrome
Craniosynostosis (Early closure of skull sutures)
Brachycephaly (Short skull)
Midface Deficiency
Frontal Bossing (prominent forehead)
Hypertelorism (eyes wide apart)
Proptosis/exophthalmos (Bulging Eyes)
McCune Albright Syndrome
Polystotic (more than 1 bone) Fibrous Dysplasia (Ground glass appearance)
Cutaneous cafe-au-lait spots
Endocrine abnormalitie=early onset of puberty
Hemifacial Microsomia
Poor Vascularizatino of 1st & 2nd pharngeal arch
Deficient Ear & Mandibular ramus on affedted side
Turner Syndrome
Females
XO Karyotype (1 X chromosome missing)
Bicsupid aortic valve
webbed neck
Sickle Cell Anemia
Autosomal Recessive
* Moon Shaped RBC
* Blaack americans
Hair on skull=chronic hemolysis
Interproximal Bone:: Step laddeer pattern
Sturge Weber Syndrome
Vascular malformation along Trigeminal Nerve
Red/purple lesions on one side of face=Port wine stains
Lymphangiomas
Periodontal Classification: Stage
Periodontal Classification: Stage
* CAL or RBL
* %RBL/Age
* Case Phenotype
* Smoking
* Diabetes
* CRP
