SYNDROMES
CLIEDO – CRANIAL DYSPLASIA
- No clavicle, so patient can bring his shoulder to midline
- Multiple impacted supernumerary teeth**
- Permanent tooth roots are mostly short
- Wormian bone present
- Small cranium
- Rare genetic disorder
- Delayed closure of fontanelles and open skull sutures and plugging forehead
- Sometimes many Dentigerous cysts
- the patient looks smaller than his relatives and shorter than his relatives
GARDNER’S SYNDROME
- Multiple osteoma
- Dermoid** tumor
- Multiple intestinal polyps
- Multiple impacted supernumerary teeth**
CROUZEN SYNDROME
- Cranio – facial dysostosis/ synostosis
- Exophthalmos + strabysmus (crossed eye – kon kannu)
- Parrot beak nose
- Class III retrognathic maxilla**
- Mentally retarded
TREACHER COLLINS SYNDROME
- Hypoplasia of the facial, especially malar- deformity in zygoma (cheek) & mandibular bones
- Mandible involved – class II retrognathic mandible open bite
- Supernumerary tooth
- Microstomia & oral fistulas Cleft palate
- Eye drop down, cleft eyelid
- External ear deformity with hearing loss
- But he is mentally normal - No retardation
VAN DE WAND SYNDROME : Same as treacher Collins but
- Genetic disorder
- Lip pits – invaginations at commissures or near midline
- Cleft lip with or without cleft palate
- Hypodontia or total anodontia.
- Normal intelligence
DOWN SYNDROME
- Trisomy 21
- Class III retrognathic maxilla
- Hypercementosis
- No caries Rx required ie preventive also
- Cause high salivation
- But because of “, perio problems are high and thus periodontal Rx is necessary
- Infective endocarditis prophylaxis required
- Low carious index , fluoride application is by varnish , inability to learn ,
- Some have cerebral palsy.
- QN - 10 yrs. old child, who is unable to differentiate the colors, and can’t tell his name or address. He is acting like: 3 years old.
RAMSAY - HUNT SYNDROME
- Facial nerve palsy + ulcer of pinna
- Ulcer in oral cavity that doesn’t cross midline
- Virus involved – varicella zoster
MELKERSEN – ROSANTHAL SYNDROME
- Facial nerve palsy + fissured tongue + chelitis granulomatosum
C/F –
- lip deviates to opp/ unaffected side
- Wide opening of eye on normal side
ECTODERMAL DYSPLASIA***
- Patient old looking.
- With hair loss or faint hair
- Having missing teeth - anodontia
- Patient came to clinic with wrinkled skin and white shiny hair, with pegged laterals teeth!!
Gorlin-Goletz ( multiple basal cell nevi syndrome Nevoid BCC syndrome)
- Multiple OKC of jaws - BCC of skin - epidermoid cysts (milia) of skin
- Bifid rib - Calcification of the falx cerebi
- Palmer& planter dyskeratosis
- Frontal bossing - Hypertelorism
- Nevi on neck and scalp
MCune Albright :
- Multiple nevi on neck and head ,
- Multiple bone deformities
- Polyostotic fibrous dysplasia of bone,
- Café-au-lait spots on the skin,
- Endocrine disturbances, e.g. Precocious puberty.
PAPPILON LE FEVRE SYNDROME :
- Hand and foot – Palmar keratosis
- Periodontitis affecting both dentitions
- early teeth loss
- generalized bone destruction
Reiters ( or reiter arthritis or reactive arthritis )
- TRIAD ; conjunctivitis + arthritis + urethritis
- TMJ inflammation.
- Addison disease : have oral melanosis
OSTEOGENESIS IMPERFECTA :
- Blue sclera , teeth wear and multiple fractures- Brittle bone
KLIENFELTER SYNDROME
- 46 + X condition
- Taurodontism
- XXY
CHRONIC MYELOID LEUKEMIA
- Philadelphia chromosome
- 9 chrom 🡪 22 chromosome
- Maroteux Lami syndrome
- RADIO – OPAQUE CYST
- Rx – marsupialization
