Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
of the head
& neck
Produced by:
Dr. Mohamed Fawzy
Albright syndrome:
- Polyostotic fibrous dysplasia
(multiple bones affected)
- Patchy skin pigmentation (caf au lait)
- Endocrine abnormality
- Facial asymmetry in 25% of cases
Apert syndrome:
- Is rare develpmental deformity
- Craniosynostosis (premature fusion of sutures)
- Syndactyly (fusion of fingers or toes)
- Severe Mid-face retrusion
lead to exophthalmus
Behcet syndrome:
- Oral, genital ulcers & uvietis
- young males
- associated with HLA-B5
- treated with thalidomide
Binder syndrome:
- Maxillo-nasal dysplasia
- Severe Mid-face retrusion
- Absent or hypoplastic frontal sinuses
Crouzon syndrome:
- the commonest of craniosynostoses
- autosomal dominant
- premature closure of cranial sutures
- increase intracranial pressure : cerebral damage
- midface hypoplasia, shallow orbit, proptosis of eyes
- Radiographically: characteristic beater copper skull
Eagle syndrome:
- dysphagia & pain in chewing and turning the head
- elongated styloid process
Ehlers-Danlos syndrome:
- abnormality of collagen
- increased bleeding & bruising
(bleeding is common in type IV)
- hyperflexibility of joints,
recurrent TMJ dislocation
- hyperelasticity of skin
- small teeth with short roots - common pulp stones
- early onset periodontal dis. in type VII
Frey syndrome:
- follows trauma to skin overlying salivary gland
- due to crossover symp.& parasymp.
innervation of skin & gland
- may follow parotidectomy
- gustatory sweating & flushing of skin
Gardener syndrome: (autosomal dominant)
- multiple osteomas of jaws & facial bones
- multiple polyps of large intestine (polyposis coli)
which have a tendency to rapid malignant change
- multiple epidermoid cysts
- multiple impacted perm. & supernumerary teeth
Hemifacial microsomia :
- 1 :5000 birth congenital
- lack of hard & soft tissues in the affected side
- usually at ramus & external ear (1st &2ed branchial arches)
Histocytosis-X :
- Histological features of infiltration by macrophages & eosinophils
- 3 broad groups of diseases :
* Solitary eosinophilic granuloma mandible in males <20y
* Hand-Schuller-Christian disease multifocal eosinophilic granuloma
causing skull lesions & affect younger groups
* Letterer-Siwe disease <rapidly progressive, pancytopenia, can be fatal>
Horner Syndrome :
- Consticted pupil miosis
- Drooping eyelid ptosis
- Unilateral loss of sweating anhydrosis on the face
- Caused by interruption of symp.nerve fiber at the
cevical ganglion
Hurler Syndrome :
- Mucopolysaccharides causing growth failure,
mental retardation
- Large head, frontal bossing
- Multiple skeletal abnormalities
- Serum & urinary acid mucopolysaccharides abnormalities
Klippel-Feil anomalad :
- Cervical vertebral fusion
- Short neck
- Low-lying posterior hairline
- Some neurological anomalies
Lesch-Nyhan syndrome :
Defect in purine metabolisim, causing :
- Mental retardation
- Cerebral palsy
- Self mutilating behaviour (particularly lips)
MAGIC Syndrome :
- stands for Mouth And Genital ulcers & Interstitial chondritis
- A varient of Behcets syndrome
Marfan Syndrome :
- Autosomal dominant condition
- Tall-thin stature
- Arachnodactyly long-thin hands
- Aortic regurgitation & floppy mitral valve
- Dislocation of the lens
- High arched palate
- Prelevant among basketball & volleyball players
Orofacial-digital Syndrome :
- One of many CLP syndromes
- Hypodontia especially laterals
- Supernumerary teeth
- Digital finger abnormalities
Papillon-Lefevre Syndrome :
- Palmoplunter Hyperkeratosis
- Juvenile periodontitis (prim.&perm. teeth)
- Hyperkeratosis of palms and soles
- Aggressive gingivitis, periodontitis after teeth eruption only
Patterson-Brown-Kelly Syndrome
Plummer-Vinson Syndrome :
( a form of iron defiecency aneamia)
- Dysphagia (due to atrophy of mucous membrane
of mouth,pharnx, oesophygus)
- Koilonychia spoon nails
- Hyperkeratosis of palms & soles
- Angular chelitis
Peutz-Jeghers Syndrome :
- Autosomal dominant
- Melantic pigmentation of
skin peri-oral & mucosa
- Intestinal polyps (small intestine)
have no tendancy to malignant change
(unlike Gardener syndrome)
- 10% of women are affected
Progeria :
- Collagen abnormality
- Causing Dwarfisim
- Premature aging, death occurs in mid-teens
- Disproportion between small face & beak-like nose
Reiter Syndrome :
- Arthritis
- Urithritis
- Conjunctivitis
- Oral lesions which resemble benign migratory glossitis in appearence
but affect other parts in the mouth
Robin Sequence Pierre Robin :
- structures derived from 1st arch
- Micrognathia (Mandibular hypoplasia)
- Cleft palate
- Eye & ear defects
Stevens-Johnson syndrome :
- Severe version of erythyma multiform
- Mucocutaneous condition which is autoimmune
in nature & preciptated by drugs or viral inf.
- Concentric red rings affects hands and feet
- Multiple mucosal involvement
Stickler syndrome:
- Commonest syndrome with cleft palate (20%)
- Myopia & retinal detachment
- Flat mid face
- Hearing loss (80%)
30% of Robin sequence patients have Stickler synd.
Trotter Syndrome:
- Unilateral deafness
- Pain in mandibular division of trigeminal nerve
- Ipsilateral immobility of palate & trismus due to invasion of the lateral wall
of the nasopharynx by malignant tumor
N.B: pterygopalatine fossa syndrome is similar condition in which 1 st & 2ed
divisions of the trigeminal are affected
Good
luck
M.Fawzy