Syndromes

Question 1

Pgets Disease

Main process, produces

Involves

Results in

Increases

Age

Onset and course

Clinical feat 2

Answer 1

• Bone broken down and replaced faster than normal
• New is weaker and less organized than normal bone
• Involves multiple areas of skeleton
• Results in enlargement of skull jaws & facial bones & deformity of spine and long bones
• Inc pathologic fracture
• Occurs in pt over 40
• Gradual onset and chronic course
• Clinical feat
  
  • Narrowing of skull foramina leads to cranial nerve dysfunction
  • Enlargement of jaws & widening of alveolar ridges
    
    • Dentures dont fit and spaces btw teeth

Question 2

Pagets Disease Xray feat early and late

2 other xray

symptoms 3

Tx

Inc risk of

Answer 2

• Xray
  
  • Early stages- decreased density of bone
  • Later- patchy poorly defined RO areas Cotton wool
  • Hypercementosis
  • Enlargement of maxilla
• Can cause bone pain, bone deformity, neurologic problems
• Tx
  
  • Calcitonin and biphosphonates
  • Inc incidence of osteosarcoma

Question 3

Hyperparathyroidism

Primary and secondary

Answer 3

• Primary- parathyroid gland hyperplasia, adenoma, carcinoma,–> inc PTH
• Secondary- Kidney disease–> hypocalcemia–> inc PTH

Question 4

Hyperparathyroidism

Clinical features 5

Xray 3

Answer 4

• Hypercalcemia leads to
  
  • Bone & joit pain
  • Renal calculi
  • Peptic ulcers
  • Cognitive impairment
  • Painful bones, renal stones, abdominal groans and psychotic moans
• Xray
  
  • Gen decrease in bone density–> osteoporotic ground glass app due to
    
    • Loss of trabeculation, lamina dura, cortical plates
  • Ocassionally giant cell tumors of bone brown tumors

Question 5

Hyperparathyroidism

Lab feat of primary and secondary

Microscopic 2

Tx for both

Answer 5

• Primary- inc serum calcium and PTH
• Secondary- dec or normal serum calcium, inc PTH
• Microscopic
  
  • Inc bone turnover
  • Giant cell lesions identical to CGCG
• Tx
  
  • Primary- excision or parathyroid glands
  • Secondary- renal dialysis or renal transplant

Question 6

Osteogenesis Imperfecta

Cause and patho 6

Answer 6

• Genetic disease
• Defective coss linking of collagen
• Osteoblasts produce defective osteoid
• Bones are brittle or fragile
• Congenital onset is more severe, fracture in utero, die
• Later onset milder

Question 7

Osteogenesis Imperfecta

Skeletal Feat 1

Xray 2

Clin feat 7

tx

Answer 7

• Multiple fractures
• Xray
  
  • thin cortical plates, excessive callus formation
  • Large skull with prominent frontal bones
• Eyes
  
  • Blue sclera
• Ears
  
  • Hearing loss
• Ligament laxity
• Class 3 malocclusion
• Diffuse blue-brown/translucent teeth
• Cranial and facial feat
• Scoliosis
• No tx or cure

Question 8

Osteopetrosis

Cause

Discovered

Answer 8

• Osteoclasts are unable to resorb bone, inc in bone density and osteomyelitis
• Discovered at birth or early childhood, or later in life
• Frequent fractures

Question 9

Ectodermal Dysplasia

2 types

Answer 9

• 2 major types
  
  • Anhidrotic: partial or complete absence of eocrine sweat glands, sebaceous and hair follicle
  • Facial abnormalities
    
    • Midface hypoplasia and saddle nose

Question 10

Ectodermal Dysplasia

Skin and appendages

Answer 10

• Skin and appendages
  
  • Fine sparse hair
  • Scanty eyelashes and eyebrows
  • Heat intolerance and fever

Question 11

Ectodermal Dysplasia oral manifestations

Answer 11

• Hypodontia or anodontia of primary and perm teeth
• Hypoplasia of alveolar ridges
• Dec vertical dimension
• Protuberant lips
• Cone-shaped teeth
• Unerupted teeth
• Xerostomia

Normal lifespan

Question 12

Cherubism

Answer 12

• Progressive nontender swelling of jaws
• Mand always involved
• Round cherub face
• Xray
  
  • Bilateral RL lesions–> expansion and sometimes perforation
  • Multiocular rl
  • Multi unerupted teeth are displaced by lesion
• Tx
  
  • lesions stabilize at puberty
  • Surgical recontouring

Question 13

Gardner Syndrome

Imp for dentists bc

Assoc with

Clin feat 3

Tx

Answer 13

• Genetic
• 3/4 have dental abnormalities or osteomas
• Asssoc with cancer
• Polyps (adenomas) of colon and will develop carcinoma of colon rectum
• Multiple osteomas of bone, precede intestinal polyps
• Multi unerupted perm teeth and supernumerary
• Tx
  
  • those w/o polyps should be evaluted periodically
  • Once polyps detedted- colectomy

Question 14

Craniofacial Dysostosis

Answer 14

• Synostosis- early closure of sutures
• Midface hypoplasia and exophthalmos
• Beaten metal skull app (brain grows, skull cant)
• Apert syndrome- cranial dystosis and webbing
• Tx
  
  • Craniectomy early to provide space for brain

Question 15

Cleidocranial Dysplasia

Skull

Shoulder

Oral

teeth

tx

Answer 15

• Skull
  
  • Fontanels are open or have late closure
  • Sutures remain open–> Wormian bones
  • Brachycephalic skull
• Clavicle hypoplasia-lack of
• Oral
  
  • High narrow plate
• Teeth
  
  • Prolonged retention, perm may fail or delay
  • Multiple unerupted supernumerary teeth
• Tx
  
  • Orthognathic surgery and orthodontics
  • Life normal

Question 16

Mandibulofacial Dysotosis (treacher collins)

Profile

Clin feat

Answer 16

• Convex facial profile
  
  • down slopping palpebral fissures
  • Hypoplasia of mand and mala bones
• Hypoplastic zygomatic arch
• Underdeveloped ramus/condyle
• Deepeed antegonial notch
• Eyes- coloboma of lower eyelid (fissure)
• Ears- deformed pinna
• Conductive hearing loss
• Oral- high palate, malocclusion

Question 17

Papillon-Lefevre Syndrome

Answer 17

• AKA Juvenille Periodontosis with Palmar-Plantar Hyperkeratosis
  
  • Severe alveolar bone loss for prim and perm teeth
  • Deep pockets, premature loss of teeth