(3) Sclerosing Dysplasias Flashcards
What is the inheritance of osteopoikilosis?
autosomal dominant
What are the radiographic characteristics of osteopoikilosis?
“spotted bones”
- multiple small (1-10mm) round/ovoid opacities in epiphysis & metaphysis
- resembles multiple bone islands (not blastic mets)
- symmetric
- long bones, carpals, tarsals
What are the clinical features of osteopoikilosis?
- 1/4 have skin lesions
- usually ASx
What is another name for osteopathia striata?
Voorhoeve disease
What are the radiographic characteristics of osteopathia striata?
variant of osteopoikilosis (stretched dots w/ growth)
- linear (striped) opacities in diaphysis & metaphysis
- parallel to long axis of long bones
- sunburst effect in pelvis
- bilateral
What are the clinical features of osteopathia striata?
ASx
What is the cause of osteopetrosis?
genetic deficiency of osteoclasts results in lack of bone resorption, inhibiting formation of normal medullary canal & bone marrow
Describe the quality of bone with osteopetrosis.
dense & hard (brittle)
What are other names for osteopetrosis?
- Albers-Schonberg disease
- marble bone disease
- chalk bones
What are the clinical features of severe osteopetrosis?
- stillbirth or die within first 2yrs
- anemia & infection
- multiple Fx’s
- blindness & cranial n defects
What are the clinical features of benign osteopetrosis?
- 50% ASx (incidental findings)
- hepatosplenomegaly (extramedullary hematopoiesis)
- facial palsies
- deafness, blindness
- dental caries (osteomyelitis of mandible)
What are the relevant lab findings of osteopetrosis?
- anemia (poor correlation w/ sclerosis)
- thrombocytopenia
- ^ serum Ca2+
What are the imaging features of osteopetrosis in the extremities?
- generalized sclerosis
- no/little trabeculation
- striated bones (“bone within a bone”)
- Erlenmeyer flask deformity (tubular bones)
What are the differentials for Erlenmeyer flask deformity?
- Sickle cell (osteopenia)
- Thalassemia (osteopenia)
- Osteopetrosis (^density)
What is another term for the “bone within a bone” appearance of osteopetrosis?
endobone appearance
How would you modify chiropractic treatment for a diagnosis of osteopetrosis?
absolute contraindication to HVLA
What are the imaging features of osteopetrosis in the spine?
- diffuse sclerosis or striated bones
- sandwich vertebrae (^density)
- spondylolysis
- spinal stenosis (cervical myelopathy)
What is the differential for sandwich vertebrae?
rugger jersey spine (decreased density in HPT)
What are the potential complications of osteopetrosis?
- patho Fx (especially femur)
- hemorrhage
- infection
- blindness
- deafness
- leukemia & sarcoma
- facial palsies
