Dysplasia 1 Flashcards
3 forms of Fibrous dysplasia
- Monostotic (Mc- 79-80%)
- Polyostotic
- Mccune- albright syndrome (2-3%)
3 characteristics of mccune albright syndrome
- early onset of puberty
- Polyostotic fibrous dysplasia
- Cafe au lat spons (w irregular borders)
Characteristics of polyostotic fibrous dysplasia
- long lesion in long bones
- expansile lucent lesions
- sclerotic rim
- mixed lesion
- shepperds crook
Neurofibromatosis Triad of findings
- Cafe au lait spots (smooth borders)
2 cutaneous tumours - Various osseous alterations of axial and appendicular skeleton
What are the criteria to dx neurofibromatosis
6+ spots
1.5cm diameter
at least 2 neurofibromas
What are potential musculoskeletal manifestations of neurofibromatosis
Kyphoscoliosis (mc) Vertebral scaloping posteriorly enlarged neural foramina Hypoplastic post elements multiple NOF
What is dural ectasia + what is it associated w
widenening of the dura due to post scaloping in neurofibromatosis
What are pressure erosions and what are they associated w
schwannoma/neurilemmoma causing focal enlargement of IVF due to neurofibromatois
What is ‘empty orbit’ and what does it lead to
Bony defect in post orbit causing herniation of the temporal lobe into the defect (pulsating exophthalmos)
Wherre are multiple NOFs mc found
mc in lower extremities
Clinical features of Marfans Syndrome
- elongated extremities/ jt laxity/scoliosis
- sparse soft tissues
- arachnodactyly (spider fingys)
- lens dislocation
- 1/3 congentital heart disease
Marfans syndrome x ray features
- tall vert
- post scaloping
- widened spinal canal
- anyurisms
- scoliosis
What addtional test should you get w someone w marfans
Doppler ultrasound
achondroplasia clinical features
- shortening of long bones (all bones by enchondral ossification)
- hyperlordodic L/S
- Trident Hand (gapping bw 3/4th digit)
Achondrolasia imaging findings (4)
- short broad pedicles
- post scalloping
- increased lordosis
- horizontal sacrum
