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Haematological / Vascular Disorders of Bone (VASCULAR (Osteochondritis…
Haematological / Vascular Disorders of Bone
HAEMATOLOGICAL
SICKLE CELL ANAEMIA
‘abdominal crisis’ from bowel infarctions; bony changes due to marrow hyperplasia, ischaemia, necrosis, infection
SPINAL
widespread
diploic space
granular skull
(spares sinuses)
growth disturbances
visceral changes
(gallstones, bowel infarction, autosplenectomy)
osteomyelitis
(bilateral, in diaphysis)
APPENDICULAR
• Osteonecrosis/infarctions
• Osteoporosis
• Osteosclerosis
•
Erlenmeyer flask deformity
•
undertubulation
of bone
• tibiotalar slant deformity, ankle valgus deformity
Erlenmeyer flask deformity
Causes
Lysosomal storage disease
– Gaucher disease - osteopenia with Legg-Calvé-Perthes disease or Hass disease
– Niemann-Pick disease
Haemoglobinopathies
– thalassaemia - coarsened trabeculation, cobweb appearance
– sickle cell disease
Bone dysplasias
– fibrous dysplasia
– osteopetrosis - diffuse sclerosis and sclerotic vertebral endplates (sandwich vertebrae)
– craniometaphyseal dysplasia
– metaphyseal dysplasia - Pyle disease
– achondroplasia
– Ollier disease
BETA THALASSEMIA MAJOR
honeycomb trabeculae
on hand and toe
filled in sinuses with maxillary hypoplasia
bone marrow hyperplasia
hair-on-end skull
FISH vertebrae
H vertebrae
due to microvascular endplate infarction
Erlenmeyer flask deformity
undertubulation
of bone
osteoporosis
due to marrow expansion
osteosclerosis
due to ischaemia after vaso-occlusive crisis e.g. infarctions
LEUKAEMIA
ACUTE CHILDHOOD LEUKAEMIA
• Osteoporosis
•
lucent metaphyseal bands
• Periostitis
•
lytic lesions
•
Splenomegaly
• chloromas
• myeloblasts
• extramedullary manifestation
ADULT LEUKAEMIA
periostitis
lytic lesions
granulocytic sarcoma
osteoporosis
lucent metaphyseal bands
splenomegaly
HAEMOPHILIA
deficiency of clotting factor
spontaneous
haemorrhage
early DJD from repeated harmarthrosis
overgrowth of epiphysis
suqared patella
wider intercondylar notch
tibiotalar slant
increased
soft tissue density
around joints (hemosiderin)
haemophilic pseudotumour (femur, ilium)
VASCULAR
OSTEOCHONDROSES
3 Aetiologies
Osteonecrosis
Avascular, femoral / tibial condyles
60yo females
complication of HIV, hip dislocation, femoral neck fractures
heavy alcohol use, sickle cell disease, lupus, corticosteroid use.
Legg-Calve-Perthes
male 4-8yo flat & fragmented
femoral head
• capsular distention
•
joint space widening laterally
, widened femoral neck
• undergrowth of epiphysis
• solid or patchy sclerosis
•
lucent crescent sign
• gradual reconstitution of normal bone density and shape
• coxa vara
Freiberg's
2nd-5th metatarsals
flat, irregular, sclerotic head
13-18yo females
Kienbock's
AVN
Lunate
excessive pressure on lunate
collapse, fragmentation and sclerosis
–ve ulnar variance (= unla falls shorter than radius line)
Kohler's
males 5-9yo
flattened
Talus / Navicular
limping from pain and swelling in foot
mimic normal growth variation before ossification
Trauma
Non-avascular
Osgood-Schlatter's
tibial tuberosity
fragmented
• 11-15yo, Boys : girls = 3:1
• bilateral
• running, kicking and jumping
• growth spurts
Blount's
Scheuermann's Dx
13-17yo
irregular endplates T7-12
thoracic kyphosis at least 3 spinal segments
multiple schmorl’s nodes
anterior wedging of VBs
loss of disc heights
thickened contracted ALL
osteophyte formation
Growth Variants
Sever's Disease
calcaneal is sclerotic, fragmented
Bone Infarctions
medullary necrosis, serpiginous calcification
Osteochondritis Dissecans
boys 5-15 yo (11-20yo peak)
medial femoral condyle, knee, talar dome, ankle, elbow
joint mouse
defect at osteochondral region of bone
pain, effusion and locking
patellar lesions
Spontaneous Osteonecrosis of the Knee (SONK)
flat articular surface in knee
Radiation Necrosis
sclerotic irregular bones