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Jeff Binder R.T. (R) Skeletal Dysplasia.

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Presentation on theme: "Jeff Binder R.T. (R) Skeletal Dysplasia."— Presentation transcript:

1 Jeff Binder R.T. (R) Skeletal Dysplasia

2 X Condition Brittle Sclerosing Melorheostosis Osteopetrosis
Osteopoikilosis Progressive Diaphyseal Dysplasia (PDD) Pyknodysotosis Osteogensis Imperfecta

3 Achondroplasia Squared off pelvis
Champaign glass pelvis (caused by anterior pelvic tilt) Squared scapula Widest pedicle spacing at L1 tapering to smallest spacing at L5 (reverse from normal) Rhizomelia: “short root”, Single long bones (humerus, femur) are short but normal diameter Bullet nose vertebra: rounded front of vertebrae by defective development of growth plate Scalloped posterior vertebra: caused by dural ectasia Canal stenosis: 3rd decade DJD: 3rd decade Trident hand: separation of 3rd and 4th fingers Hyperlordotic lumbar spine Small foramen magnum: MOST LETHAL ASPECT

4 Osteopetrosis “BONE WITHIN A BONE”
Fetal bone (primordial) never retreats “chalk bone” appearance (very dense on film) Erlenmeyer flask deformity: flared metaphysis of long bones Dental caries and abscess formation associated Sandwich vertebra (also called rugger jersey) Anemia is the most lethal aspect Reduction in red marrow due to decreased medullary bone Increased urine Ca levels (only slightly) abnormal bone scans (increased bone production)

5 Melorheostosis Normal Cortical bone distributed improperly
Strong bones Bone grown inside and outside of normal bones Only dysplasia that does this Dripping wax appearance No anemia Cortical thickening and wavy appearance Can cause peripheral nerve entrapment

6 Osteogenesis Imperfecta
Mucopolysaccharidoses Brittle bone disease NOT sclerosing Arachnodactyly: Long finger/toe appearance due to thinner bones Brachydactyly: short finger/toe appearance due to premature growth plate closure Sheppard’s crook deformity: hook like deformity of the femure Blue sclera: caused by brown choroid visualized through abnormal Dental problems Bluish gray to yellowish brown opalescent teeth Malformed dentin leading to easy chipping and severe caries Osteoporosis Wormian bones: not pathognomonic of OI Multiple fractures: causes excess callus formation on bones Biconcave vertebral bodies Undertubularization: narrow bones

7 Osteopoikilosis Spotty bone disease Benign Asymptomatic
Normal cortical bone growth Completely within bony margins If not within bony margins look for another diagnosis R/O osteoblastic mets in patients over 50yrs Bone scan will be normal in osteopoikilosis; abnormal in osteoblastic mets (hot spots) MRI will show a void in signal Cortical bone is black on MRI therefore areas of cortical bone growth are black

8 Spondyloepiphyseal Dysplasia
“heaped up vertebra” or “humped shaped vertebra” Caused by the non-ossification of the ring epiphysis Thin disk spaces Short vertebral bodies= short stature; Platyspondly Premature DJD Hip and knee invovlement Subchondral chondrosis

9 Progressive Diaphyseal Dysplasia
Engelmann’s Disease Cortical thickening of long tubular bones CORTICAL BONE ADDED ONLY TO THE INTERIOR OF BONES Poor muscular development Increased osteoblastic activity Bone scan will show Fusiform widening of only the Diaphysis

10 Pyknodysostosis Sclerosing and brittle bone disease
Altered craniofacial ratio “elfin like features”; small face, normal sized head Obtuse angle of the jaw Acroosteolysis: reabsorption of the distal phalanges Madelung’s deformity Bowing and overgrowth of the radius contributing to abnormal radioulnar articulations Dental carries with possible osteomyelitis of mandible

11 Marfan’s Syndrome Arachnodactyly: elongation of the fingers without an increase in width Lens (eye) dislocation Aortic aneurysms 45% acquire scoliosis Tall thin people Positive thumb sign Pectis excavatum: sternum close to the spine “caved in” 1/3 have congenital heart disease Atrial septal defect is the most frequent Failure to produce normal collagen Poor quality Dental carries

12 Cleidocranial Dysostosis
Midline structure deformities Clavicular hypoplasia Pubic symphysis midline deformity Spina bifida Cleft palate Wormian bones Widening of coronal and saggital sutures “hot cross bun appearance” Funnel shaped chest Hypoplastic and tapered distal phalanges Biconvex vertebral bodies

13 Other presentations Brachycephaly: “short skull”
Premature coronal suture closure P-A distance decreased Scaphocephaly: “boat head” Premature saggital suture closure S-I distance decreased Synphalagism No joints in a finger; finger 1 continuous bone Syndactyly: “mitten hand” No soft tissue separation in the hand

14 Trichorhinophalangeal
Hair, nose, finger Course hair Long nose Finger abnormalities

15 Stippled epiphysis Fragmented primary ossification center Benign

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