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Jeff Binder R.T. (R). ConditionBrittleSclerosing Melorheostosis X Osteopetrosis XX Osteopoikilosis X Progressive Diaphyseal Dysplasia (PDD) X Pyknodysotosis.

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Presentation on theme: "Jeff Binder R.T. (R). ConditionBrittleSclerosing Melorheostosis X Osteopetrosis XX Osteopoikilosis X Progressive Diaphyseal Dysplasia (PDD) X Pyknodysotosis."— Presentation transcript:

1 Jeff Binder R.T. (R)

2 ConditionBrittleSclerosing Melorheostosis X Osteopetrosis XX Osteopoikilosis X Progressive Diaphyseal Dysplasia (PDD) X Pyknodysotosis XX Osteogensis Imperfecta X

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: 3 rd decade  DJD: 3 rd decade  Trident hand: separation of 3 rd and 4 th 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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