Dysplasia's Test 2 Diagnostic Imaging Summer 2005 Kuhn



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DYSPLASIA's Test 2 Diagnostic Imaging Summer 2005 Kuhn

 

Achondroplasia, marfans, osteogenesis imperfecta, osteopetrosis, cleido cranial dysplasia



 

 

See and identify by definition



  • Spondyloepiphyseal dysplasia, Hurlers syndrome, morquio syndrome, melorheostosis, osteopoikilosis, progressive diaphyseal dysplasi, pyknodysostosis

 

 

Achondroplasia - most common organized dysplasia and most common form of dwarfism



  • Gait abnormalities

 

Cleido cranial dysplasia

  • 10% of these patients have total loss of clavicle

  • Abnormality of clavicle

 

Sclerosing dysplasia - I see one a year in the reading practice so you might see one every ten years or so

  • Sometimes there are clinical implications depending on the dysplasia

 

 

Next two weeks in this section then to normal variance



 

Achondroplasia

  • Key x-ray findings

  • Disproportionate shortness and body lengths

    • Spine is within the normal range according to the book

    • Extremities are much shorter than normal

      • The humerus and femur are smaller than the attaching bone (rhizomelia - short root)

    • Head size is normal, shape of there nose is flatter (depressed nasal ridge)

      • Wider R to L and shorter A to P (brachicephali - short skull)

    • Hands

      • Lengths are shorter, but width is the same

      • Have large gaps between the third and fourth fingers (trident hand), dividing the digits up when the expand them to thumb, 2nd and 3rd, 4th, and 5th

    • Hips - Coxa valga, coxa vera (hip abnormalities)

    • Knee - can be bowed leg or inward turning at the knee

    • Increased lordosis of lumbar spine and an antiverted pelvis with possible abnormal pelvic bowl. (ASIS - ant. inf.) this is called a champagne glass pelvis.

    • Scapula - looks more like a 4 sided structure instead of 3 sided structure that we are use too

    • Prominent buttock - possibly due to antiverted pelvis

    • Bones made enchondrally, they have maldevelopment of the intramembranous bone growth

    • Do not achieve normal growth within the bones

  • SLIDES OF DYSPLASIA

    • Achondroplasia

    • Remanance of the pelvis still fusing, squared off appearance of the ilium

    • Humerus is thinner and shorter with a fairly good size deltoid attachment

    • Spinal demention is smaller than normal

      • Widest space is L1 between the pedicles

      • L5 is the smallest space

      • Posterior laminectomy can be done to open space if patient cannot get relief

    • Lateral view - central canal is too small (posterior scalloping) this is aquired because of the years and years of CSF pulsations, expansion of the dura - dura ectasia

    • Bullet nose vertebra - classic features associated with bigger types of dysplasia

    • Bowed femur - large femur head

      • If there is a problem then your body will not get the piezo effect to creat increased bone growth

    • Joint fixation leads to joint malfunction

    • Submental vertex view - smaller foramen magnum, usually did not survive the the birthing process and this is why

    • 80% of achondroplasia you cannot find the link in the family

  • Cleidocranial Dysplasia

    • Lungs are narrow and funnel chest present (when the top is small and large at the base) Neither clavicles are present

    • Cleido cranial dysplasia (when the clavicle is not present)

    • Pg 726 in Yochum Rowe - Cleidocranial dysplasia

    • Some strength lost due to lever action that clavicle provides

    • Inner digitation - this is how the sutures in the skull or described as normally

    • Pubis symphysis is not developed giving hypermobility in the SI joint. So SI DJD could occur more often.

    • Midline defects of cleidocranial dysplasia

    • Cleft palate ,hair lip, absence of a pubic bone, arched palate, persistant metopic suture - right and left frontal bone,

  • Marfans syndrome

    • Violation of length/width ratio in feet. Feet are very long showing arachnodactly (in the hands and feet, spider like appearance)

    • NBA players, more in high school and college since the rigor of the NBA is harder on marfans

      • 6ft is the average height, low 7ft is the highest…this is in men and women

      • Pectis excavata (caved in chest) - this is present in marfans syndrome

      • Disection of aorta can be common too

      • Lens dislocation - is one of the most common clinically significant signs

      • Special order shoes

      • Square looking thoracic cage with high arched ribs

      • Pectis excavata will push heart further to the left

      • In fingers it looks like a width problem sometimes

    • Posterior scalloping - turners, TB, achondroplasia, lymphoma, neurofibromatosis

      • All of the above diseases can cause the posterior scalloping

      • Requires an historical review and may cause you to order a study

  • Osteogenesis imperfecta

    • Femur - young or old (look for physis or osteoporosis)

      • Not a normal callous - more callous than usual

    • Tarda forms - make it home and may escape the dx for 30 to 40 years

    • Congenita forms - notice at birth

    • Thin cortices, weak bone that can break and is part of a brittle bone disease

    • Brittle Bone diseases

      • Osteopetrosis, piknodysostosis, osteogenesis imperfecta

    • Tubularization bone empty in the middle (width is smaller in most circumstances, sometimes premature maturation can take place too (this is when the plate closes sooner than normal and so the bone gets trapped in a very small dimension)

    • Exuberant callous formation (mostly hematoma where the body is trying to fix the problem in the body)

    • Hematoma that turns into callous formation

    • Violation of length and width ration - the width is very narrow on the metacarpals (this is a sign of OI), this is due to poor crystal lattice formation, a blue sclera is present on this patient,

    • Sclerosing dysplasia = much greater bone density (Mallory Ostosis ) dripping wax appearance, more bone put down (picture of thumb with dripping candle wax appearance - this can be clinically significant on some films

      • Neurovascular compression is a common symptom when the bone is getting bigger

    • An osteoid on the humerus due to mallory ostosis - this disease can add bone to the outside or inside of bone…this makes this disease different from the rest. Most diseases cannot add to both.

    • On finger there is an added callous or bone formation on the phalanx and meta carpal. This is much more suddle, this is no mechanical stress or piezo electric effect present

    • Could be a stress fracture - whiter on the inside of bone, Mallory Ostosis

    • Sclerosing dysplasia - osteo petrosus (hard, and dense) - this is also a member of the brittle bone disease. This is also called chalk bone. This makes the bone easy to break. The body is unable to absorb fetal bone matrix. This mixes the fetal and adult bone. This does not allow for a good crystal lattice since it never was completed

      • Red marrow is harder to produce when you have this disease, can lead to anemia

      • Tarta - less fracture incidents, not all bones are included equally, not as much anemia

      • Primordial or fetal bone - Involves whole skeleton, anemia, susceptible to infection due to decreased WBC count

    • Femur neck is too small - coxa vera, this also allow for less weight to be held on the bone

    • Sandwich vertebra - this looks like an oreo double stuffed. Just the contrast is reversed. The vertebra is White on the ends and darker in the middle

      • Ruggered jersey spine - hyperparathyroidism causes this (looks like sandwich spine)

      • Osteo petrosis - sandwich type - brittle bone disease

    • SI joint not normal (edge of the film diagnosis…when the problem is on the edge of the film)

    • Wedge shaped fracture in vertebra

    • NON union fracture

    • Knee - increased density and sclerosis is present due to the non union fracture, this is not normal bone which makes it harder to distinguish the broken bone (brittle bone disease)



 

 

 



 

Main features of:

 

Cleidocranial



  • Defect in clavicle

  • Wormian bones - in suture lines

  • Maxilla not formed often, palate high arched, cleft palate, hair lip

  • Absence of pubic bone

  • Narrow shoulders - funnel chest

  • More mobility in shoulders

  • Pelvis is on the smaller side, and SI will have more instability or mobility due to missing the pubis symphysis. This can cause DJD to occur at an earlier time.

Marfans

  • Arachnodactly - spider fingers and toes

  • Lens dislocation

  • Pectis Excavata

  • Aorta dissection or aortitis

  • Thumb sign, can fold thumb into hand and it will stick out past the pinky finger

 

Osteogenesis Imperfecta

  • 2 forms - 1 for being recognized at birth and another later on in adulthood

    • Tarta - this is adult hood

    • Congenita - recognized at birth

  • Bone is very fragile and will break after a lesser degree of force

  • Abnormal development of tissue and even the layers of the eyeball

    • They are missing a brown choroid layer which allows the venous bed to show through giving the appearance of a blue sclera

  • Some people can have in upwards of 1000 fractures

  • Exuberant callous formation - this can be due to the fracturing that takes place

    • This can cause any bone to look like it has football pads or some type of pads on them

 

Femur is filled in (too much bone) Osteopetrosis is occurring, chalk bone, some anemia occurring within the bone.

 

Sunrise view of the patella (bone with in a bone) Brittle bone



 

Sclerosing dysplasia

  • Osteopoikilosis

    • Spotted bone or poka-dotted bone structure

    • Cortical bone with in the medullary cavity

    • Periarticular - shows up more prevalent closer to joints

    • Only in the bones

  • Stippled epiphysis (in the young person and is around the growth plates)

  • Synovio chondro metaplasia (group of poka-dots again) - learn this next tri…

    • This is when dots are outside bone and is an

 

Apperts - Mitten hand, skull is not normally shaped being to short front to back

Scaph (shallow draft boat) ….Schapocephaly - premature closure of the coronal suture



  • GROWTH PLATES JUST COMPLETE EARLY

 

Styphilopiphysis (stiphyl = poka dotted)

  • Looks like chunks of bone within the growth plate (chipping)

 

 

Spondyloephysial dysplasia (SED)- Heaped up vertebrae is the x-ray sign…the target is the endplate of the vertebrae

 

Progressive diaphyseal dysplasia - filling in of the medullary portion of the diaphysis of the long bones

 

Engelmanns - no brittle bone, denser trabeculae and cortical

 

Brittly Bone diseases



OI

OPetrosis



Picknodysostosis - normal intelligence, cannot handle high impact sports as well.

No teeth


Skeletal maturity of spine

Elfin like features - Small faces, near normal size heads, more head than face, patches of dense bone in the skull

Resorption of distal phalanx - acroosteolysis (missing fingertips)

 

 



 

Normal Variance - will see slight differences and major differences

 

Theodore Keets (normal variance and congenital anomalies)



 

Get the hits

 


  • 2 different slices of the skull, bone is white, this is a CT scan

Diploic space, incidental finding - the diploic space is missing and frontal bone is too thick, (hyperostosus internus frontalis)

 


  • Hole is in back of the top of the skull, the holes are bilateral

    • Osteolytic?, Geographic?, Bullet?, infection? Congenital?

    • Parietal foramen - is it palpable…yes

    • These people can do pretty much anything

 

  • Down's sydrome (trisomy 21)

    • Most common autosomal syndrome

      • 1:600 births

    • Recognizable at birth

      • Brachycephaly - from front to back it is too small

      • Small nose with a flat bridge

      • Slanting eyes

      • Protruding tongue

      • Depressed cerebration a constant

    • Clinical reminders

      • Leukemia much more common

      • Abnormal transverse ligament 20%

        • Stress views

        • Pre-participation exam

 

  • ADI

    • Spinolaminar line (not complete)

    • Transverse ligament failed

    • C1 is intact, yet forward

    • Odontoid is abnormal

    • Need MRI,

 

  • Failure of segmentation…BLOCK VERTEBRA

    • Embryological failure of sclerotome segmentation and separation first described by macalister in 1893

 

  • Posterior arch of C1 is next to the occiput, ADI is large

    • Occipitalization, Craniovertebral senostosis

    • This person does not have occiptal atlanto joint working

    • Plastic deformation can occur too

 

  • Cervical Block vertebra at C3 and C4

    • Both anterior and posterior fusion is usually congenital

    • Rudamentary disc make up

    • When surgery is done, there is usually only one fusion, (post or ant)

    • Wasp waist appearance

    • DJD usually occurs at the levels above and below

    • Congenital vs acquired

      • Do they have scars?

 

  • Anomalies of the cervical spine

    • Hypoplasia and agenesis

      • 7th fetal week-lateral masses begin to ossify

      • 2nd year - posterior tubercle forms and unites the arches

      • 2-5 years - completion of fusion

 

  • No C1 visible (agenesis of the posterior arch of C1)

    • Spina bifida, C1 is absent, supposed to be a ring structure not a C structure

 

  • Mega Spinous - when a spinous is bigger than normal

    • If you are missing bone somewhere it is likely that the bone is somewhere else

 

  • Physiologic calcification

    • Cartilage is the 2nd most common thing to pick up calcium

    • Osteoid is 1st

 

  • Os Odontoidium

    • Overall considered uncommon

    • Ununited ossification center

    • Long-standing non-union fracture

 

  • Odontiod is missing

    • Do MR image to check on cord

 

 

Clinical Questions



(aka your turn to talk)

  • Does the patient know?

  • Should the doctors know? Through palpation

  • Clinical relevance?

  • Documentation imperatives

 

OS Odontoidium

Patient may not know

Doctor probably should suspect an abnormality

Not recent

Atlanto axial instability

Sub occipital muscle spasms and headaches

VBAI


 

Occiptal atlanto joint and dealing with odontoid



  • Plastic injury

  • ADI 3mm adult, 5mm child

  • Limit translation

 

ADI instability is a problem with blocked vertebra, and DJD is next with the most common complications.

  • Osteophytes are linked to DJD

 

 

Aquired Fusion (ankylosis)

Surgical

Osteomyelitis or discitis

Inflammatory joint disease

 

Is the block congenital anomaly, acquired?



 

Multiple blocked vertebra - Klipple file syndrome

Sprangles deformity

  • Unilateral deformity of the scapula

 

Klipple file syndrome - common signs

Omovertebral bone

Sprengles deformity

Treja Coli

 

7-26-05

Scramble spine - hemi vertebra

Sacralization

Occipitalization

 

A - one sided

B - bilateral

TP

1A - single TP measuring above 19mm (normal disc herniation)

1B - both TP measuring above 19mm (normal disc herniation)

Joints (accessory articulations)

2A - single accessory joint (most clinically significant)

2B - bilateral accessory joint (herniation of disc is lower than 2A but is highly possible, 2nd most clinically significant)

Bars

3A - bar connecting TP of one side to the sacrum (protect from herniation)

3B - bar connecting TP of both sides to the sacrum (protect from herniation)

Bar and accessory joint both

4 - both bars and accessory joints (protect form herniation)

 

Tropism cannot be seen on plain film

 

End plate fracture is an aquired normal variant



  • Axial load event, Nucleous pulposes thru growth plate = limbus

  • Schmorls nodes - thru end plate

  • HNP - thru the annular fibers

 

No Sacrum - appears almost butterfly shape

Cupids Bow locks L5 and sacrum together

AKA: notocordal persistancy

 

Should see 10cm or more between T8 and sternum,



Posterior deflection of the sternum - pectus excavata

Pigeon chest - pectus caronata

Kyphosis will be reduced too, abnormal cardiac sounds are all part of straight back syndrome.

 

Kyphosis increases as you get older normally.



 

When measuring femur we check 3 things.

Shoulder is the one we report on.

 

Know measurements and lines for Final



 

When articular cartilage is broken off it tends to ossify over time.

Fabella - accessory ossicle which would be a normal variant

 

What lives in a cysts?



  • Infection, uterine cysts, iliac artery aneurysm

  • Fetus

 

Half Sphericle Spondylosis Sclerosis

 

Myerdings - grade 2



Ullmans line - would assure us of the retrolisthesis

Percentage of anteriority - measure the entire end plate then measure the amount of anteriority (how much end plate is not aligned)

  • this is the preferred method

  • If beyond 3mm then there is a problem (translation)

  • Surgery will have to be used if translation continues or is too bad

 

Know and discuss 5 major categories that cause anterolisthesis



  1. Dysplastic -

  1. Isthmic - acquired and healed incorrectly

  1. Degenerative - the facet joints truly control the AP position of the vertebral bodies, thinning of the articular cartilages will limit the amount of translation

  1. Traumatic - fracture of a posterior

  1. Pathologic - metastasis, pagets disease, stretched or fractured bone due to pathology

  1. Iatrogenic - surgery

 

It is not about the anterolisthesis as much, but do they have movement? You want to look at this part. Pain control treatments are very valuable to people.

 

The most frequent cause for anteriority:

Isthmic is #1 (most frequently stressed is pars interarticularis)



  • Eskimos have 40% anterolisthesis

  • Due to having child on back and the child having to learn to walk in snow shoes

  • Out of 2100 cadaverrs - 0 posterior elements maldeveloped (dysplastic)

Degenertaive is #2

Dysplastic is #6

 

3mm of translation needed to determine as an anterolisthesis



 

Common terms to be aware of

AP at lumbo sacral junction:


  • Upside down napolean hat…aka…Brow's line of Brailsford

 

ONE of a kind territory:



  • In bone, Dark lesions in a T1, bright in T2, weighted image is bad news usually

  • There was such an abnormality that the cancer was missed

 

 

Since we can not take the facet joints in a complete circle we have asymmetrical facet joints



 

Painful extension



  • Local and can go scleratogenous

  • Local pain and segmental distirbution

 

Sprengles - has to due with the clavicle, unilateral elevation of the scapula

 

Omovertebral Bone



 

Klipple file syndrome = multiple block vertebra syndrome, webbed neck,

 

Rhomboid Fossa - extra depth, muscular attachment site…no clinical significance



  • Second most frequently fractured bone in the body

 

Butterfly vertebra - when growth centers do not grow correctly



  • We do not know why this happens

  • Void in genetic code?

 

Hypertrophic TP and rib on other side (cervico thoracic transitional vertebra)

 

Cervical ribs - used to remove them and then after the surgery things actually got worst most of the time.



 

Lumbar Ribs - note this so the doctor knows.

 

Intrathoracic rib - has pleural tissue around it, and goes down through the thoracic area (goes anterolateral)



 

Synostosis - bones that are connected together when they are supposed to be separate

 

Spina Bifida Occulta



 

Harrington Rods

SbS

Cupid Bow



Megan blasé

 

Sesmoid Bones - these bones can break and undergo inflammation



  • Bipartate normal variance

Senostosis

 

Peronious Brevis calcification



Dystrophic calcification - where the ligaments become calcified after multiple traumas

  • Consequence is a higher rate of spontaneous rupture

  • Os peronei or calcifictendonitis

  • Ossicle will not hurt when you poke on it

 

Sprengle's - shoulder did not move down, High shoulder



  • Can be related to Klipple Fiel Syndrome

 

AC joint

 

Limbus, schmorls nodes, herniation….all the same?



  • Limbus - when the disc goes through a growth plate

  • Schmorles nodes - disc through an end plate

  • Herniation - through the annulus fibrosis

 

Supracondular process (on the humerus, distal 1/3 directed towards the elbow joint)



  • Struthers ligament goes from the humerus to the radius

  • Osteochondroma could be a possibility, but has different features

    • Mixed presentation

    • Bone and cartilage

    • Pedunculated form (narrow base with a big head)

    • Sessile form (large base, smaller head)

    • Wide distribution

    • Pointing away from the elbow joint

 

Normal radial ulnar articular surface should be a straight line…not V shaped

Madelung deformity


  • Not as mechanically stable

  • Scaphoid fractures more often

  • Carpals are disorganized

  • V shape of articular surface

  • Posterior ulna on lateral view. Ulna is not participating

    • Bayonet Wrist

 

Non union fracture or un-united growth center



  • Ulnar styloid process takes a lot of force to break it

 

Radius and ulna alignment



  • Is the radius too long?

  • Ulna minus…the ulna is not long enough

    • Increased injury risk

  • Carpals are normal

  • Bone plus minus to describe a short bone

 

V shaped wrists - is this MADELUNGS deformity…aka…bayonet wrists

 

PIP of the middle ray is gone



Small 5th metacarpal

 

 



 

 

Imaging modalities



 

 

Dysplasia - interesting because of what they do to the bone



  • Apperts - brachycephaly…premature closure of the coronal suture

    • Long AP - scaphocephaly…midsaggital closed early

  • Cleidocranial dysostosus

  • Brittle bone diseases

  • Acroosteolysis

  • Arthritides

  • Endocrine disease

 

Rhizomelia, bullet nose vertebra,

 

Heaped up vertebra - spondyloepiphyseal dysplasia



Most common cause for leathal dwarfism is the small foramen magnum causing hydrocephalus

 

Cleido Cranial dysotosis



  • No clavical

  • Funnel chest

  • Increased mobility in shoulder

  • Drooping or sloped shoulder sign

  • Wormian bones

  • Pubic bone width is increased - midline defect

 

Midline defect - cleft palat, pubis

 

Arachnodactly



  • Width smaller, length longer

  • Thumb sign - thumb stick out side of fist when thumb is closed within the hand

    • Usually seen with Marfan's syndrome

 

Marfan's - lens dislocation, scoliosis, pectus excavatum (missing right heart border), look for murmers

 

Posterior scaloping



 

Osteogenesis imperfecta

Cortex to trabecular = 1 to 1 (that's what it should be)

Blue sclera

Trauma


Thin cortex

Tarda - go further along in life with out fractures, if congenitalthen you have to work harder

 

Too skinny, OI



  • Osteo imperfecta…skinny metacarpals are present

 

Short stubby hands Melhorheostosis.



  • Neurovascular compression

  • Can fill in bone and lay bone on the outside

 

Sclerosing dysplasia - inward filling with out ward pump

Melhorheostosis

 

Osteopoikilosis



Stippled epiphysis

Transitional segment 2a

Heaped up vertebra

Long bones and diaphyseal portion of long bones - Angles mans

Elfan features - nose is different

Multiple zones of increased density with fractures that do not heal well - picknodysostosus

Finger tips and toe tips breaking - ackroosteolysis

 

OI, Osteopetrosis, picknodysostosis are all brittle bones diseases



 

Limbus is acquired by axial compression - through the growth plate.



  • Fractures do not come with corticated margins

 

CT study Bone window, attenuation image, totally based on differential absorption

 

Anterolisthesis is most often measured in percentage



  • 7 in 100 have this

 

Asymetric motion is apparent with tropism, not seen well on plain film, must use CT or MRI

 

CT - not looking for symmetry, not a complete circle



 

Knife clasp - LONG SP off L5

 

Hyperostosus internous - thick calvarium



 

Bilateral = congenital anomalies

 

No transverse ligament creates to opportunity for anterior translation



  • Spinolaminar line broken

  • ADI space

 

MRI should be performed (T1 is present on slide)

 

Atlanto Axial instability with block Vertebra (DJD)



 

Mega spinous…where did that bone come from? Probably posterior arch of C1

 

Spondyloscesis (267 & 268)



  • 2 C bones make up C1

 

Posterior ponticle - calcification of the ligament



  • 3 catagories

    • Dystrophic, metastatic, physiologic

Spinolaminar line does not include C1, however some other stuff is normal

 

Corticated margins



 

Developmental Cleft Vertabrae (parallelism present this is why it is not a fracture)



 

 

 



 

 


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