MS Rad 09-13 Congenital & Developmental Disorders Flashcards

Terms Definitions
Achondroplasia Radiologic Features
Shortening of long, tubular bones. Look at spine & pelvis
Achondroplasia Radiologic Features:
skull normal size, normal size brain
Achondroplasia Radiologic Features: face
made of enchondral bone formation = small relative to the skull
Achondroplasia Radiologic Features: skull base
enchondral bone formation = small --> hydrocephalus & paraplegia from constriction at base of skull & spine
Achondroplasia: lateral view of pelvis
narrowing of interpediculate distance in the lumbar spine
Achondroplasia: lateral view of spine
intervertebral disc spaces are widened & as high as the adjacent vertebral bodies. Short pedicles = spinal stenosis. Short pedicles & narrow vertebral canal. Bullet shaped vertebral bodies in the lumbar spine. posterior scalloping of the vertebral bodies. Slight kyphosis in lumbar spine.
Achondroplasia: Radiological Features of Bones
proximal longs bones are shortened, short & thick tubular bones, notched (V-Shaped) growth plates creating ball-in-socket epiphyseal-metaphyseal junctions, flared metaphyses, fibular overgrowth
Achondroplasia: Hands & Feet
short & stubby, with tips squared off. 2-4th fingers are = length.
Osteogenesis Imperfecta: Etiology
Inadequate osteoid formation, normal mineralization. result is thin, osteoporotic, fragile bones with severe bowing & fxs. Trabecula is too lucent.
Osteogenesis Imperfecta: Type I
90%, tarda form, normal life expectancy, blue sclera, laxity of ligaments, dental abnormalities, deafness, otosclerosis
Osteogenesis Imperfecta: Type II
10%, congenital, death in utero or neonatal period due to lung hypoplasia
Osteogenesis Imperfecta: Type III
fx @ birth, progressive limb deformity, normal sclera & hearing
Osteogenesis Imperfecta: Type IV
bone fragility, normal sclera & teeth
Osteogenesis Imperfecta: Long Bones
lucent, general demineralization, short telescoped long bones secondary to in utero fx, lots of fx, bowing of the bones, healing concertina-like fxs, *multiple fxs in various stages of healing*, bones are thin, have few trabecula, & thin cortices
Osteogenesis Imperfecta: Ribs
beaded appearance secondary to multiple fx, small, irregular, ribbon-like healing fx
Osteogenesis Imperfecta: Legs
bowed, healing concertina-like fx
Developmental Dysplasia of the Hip: Patholgoy
at birth or months/years later, an abnormally lax joint capsule allows the femoral head to fall out of the acetabulum, leading to deformity; more common on left side
Developmental Dysplasia of the Hip: Predisposing Factors
ligamentous laxity (estrogen effects = female: male is 6:1), acetabular dysplasia (increased angle, shallow acetabular fossa), breech presentaiton at birth
Developmental Dysplasia of the Hip: Hilgenreiner's Line
horizontal line drawn through the triodiate cartilages
Developmental Dysplasia of the Hip: Perkin's Line
vertical lines drawn through the lateral edge of the acetetabulae
Developmental Dysplasia of the Hip: Location of Femoral Head on XRay
normal: inferior medial quadrant
Developmental Dysplasia of the Hip: Shenton's Line
smooth arc drawn from the medial femoral neck through the superior margin of the obturator foramen
Developmental Dysplasia of the Hip: Acetabular Angle
Hilenreiner's method: acetabular angle should increase with age: 0-1 = <34 degrees; 1-4 = <28 degrees; >4 = <25 degrees; high angle --. shallow acetabulum
Clubfoot: Pathology
foor is at a right angle to the tibia; 1:1000; ; metatarsal bones seen in the same plane as tibia & fibula; parallwl talus and clacaneus on both AP and lateral views
Clubfoot: secondary
trisomy 18, amniotic band syndrome, meningocele
Arthrogryposis: Etiology
1:3000; "curved/hooked" joints, contractures of at east 2 sets of joints & muscles; believed to be caused by oligohydramnios and conditions limiting fetal movement; low vol. of amniotic fluid --> less movement --> joint contracture
Arthrogryposis: Akinesia Sequence Upper Extremity:
waiter's sign, Adduction & internal rotation of humerus, elbow extension 7 wrist flexion, ulnar deviation.
Arthrogryposis: Akinesia Sequence Lower Extremity
Rigid clubfeet are most common deformity, hips are usually flexed, externally rotated & abducted, persistent "pike" position
Congenital vertical talus: etiology
axis of talus is steep, convex plantar surface of the foot, which may result in *rocker bottom feet*
Congenital vertical talus: associations
Meningomyelocele, arthrogryposis, trisomies 13 & 18
Normal Femoral Neck
angle between neck & shaft = 120-135 degrees
Femoral neck: Coxa Vara
angle <120 degrees, causes the knee to move medially, short femoral neck, limping & deceased physical activity will results,
Femoral neck: Coxa Vara Causes
idiopathic, malaligned healed fx of the femoral neck, legg perthes disease, slipped capital femoralepiphysis, rickets, fibrous dysplasia
Femoral neck: Coxa Vara Trendelenburg test
if abductors are not functioning, hip will dip on the side witht he foot off the ground (opposite side)
Femoral neck: Coxa Vara Treatments and Complications
splintting & decreased physical activity; degenerative joint disease & AVN
Cleidocranial Dystostosis: Etiology
defect in ossification: clavicle is absent, partially absent or unfused, coxa vara femoral neck, diastasis of the pubic symphsis, *tubular bone sof hands have short distal phalanges*, supernumeray epiphyses, abnormal cortical formation (too thin)
Legg-Calve-Perthes: Etiology
idiopathic AVN of femoral epiphysis, M>F, ages 5-8, fragmentation and flattening of sclerotic femoral epiphysis, metaphyseal irregularity
Legg-Calve-Perthes: Tx
bedrest, abduction stretching and bracing; 50% improve w/o tx
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