pituitary problems, achondroplasia, marfan

Question 1

what accounts for 10% of all neoplasms within the cranium?

Answer 1

pituitary tumors

Question 2

what percent of people have some kind of pituitary adenomas in autopsy studies?

Answer 2

25%

Question 3

symptoms of pituitary tumors

Answer 3

headaches
visual disurbances
generalized discomfort in exremities
symptoms vary depending on type of tumor

Question 4

can you tell on xray if someone has a pituitary tumor?

Answer 4

possibly, it is possible to have a pituitary tumor with an enlarged sella turcica

Question 5

what are the potential causes for an enlarged sella?

Answer 5

empty sella
tumor
normal
aneurysm

Question 6

generalized description for acromegaly and gigantism

Answer 6

pituitary adenoma secreting too much growth hormone
in the adult intramembranous bone tissue and subcutaneous hypertrophy
prominent in the hands and feet
excessive growth hormone before growth centers close manifest gigantism
predisposes to DJD
reduces lifespan

Question 7

etiology of acromegaly

Answer 7

oversecretion of growth hormone from a pituitary adenoma occuring AFTER the closure of open growth centers

Question 8

signs and symptoms of acromegaly

Answer 8

soft tissue thickening
coarse facial features
enlarged jaw, hands, feet, head
spreading teeth
change or loss of normal menstrual function
macroglossia
malocclusion
protruding jaw with malocclusion
protruding frontal sinuses
prominent forehead
enlarged sella turcica

Question 9

radiographic features for extremities for acromegaly

Answer 9

spade-like distal tufts
hooking osteophytes
increased joint spaces
widened shaft
increased tissue thickness

Question 10

etiology of gigantism

Answer 10

same as acromegaly except onset of growth hormone oversecretion occurs prior to skeletal maturation

Question 11

causes of cushings/hypercortisolism

Answer 11

exogenous corticosteroid administration

increased in patients requiring immunosuppression

Question 12

features of cushing’s

Answer 12

excessive glucocorticoid steroids, released by the adrenal cortex
occassionally anterior pituitary tumor
patient is obese, especially in the upper thorax and face
accelerated hair growth
hypertension

Question 13

clinical features of cushing’s

Answer 13

moon face
buffalo humb
abdominal striae
back pain (secondary to compression fx)
easy bleeding

Question 14

radiographic features of cushing’s

Answer 14

generalized osteopenia
compression fractures
AVASCULAR NECROSIS
atherosclerotic plaquing

Question 15

achondroplasia background

Answer 15

MC congenital dwarfing skeletal dysplasia
marked by hypochondroplasia
normal life expectancy and mental status
normal or near normal trunk length with marked rhizomelic micromelia

Question 16

radiographic features of achondroplasia

Answer 16

narrowing of the spinal canal
small foramen magnum
symmetric shortening of all long bones
champagne glass pelvis
broad ilia, ribs and sternum
short, thick and tubular extremtiies
metaphyseal cupping

Question 17

spinal radiographic features of achondroplasia

Answer 17

posterior body scalloping
short pedicles with narrowed interpediculate distance leading to a narrow, trefoil spinal canal
decreasing caudally
rounded bullet vertebrae
platyspondyly
horizontal sacrum

Question 18

skull radiographic features of achondroplasia

Answer 18

macrocephaly
frontal bossing
foramen magnum stenosis
associated with arnold chiari malformation

Question 19

clinical features of achondroplasia

Answer 19

waddling gait, broad and flat nasal bridge

Question 20

clinical presentation of cleidocranial dysplasia

Answer 20

defect of intramembranous bone growth

Question 21

radiographic features of cleidocranial dysplasia

Answer 21

skull, clavicle, midline defects, pubic diastasis
inverted “pear shaped” skull
wormian bones
CLAVICLES hypoplastic or absent

Question 22

clinical features of cleidocranial dysplasia

Answer 22

generalized dysplasia, below average height, large head, drooping shoulders

Question 23

marfan syndrome background

Answer 23

collagen disorder fails to produce normal collagen

connective tissue disorder

Question 24

marfan syndrome imaging

Answer 24

scoliosis, acetabular protrusion, pectus excavatum

Question 25

clinical marfan syndrome

Answer 25

tall stature, arachnodactyly, heart valve defect, arotic aneurysm, lens dislocation

Question 26

osteogenesis imperfecta

Answer 26

inherited disorder marked by abnormal type I collagen formation
at least 4 subtypes exist ranging from mild osteopenia to dwarfism with multiple fractures

Question 27

what are the 4 major clinical criteria for osteogenesis imperfecta

Answer 27

skeletal fragility
blue sclera
abnormal dentition
premature otosclerosis

Question 28

radiographic features of osteogenesis imperfecta

Answer 28

osteopenia, bowed long bones, thin cortices, multiple fractures, kyphoscoliosis

Question 29

clinical features of osteogenesis imperffecta

Answer 29

may be lethal at birth
often confused with child abuse
treatment is directed at limiting deformity and injury

Question 30

background for osteopetrosis

Answer 30

goup of entities, preresenting a type of sclerosing bone disease
hereditary and familial
abnormality of decreased osteoclastic activity

Question 31

imaging assocated with osteopetrosis

Answer 31

loss of medullary space
pathological fractures
dense bones, thick cortices
hypoplasia of frontal sinuses
"bone within a bone" or "endobone"
"sandwich" vertebra
undertubulation of extremities resulting in an "erlynmeyer flask" deformity