Chapter 46 - Miscellaneous Bone Lesions (CHERI Notes) Flashcards
The most common cause of dwarfism is ___. A congenital hereditary disease of failure of endochondral bone formation.(p.1090)
ACHONDROPLASIA
- the FEMURS and HUMERI are more profoundly affected than the other long bones; although the entire skeleton is abnormal.
DIAGNOSIS?
Characteristic finding is that the spine typically has narrowing of the interpeduncular distances in a caudal direction. (p.1090)
ACHONDROPLASIA
- the opposite of normal; in which the interpeduncular distances get progressively wider as one proceeds down the spine.
- the long bones are short but have normal width; giving them a thick appearance.
The term______ refers to the lack of blood supply with subsequent bone death and ensuing bony collapse in an articular surface. (p.1090)
AVASCULAR NECROSIS (AVN) or OSTEONECROSIS
- the etiology of AVN is an extensive differential that most commonly includes trauma; steroids; aspirin; renal disease; collagen vascular diseases; alcoholism and idiopathic causes.
- radiographic appearances range from PATCHY SCLEROSIS to ARTICULAR SURFACE COLLAPSE AND FRAGMENTATION.
Late and inconstant sign of AVN. (p.1090)
Just before collapse; a subchondral lucency is occasionally seen.
___ is extremely valuable in demonstrating the presence and extent of AVN; even when plain films are apparently normal. (p.1090)
MR
- considered to be the most efficacious way to evaluate a joint for AVN.
- it is useful not only in AVN of the hip but also in the knee; wrist; foot and ankle.
_____ OSTEOARTHROPATHY is manifested by clubbing of the fingers and periostitis; usually in the upper and lower extremities.; which might or might not be associated with bone pain. (p.1090)
HYPERTROPHIC PULMONARY OSTEROARTHROPATHY
- It is most commonly seen in patients with LUNG CANCER; but many other etiologies have been reported; including bronchiectasis;
GI disorders and liver disease.
The differential diagnosis for periostitis in a long bone without an underlying bony abnormality would include ____. (GIVE 6). (p.1090)
- Hypertrophic pulmonary osteoarthropathy
- Osteoarthropathy
- Venous Stasis
- Thyroid acropachy
- Pachydermoperiostosis
- Trauma
_____ is a rare; idiopathic disorder characterized by thickened cortical new bone that accumulates near the ends of long bones; usually only on one side of the bone; and has an appearance likened to “DRIPPING CANDLE WAX”. (p.1090)
MELORHEOSTOSIS
- It can affect several adjacent bones and can be symptomatic
The _____ are a group inherited diseases characterized by abnormal storage and excretion in the urine of various mucopolysaccharidoses
such as keratin sulfate (____) and heparan sulfate (______). (p.1090)
MUCOPOLYSACCHARIDOSES;
MORQUIO; HURLER
- these patients have short stature; primarily from shortened spines;
and characteristic plain film findings - the pelvis in this disorders is similar in appearance to that of achondroplasts; with wide; flared iliac wings and broad femoral necks.
In the spine; patients with ______ have platyspondyly (generalized flattening of the vertebral bodies) with a central anterior projection or “beak” off the vertebral body;
as viewed on a lateral plain film. (p.1091)
MORQUIO
______ show platyspondyly with a beak that is anteroinferiorly positioned. (p.1091)
HURLER AND HUNTER
A characteristic finding in the hands
in MUCOPOLYSACCHARIDOSES) is a _____. (p.1091
POINTED PROXIMAL FIFTH METACARPAL BASE THAT HAS NOTCHED APPEARANCE TO THE ULNAR ASPECT.
_____; also known as DIAPHYSEAL ACLASIA; that
is a not uncommon hereditary disorder that seems to affect multiple members of a family with multiple osteochondromas or exostoses.(p.1092)
MULTIPLE HEREDITARY EXOSTOSIS
- the KNEES are virtually always involved
- under tubulation (a widened diameter of the bone) is invariably present at the site of exostosis.
- the incidence of malignant degeneration in this population has been reported to be as high as 20%; but this is a gross overestimation;
with malignant degeneration being extremely rare.
An ______ is a cartilage-capped bone outgrowth that may be pedunculated or sessile in appearance. (p.1092)
OSTEOCHONDROMA
As with SOLITARY OSTEOCHONDROMAS;
the more ____-situated lesions are more prone to undergo malignant degeneration; whereas the more peripheral lesions are less likely to do so. (p._____)
AXIALLY-situated
- The proximal femurs are frequently involved and have a characteristic appearance
DIAGNOSIS? (p.1092)
The classically described radiographic appearance is cortically based sclerotic lesion in a long bone that has a small lucency within it that is called the NIDUS.
OSTEOID OSTEOMA
- The etiology of osteoid osteroma is unknown.
- It is a painful lesion that occurs almost exclusively in patients less
than 30 years of age and is treated successfully with surgical excision
or thermal ablation
It is the ____ that causes the pain and the surrounding reactive sclerosis in OSTEOID OSTEOMA. (p.1092)
NIDUS
- if the nidus is surgically removed or thermally ablated; complete cessation of pain is the rule.
- CT is often very helpful in the demonstrating (of OSTEOID OSTEOMA) the exact location or the nidus.
TRUE OR FALSE?
(in OSTEOID OSTEOMA) (p.1092)
- If the nidus of an osteroid osteoma is located in the medullary rather than the cortical portion of a bone; or if it is located in a joint; there is much less reactive sclerosis present.
TRUE
- this gives the lesion a different overall appearance than the more common cortical lesion in that it does not appear as sclerotic.
Up to 80% of OSTEOID OSTEOMAS; are located _____. (p.1093)
INTRACORTICALLY;
- with the remainder being in the INTRAMEDULLARY part of a bone.
- rarely; an osteoid osteoma will be present in the periosteum; causing exuberant periostitis
TRUE OR FALSE? (In OSTEOID OSTEOMA)
- The nidus itself is usually lucent but often develops some calcification within it.
TRUE
- It then has the appearance of a sequestrum; as seen in OSTEOMYELITIS.
- if the nidus calcifies completely; it blends in with the surrounding sclerosis and cannot be seen on most radiographs.
- Therefore; the diagnosis of an osteoid osteoma in no way depends on seeing a nidus.
TRUE OR FALSE?
Because an osteoid osteoma resembles osteomyelitis; regardless of the appearance of the nidus; it can be difficult to differentiate two radiographically. (p.1094)
TRUE
- it cannot be reliably done with plain films; CT or MR
- because the nidus is extremely vascular; it avidly
accumulates radipharmaceutical bone-scanning agents. - an osteoid osteoma will have an area of increased
uptake corresponding to the area of reactive sclerosis but; in addition; will demonstrate a second area of increased uptake corresponding to the nidus (DOUBLE-DENSITY SIGN)
TRUE OR FALSE.
Osteomyelitis has a photopenic area corresponding to the plain film lucency that represents an avascular focus of purulent material. (p.1094-1095)
TRUE
- The natural history of an osteoid osteoma is presumed to be spontaneous regression; as they are rarely seen in patients older than the age of 30.
______ (aka ________disease); this disorder is manifested by multiple 2-to 3 mm- thick linear bands of sclerotic bone signed parallel to the long axis of a bone.(p.1095)
OSTEOPATHIA STRIATA
(aka VOORHOEVE DISEASE)
- It usually affects multiple long bones and is asymptomatic; hence; it is usually an incidental finding.
_________ is a hereditary; asymptomatic disorder that is usually an incidental finding of multiple small (3 to 10 mm) sclerotic bone densities affecting primarily the ends of long bones and
the pelvis. (p.1095)
OSTEOPOIKOLOSIS
- It has no clinical significance other than it can be confused for diffuse osteoblastic metastases.
______ is a rare familial disease that is manifested by thickening of the skin of the extremities and face; clubbing of the fingers; and widespread periostitis (p.1096)
PACHYDERMOPERIOSTOSIS
- seem to be more common in black patients
- the periosteal reaction is similar to that of hypertrophic pulmonary osteroarthropathy; but pachydermoperiostitis is only occasionally painful.
______ is a noncaseating granulomatous disease that primarily affects the lungs.
- causes a characteristic LACELIKE PATTERN of bony destruction in the hands. (p.1097)
SARCOIDOSIS
- when the musculoskeletal system is involved; the HANDS are mainly affected; with the spine and long bones only infrequently involved.
- Multiple phalanges are typically affected in either one or both hands.
- it is so radiographically characteristic that there is almost no differential diagnosis for this pattern.
______ (aka ____); this poorly understood disorder is an idiopathic process that begins with a painful hip with no underlying disorder or other findings other than osteroporosis; which
is limited to the painful hip. (p.1097)
TRANSIENT OSTEOPOROSIS OF THE HIP
(IDIOPATHIC TRANSIENT OSTEOPOROSIS OF THE HIP)
- MR findings are similar to early AVN
T1WI: low signal seen througout the femoral head
and neck - edema is typically greater than with AVN and no well-demarcated margin is present.
- self-limited with full resolution
- it tends to occur more often in males.
A similar process like transient osteroporosis of the hip which occurs in the KNEE is called ____? (p.1097)
PAINFUL BONE MARROW SYNDROME
- seen most commonly in the medial condyle; but can occur laterally or in the proximal tibia adjacent to the joint.
- protected weight-bearing is recommended to prevent insufficiency fractures
Painful bone marrow edema has been reported in the hip (ITOH); knee; distal clavicle and ankle.
It can occur in the several different locations over time or simultaneously and then is called ____. (p.1097)
REGIONAL MIGRATORY OSTEOPOROSIS
