 Osteogenesis Imperfecta

Deff : kelainan herediter jar. ikat berupa sintesa atau

struktur abn. Dr procollagen I ( defisiensi pemben

tukan periosteal dan subchondral )

Osteoid normal, tp pembentukan tl kurang → tl panjang

Ke – mineral → tl tipis dan melengkung ( bowing ), meta

Physe melebar
Tanda Klinis :

 Osteoporosis . Tl fragile, mdh fraktur

 Blue sclera

 Dentinogenesis imperfecta

 Otosclerosis prematur

OI :

o Congenital : > berat, ditemukan saat lahir ,bowing ekst atas bwh

o Tarda : dpt normal dlm kehidupan

Gambaran Ro :
 Osteoporosis berat → fraktur spontan
 Deformitas tulang, penipisan tulang → bowing
 Tulang panjang btk seperti terompet
 Skull : calvaria tipis, pelebaran sinus frontalis,
mastoid
platybasia → basis kranii mendatar, tabula int
/ekt
tipis
 Vertebra : corpus flat, kyposcoliosis
 Pelvis : menyempit, protussion deformity acetabulum
Blue sclera
bowing
Osteoporosis berat, healing fraktur
Bowing, healing fraktur
Osteoporotik, fraktur
severe osteoporosis

healing fracture
osteoporosis,

bowing deformities

healed ulnar fracture

callus formation
platybasia
Achondroplasia
 Chondrodystrophy
 Kerdil ( Dwafism )
 Kel. Herediter ( autosomal dominant )
 Deff : mrpk ggn pertumbuhan pembtkan tl enchondral
 Patologis : retardasi & irregularitas pertmbhan cartilago
ujung epifise ,enchondral
Gambaran Ro
 Skull : kpl besar, basis kecil, sella kecil
 Skapula kecil
 Pelvis : cav spt gelas champagne ala ilii segiempat
sacroiliac sempit & pendek
 Tl panjang : pendek, tp fibula > pj tibia
radius > ulna
meta spt paruh (beaking shape)
 Grs epifise irreguler
 Vertebra : interpedincular menyempit
 Jari pendek, gemuk (stubby)
Shortening of tubular bones with a normal
shaft caliber
Short extremities and ribs-versus-trunk
length
Short phalanges
Ball-in-socket epiphyses
Metaphyseal flaring and cupping
Circumflex or chevron seat on the
metaphysis
Squared iliac wings and narrow sacroiliac
notch (champagne glass)
Fingers widely opposed and equal length
(trident hands)
Enlarged skull vault and mandible
Small foramen magnum
Narrow anteroposterior (AP) diameter of
the spine with a concave posterior surface
Decreased lumber interpediculate distance
and narrow spinal canal
Hypoplastic (bullet nose) thoracolumbar
junction vertebrae
Narrow vertebral interpediculate distance
shortening of the bilateral femurs

with metaphyseal flaring.

 shortening of the humerus

with posterior bowing

..
enlarged calvaria with

a shortened skull base

and frontal bossing

bowing of the humerus
Champagne-glass pelvis with

squared iliac wings

Trident hands
Shortened ribs
Osteopetrosis
 Marble bone disease
 Tjd defek pd absorbsi spongiosa primer dlm proses
enchondral bone formation
 Seluruh tl terkena → gban sklerosis seluruh tl baik pada
epifise, meta maupun diafise
 Antara cortex medulla sulit dibedakan
 Ro : tl spt botol erlenmeyer
basis kranii menebal, obliterasi foramina basalis,
→ tuli, atrophy optica, hydrocephalus
Basis kranii tebal
Rickets dan Osteomalacia
 Terjadi deffesiensi vit D
 Patologis : - ricket : ggan pertumbuhan & mineralisasi
growth plate
- osteomalacia : mineralisasi
inadekuat/delayed
 Osteomalacia → tjd dewasa
 Rickets : tjd anak-anak
 Ro : osteomalacia → trabekulasi tulang jarang dan kasar
rickets → epiphyseal line melebar
cupping & fraying metaphyse
 Pd costa → rachitis rosary

Tl panjang → bowing

• Etiologi : 1. Abnormalitas metabolisme vit D

Deff vit D : diet ↓ , sinat mthr ↓ , malabsorbsi D

2. Kelainan metabolisme phosphat

3. Deff kalsium
cupping and fraying

of the metaphyseal
cupping & fraying metaphyse
Tl panjang → bowing
The enlarged ends of the ribs Rachitic rosary
 Scurvy
 Caused by a lack of vitamin C
 Manifests as collagen defects, abnormalities in bone
maturation, epiphyseal disease, lifting of the periosteum,
and hemarthroses
 Gjl : nyeri, iritable
 Ro : - Wimberger’ s sign : epifise kecil, dibatasi
sclerotic rim
- Frankel’ s line : calcification metaphyse
- Trumerfeld zone : dibawah frankel line ada
daerah luscent ke – mineral osteoid
- Pelkan’s spur : mdh fraktur spur tepi cortex
Transverse metaphyseal lines of increased

and decreased opacity (Trümmerfeld zone)

metaphyseal beaks known

as Pelkan spurs
Dr. Lilis,SpRad.
BASIS TYPES CATEGORIZATION

 INFLAMMATORY
 DEGENERATIVE
 METABOLIC
INFLAMMATORY
 Soft tissue swelling and edema
 Uniform loss of joint space
 Bone erosions
 Juxtaarticular osteoporosis
 Periostitis
 Monoarticular or polyarticular
 Rheumatoid arthritis,psoariasis, ankylosing
spondylitis, Reiter’s syndrome
DEGERATIVE
 Nonuniform loss of joint space
 Osteophytes
 Subchondral sclerosis
 Subchondral cysts
METABOLIC
 Soft tissue masses within the periarticular soft tissue
 Well-marginated bone lesions
 Relative normal joint space
 Degenerative changes (sometimes)
 Gout arthritis, amyloidosis (rare)
THE MOST PREVALENT TYPES
 OSTEOARTHRITIS (DEGERATIVE JOINT
DISEASE)
 RHEUMATOID ARTHRITIS
 LUPUS ERYTHEMATOSUS
 ANKYLOSING SPONDYLITIS
 GOUT
 JUVENILE RHEUMATOID ARTHRITIS
 SCLERODERMA
DEGERATIVE JOINT DISEASE
GENERAL CONSIDERATIONS
 NONINFLAMMATORY DEGERATION OF JOINT
CARTILAGE WITH SECONDARY EFECTS ON
ADJACENT BONE
 THIS IS THE MOST COMMON FORM OF
ARTHRITIS
 SYNONYMS INCLUDE OSTEOARTHRITIS
DEGERATIVE JOINT DISEASE
CLINICAL FEATURES
 Pain, stiffness, crepitus, deformity, swelling with
normal laboratory studies
 Three types identified: primary, secondary, erosive
 Primary: unknown etiology, 5th to 6 th decade,
females 10:1, weight-bearing joints
 Secondary: known etiology, 2nd to 6 th decade,
equal sex, any joint
 Erosive osteoarthritis: inflammatory etiology, 4th to
5 th decade, females 3:1, interphalangeal joints
DEGERATIVE JOINT DISEASE
PATHOLOGIC FEATURES
 BEGINS FOCALLY AND GRADUALLY INCREASES
IN SIZE
 INITIAL LOSS OF CHODROITIN SULFATE WITH
SECONDARY STRESS EFFECTS ON ADJACENT
BONE
 ESCAPE OF SYNOVIAL FLUID INTO
SUBCHONDRAL BONE FORMS SUBCHONDRAL
BONE CYSTS
DEGERATIVE JOINT DISEASE
RADIOLOGIC FEATURES
 ASYMETRIC DISTRIBUTION, NONUNIFORM LOSS
OF JOINT SPACE, OSTEOPHYTES, SUBCHONDRAL
SCLEROSIS, SUBCHONDRAL CYSTS, LOOSE
BODIES AND SUBLUXATION
 SPINE: C5-7, T2-4, T9-12, L4-5.
 CERVICAL: Osteophytes, loss of disc height, IVF
encroachment, occasionally related to vertebral artery
syndromes and spinal stenosis (< 12 mm)
 THORACIC: Osteophytes on right side, increasing
kyphosis
 LUMBAR: Osteophytes, loss of disc height, facet
arthrosis, vacuum sign, anterolisthesis (L4), stenosis
(12 mm), instability
 Vacum phenomen
Osteophytes
bilateral medial femorotibial

compartment narrowing
←
subchondral sclerosis,

prominent osteophytes

a large Egger cyst

narrowing, osteophytes, and

subchondral cysts affecting

the distal interphalangeal joints

narrowing and osteophytosis

affecting the first

carpometacarpal joint.
Transverse CT scan

joint narrowing, osteophyte formation,

↑ and subchondral cysts

typical of osteoarthritis
joint narrowing, subchondral sclerosis,

osteophytosis
INFLAMMTORY DISORDERS
RHEUMATOID ARTHRITIS
 Generalized connective tissue disorder
 Unknown etiology, onset is usually between 20-60 years of
age, <40: females 3:1; >40: 1:1
 Selectively targets synovial tissue of the particularly
peripheral joints (hands and feet)
 The synovium of tendon sheaths and bursae may be also
affected.
 Bilateral symmetry
 Other body systrems maybe involved (the heart, lungs
small blood vessels, nervous system, eyes and
reticuloendothelial system.
GENERAL RADIOLOGIC FEATURES IN
RHEUMATOID ARTHRITIS
 Bilateral symmetry
 Periarticular soft tissue swelling
 Uniform loss of joint space
 Marginal erosions
 Justaarticular osteoporosis
 Justaarticular periostitis
 Large pseudocysts
 Joint deformity
 GENERAL RADIOLOGIC FEATURES OF
JUVENILE RHEUMATOID ARTHRITIS
EARLY
* soft tissue swelling
* osteoporosis
* periostitis

LATE
* uniform loss of joint space
* articular erosions
* growth distrurbances
* intraarticular bony ankylosis
* joint subluxation
* epiphyseal compression fractures
 Soft-tissue swelling and early erosions

in the proximal interphalangeal joints

←
in a patient with rheumatoid arthritis
Well-defined bony erosions in

the carpal bones and metacarpal

in a patient with rheumatoid

arthritis of the hands.

Ankylosis of the carpal bones

enlargement of the erosions

Subluxation in the metacarpo

phalangeal joints
Erosions

subluxation
 ANKYLOSING SPONDYLITIS
 SYNONYMS:
* Marie strumpell’s Disease
* Bechterew’s disease
* Pelvospondylitis ossificans
* Rheumatic spondylitis
* Rhizomelic spondylitis
* Spondylitis ankylopoietica
* Rheumatoid arthritis of the spine
* Rheumatoid spondylitis
RADIOLOGIS FEATURES OF ANKYLOSING
SPONDYLITIS
 EARLY
Localtion: Sacroiliac, thoracolumbar,
atlantoaxial
apophyseal joint erosions and ankylosis
atlantoaxial instability
lumbar hiperlordosis
marginal syndesmophytes
vertebral body corner erosions(Romanus lesion)
vertebral body corner sclerosis (shiny corner)
vertebral body squaring
 RADIOLOGIC FEATURES OF
ANKYLOSING SPONDYLITIS
LATE:
discovertebral instability (Andersson
lesion)
end plate concavity (ballooning)
facet fusion (trolley track)
fractures (carrot stick)
ligamentous ossification (dagger signe)
marginal syndesmophytes (bamboo spine)
ostreoporosis
peripheral joint infolvement
posture changes (kyphosis, chin on chest)
vertebral body atrophy (cervical spine)
complete fusion of

both sacroiliac joints.

Interspinous ossification

T12-L2 lateral syndesmophytes,

and interspinous ligament ossification

from the T12 to L1 and L2 to L4 levels.

Vertebral fusion

ankylosis of all cervical facet joints

from C2 downwards
Bamboo spine

complete fusion of the vertebral bodies.

Extensive facet joint ankylosis

posterior ligamentous ossification

joint space narrowing and

a collar of osteophytes , sacroiliac joint ankylosis

 PSORIATIC ARTHRITIS
 Psoriasis is a common skin disorder associated with joint
disease and characterized by peripheral joint destruction
and deformity: sacroilitis and nonmarginal syndesmophyte
formation
 Age 20 to 50 years; equal sex ratio
 Skin lesion characteristic, usually on extensor surface
(knees, elbows, back) also scalp, abdomen and genital
region. Lesions are well-defined, dry, raised, red and silvery.
 Severity and duration of skin disease show no relationship
to the onset of arthritis.
 Presence of nail changes seen in 80% of arthritis patients
 Arthritis is usually in peripheral joints especially DIP joints
RADIOLOGIC FEATURES OF PSORIATIC
ARTHRITIS
 General features include soft tissue swelling, normal bone
mineralization, erosions and tapered bone ends, prominent
justaarticular fluffy periostitis and joint space widening or bony
ankylosis.
 Hands and feet: asymmetric involvement, ray pattern, most commonly
involves DIP joints, no osteoporosis, mouse ears sign, widened joint
space due to fibrous tissue deposition and bone resorption, “pencil in
cup “ deformitry, opera glass hand deformity, no ulnar deviation
 Sacroiliac joint: involved in up to 50% of psoriatic arthritis patients
usually bilateral but asymmetric and unusual to be narrowed and
ankylosed.
 Spine: atlantoaxial subluxation and dislocation, normal apophyseal
joints, syndesmophytes of two types: nonmarginal (most common)
and marginal
fusion of the sacroiliac joints
cervical spine shows posterior

element fusion.
psoriatic rash and

sausage swelling on

the right second finger

 REITER’S SYNDROME
 A triad of urethritis, conjunctivitis and polyarthritis
 Affects males 50:1, 18 to 40 years of age
 Arthritis predominantly of lower extremity,
especially foot, calcaneus, ankle, knee, sacroiliac
and thoracolumbar spine, residual deformities if
chronic
 Bilateral conjunctivitis, non specific urethritis,
mucocutaneous lesions (keratodermia
belnorrhagicum)
 No organism isolated, inflammatory joint changes
 RADIOLOGIC FEATURES OF REITER’S
SYNDROME
 Swelling, osteoporosis, uniform loss of joint space, erosions, periostitis
 Specific target sites: forefoot, calcaneus, ankle, knee, sacroiliac, spine.
* Foot: metatarsophelantgeal,
interphalangeal joints
* Calcaneus: Plantar and achiller
insertions
* Ankle: Loss of joint space, swelling, periostitis
* sacroiliac: erosions, sclerosis, loss of joint margin,
asymmetric and often unilateral
* spine: thoracolumbar, asymmetric, skip non marginal
syndesmophytes and raely atlantoaxial instability
 GOUT ARHTRITIS
 Disorder of purine metabolism where hyperuricemia leads to
deposition of sodium monouratre crystals into cartilage,
synovium, periarticular and subcutaneous tissues
 These crystals evoke a strong inflammatory arthritis usually
in the lower extemity
 Affects males 20:1, usually in the fourth and fifth decades
 May be primary or less commonly secondary
 Four stages apparent: asymptomatic hiuperuricemia, acute
gouty arthritis (especially the first metatarsophalangeal
joint), polyarticular gouty arthritis (chronic, long-standing
disease) and chronic tophaceous gout (soft tissue
accumulations of sodium monurate)
RADIOLOGIC FEATURES OF GOUT ARTHRITIS
 General features include dense soft tissue tophi,
preservation of joint space, bone erosions (marginal,
periarticular, intraosseous, normal bone density, periosteal
new bone, secondary degenerative joint changes,
chondrocalcinosis dan avascular necrosis
 The most frequent targeted areas of involvement are the
first metatarsophalangeal joint, other metatarsophalangeal
joints, the hands dan wrists
 Spine and sacroiliac articulations show infrequent erosions.
Occasional epidural tophi occur leading to compression
myelopathy
chronic tophaceous gouty arthritis,

extensive bony erosions

Sclerosis and joint-space narrowing

in the first metatarsophalangeal joint