Caffe Disease nama lainnya Infantile Corticale hyperostosis

1. Caffey Disease
Infatile Cortical Hyperostosis

2. Nama Lain
• Infantile cortical hyperostosis
Imaging
1. Asymmetric, polyostotic cortical hyperostosis
• Mandible meliputi > 80% cases
• Paling banyak: ribs, clavicles, scapula, skull, ilium
• Diaphyses, ± metaphyses, jarang pada epiphyses
• Berupa periostitis yaitu bergabung dalam korteks tulang dan bowing
• Dapat bertahan beberapa tahun, disertai dengan remodelling

3. Top Differential Diagnoses
1. Prostaglandin therapy for congenital heart disease
2. Metastatic disease: Neuroblastoma, Ewing sarcoma
3. Osteomyelitis: Variable distribution of process
4.Trauma: Accidental and nonaccidental

4. Variable distribution ± metaphyseal corner fx
• Physiologic: Asymptomatic, bilateral, resolves by 6 months of age
• Pathology
Autosomal dominant transmission reported
1.Acute:
Inflammation in periosteum ± soft tissues
Periosteal new bone ± cortical resorption
2.Subacute stage:
Periostitis ossifies, incorporated into cortical bone

5. • Clinical Issues
 Triad: Fever, soft tissue swelling, hyperirritability
 < 5 months of age; may occur in utero
 M = F; no race predilection
 Most commonly self-limited condition
 Resolves without sequelae after 6-9 months
Rarely protracted course with multiple relapses

8. OSTEOPETROSIS
Terminology
• Albers-Schonberg disease
• Marble bone disease
Imaging
• Berhubungan dengan Hiperostosis dan sklerosis, distribusi di skull dan spine.
• Undertubulation of metaphyses
• Bone-in-bone appearance
• "Rugger jersey" or "sandwich" vertebrae
• MR: "Black bone"
• Bone scan: Superscan

9. Top Differential Diagnoses
1.Pycnodysostosis
• Short stature, acroosteolysis, obtuse mandibular angle, delayed
closure of sutures
2.Progressive diaphyseal dysplasia
• Irregular cortical and endosteal thickening; primarily diaphysis,
spares epiphyses

10. Pathology
• Abnormal osteoclast function creates imbalance between bone
formation and resorption
Genetics
1. Infantile type: Autosomal recessive
2. Adult types: Autosomal dominant
3. Intermediate type: Autosomal recessive
4. Sly disease: Autosomal recessive

11. • Clinical Issues
1. Infantile type: Failure to thrive, marrow failure
2. Adult types: Often incidentally discovered
3. Intermediate type: Variable
4. Carbonic anhydrase type II deficiency (Sly disease)
5. Cerebral calcifications, renal tubular acidosis

14. LEGG-CALVE-PERTHES
Terminology
Legg-Calve-Perthes (LCP)
Definitions
• Osteonecrosis of femoral head epiphysis during childhood
Imaging
1. Early radiographic findings
a. Effusion and lateral subluxation of femoral head
b. Fragmentation of femoral capital epiphysis
c. Sclerosis and flattening of epiphysis
2. Mid-term radiographic findings, if progresses
a. Lateral extrusion of portion of femoral head
b. Metaphyseal irregularity, "cystic" changes

15. 3.Late radiographic findings with severe progression
a.Coxa plana deformity, I acetabular congruence
b.Coxa magna deformity (short broad head/neck)
c. Growth disturbance (25% have premature physeal closure, 90%
show decreased growth resulting in limb length discrepancy)

16. • MR:
Early (avascular or necrotic) phase
1. Low or intermediate T1 signal epiphysis
2. Variable SI on T2WI/STIR: May see curvilinear low
3. SI or high SI edema
4. Partial or complete nonenhancement (normal hip shows early and
rapid enhancement)

17. Revascularization and reparative phases
 Heterogeneous epiphyseal signal on T1WI, T2WI/ STIR
Revascularized areas of epiphysis show t SI on T2WI/STIR and
enhancement (even h y pere n h a n cem e n t )
Morphologic epiphyseal abnormalities
 Abnormal; physeal enhancement 2° to presence of abnormal
transphyseal blood vessels
Early bony bridging (physeal bar)
 Metaphyseal abnormalities corresponding to "cysts" seen on
radiograph (cartilage, fibrosis

18. • Morphologic epiphyseal abnormalities
■ C oxa plana, fragmentation
■ Lateral femoral head subluxation, I containm ent by acetabulum
o Physeal involvement
■ Irregularity of growth plate
■ Abnormal enhancem ent 2° to presence of abnormal
transphyseal blood vessels
■ Early bony bridging (physeal bar)
■ Cystic change

19. • Metaphyseal involvement
■ Abnormalities corresponding to "cysts" seen onradiograph
- May consist of physeal cartilage extensions
- Some contain granulation tissue, fibrosis, or fat necrosis
■ Morphologic changes of coxa magna (short, broad Neck)

20. DIFFERENTIAL DIAGNOSIS
1. Immune-Mediated and Viral (Toxic) Synovitis
• Self-limiting acute synovitis (3-10 days)
• Boys < 4 years old (generally younger than LCP)
• Significant effusion; no epiphyseal abnormality

21. 2. Septic Hip
• Acutely ill with fever
• Increased white blood cell count + sedimentation rate
• Hips held in flexion, abduction + external rotation vs.nhip adduction in LCP
• Joint effusion ± joint debris ± reactive marrow edema
3.Juvenile Idiopathic Arthritis (JIA)
• Epiphyseal crenulation or erosions may mimic LCP
• Chronic disease: Valgus hip, gracile femoral shaft, hypoplastic iliac wing
• Thigh muscle hypoplasia
4.Juvenile Osteonecrosis
• Distinction: Identifiable etiology of osteonecrosis
• Sickle cell, less likely thalassemia
• Idiopathic thrombocytopenia purpura
• Hip dislocation

22. 5.Epiphyseal Dysplasias
• Not isolated to femoral capital epiphysis
6.Hypothyroidism and Cretinism
• "Cretinoid" hip: Fragmented, small femoral capital epiphysis
• Significant delay in skeletal maturation

31. OSTEONECROSIS OF HIP
• Synonyms
Ischemic necrosis, osteonecrosis (ON), aseptic necrosis, avascular necrosis (AVN)
• Definitions
Necrosis of cellular elements of bone 2° to ischemia
Demographics
• Age : 3rd through 6th decades
• Gender: M:F = 4:1
• Epidemiology
1 5 ,0 0 0 cases hip ON in USA per year
Steroids responsible for 3 0 -4 0 % of nontraum atic ON
Alcoholism responsible for 2 0 -4 0 %
10% of hip arthroplasties performed for ON

32. •General Features
Best diagnostic clue
1 Radiograph
■ Early: Patchy sclerosis in femoral head
■ Late: Irregularity, fragmentation, collapse of femoral head articular surface
2. MR: Double line sign
Location
o Anterior weight-bearing femoral head early
Size
Size of infarct quite variable, ranges from small focus to involvement of entire femoral head
■ Assess extent of disease using visual inspection
Morphology
o Factors associated with articular surface collapse: Size of infarct, lateral location within
head

33. Imaging Reco m m en d a tio n s
• Best imaging tool
° MR is most sensitive and specific
• Protocol advice
° Any MR protocol for hip pain should include at least 1 sequence
that images opposite hip
■ Aids detection of asymptomatic disease
■ T1WI &/or STIR coronal ideal
o MR imaging: Use coronal & sagittal planes to fully demonstrate
extent; small FOV of each hip best

34. • Radiographic Findings
• Early: Patchy sclerosis of femoral head due to new bone formation along
necrotic trabecula
• Advanced findings
Crescentic subchondral lucency indicative of fracture, may precede articular
surface collapse
■ Frog lateral or false profile lateral views show best
■ Orientation parallels articular surface
■ Limits surgical options
Articular surface collapse
■ May be subtle, requires close inspection, visible cortical break may not be
evident
■ Often easier to appreciate on radiographs vs.
MR
° Articular surface fragmentation
• 2° osteoarthritis (OA): Joint space narrowing, acetabular subchondral
sclerosis, osteophytes; femoral & acetabular articular surfaces incongruent

35. CT Findings
• Bone CT
• Osteoporosis and distortion of bony "asterisk" of femoral head
trabeculae
• Sagittal & coronal (to lesser degree 3D reformats) useful to visualize
articular surface collapse
• Most sensitive modality for subchondral fracture o
• Overall not as sensitive (55%) or accurate as MR

36. • MR Findings
• MR is 97% sensitive, 98% specific for ON
• Initial MR findings: Nonspecific bone marrow edema
• menurunnya T1W signal & meningkat signal on fluid-sensitive sequences
• May extend from femoral head into femoral neck
During 1st few months following infarct, infarcted bone will appear normal; MR changes do not occur until healing has begun
• o Stages within infarcted bone progress from normal marrow -*• hemorrhage -*• edema -*■ fibrosis
■ T1WI: Bright marrow -* hypointense -* dark
■ T2WI: Hypointense marrow -*■ bright -*• dark
Pathognomonic finding: Double line sign
o Low SI line at periphery of infarct with bright inner ine forming reactive interface with infarcted bone
• MR ability to detect subtle articular surface collapse poorer than radiographs; often easier to appreciate on sagittal images, least
apparent on axial images
o Crescentic fracture may not be visible, does not always precede collapse
• Associated edema extending from infarct into head/ neck correlates with pain, risk of collapse
o Edema found in 48% of patients with ON
o 72% of cases with edema occur in Steinberg stage III disease (ON with subchondral lucency)
o May presage collapse of head & suggest latest point where core decompression may be efficacious
• Joint effusion: i T1W signal, t T2W signal (at any stage)
• T1 C+: Decreased enhancement in early ON; later, absent enhancement of nonviable segments

37. Differential Diagnosa
1Bone Marrow Edema Pattern
• Extensive differential diagnosis, including transient osteoporosis of
hip, infection, neoplasm; may require time before definitive diagnosis
becomes apparent
2.Insufficiency Fracture of Femoral Head
• Patient population: Elderly, osteoporotic women
• ± significant articular surface collapse, fragmentation
• Does not develop double line sign
• Usually absent risk factors for ON

38. • General Features
• • Etiology
1. Post-traumatic: Disrupted blood supply
• ■ Hip dislocation: If not reduced within 12 hours, 50% develop ON
• ■ Subcapital fracture: 30% of displaced femoral fractures develop
ON
2.Corticosteroid use: Enlargement of intramedullary fat cells and
marrow pressure inhibits blood flow

39. 3. Other etiologies
■ Sickle cell anemia: Sickled cells thrombose in microvasculature at
low oxygen tension
■ Gaucher disease: Marrow packing -*■ t pressure
■ Systemic lupus erythematosus (SLE): Vasculitis + steroids; 5-40%
develop ON
■ Caisson disease: Nitrogen air embolization from dysbaric
phenomena
■ Radiation: Vasculitis results in osteonecrosis
■ HIV/AIDS: May relate to antiretroviral therapy or hyperlipidemia
■ Alcohol abuse: Likely due to fat emboli from liv

40. Staging, Grading, & Classification
Steinberg classification: Based on radiographic appearance & location
of lesion
• Stage 0: Normal radiographs, MR, & bone scan of at- risk hip (often
contralateral hip involved, or patient has risk factors & hip pain)
• Stage I: N1 radiograph, abnormal bone scan/MR
• Stage II: Cystic or sclerotic radiographic changes
• Stage III: Subchondral lucency or crescent sign
• Stage IV: Flattening of femoral head
• Stage V: Joint space narrowing
• Stage VI: Advanced degenerative disease

41. Other signs/symptoms
• Effusion especially with collapse contributes to pain
• A traumatic ON com m only bilateral (3 0 -7 0 % at time of
presentation) but progresses asymmetrically
• 1 /3 of asymptomatic contralateral hips will go on to pain,
collapse; with cystic changes, more likely to progress to collapse
• Contralateral asymptomatic disease in 60%
• SCFE (slipped capital femoral epiphysis) and DDH (developmental
dysplasia hip) both at risk for ON
• Average of 4 years between onset of symptoms between the hips