대한근골격영상의학회

Title : Case 741

Age / Sex : 48 / F Chief complaint : 무릎 통증, 보행장애, 지적장애, 청력 감소 가족력: (+) What is your diagnosis? Two weeks later, you can see the final diagonosis with a brief discussion of this case (Please submit only one answer). Courtesy : 지충근, Choong Guen Chee, 서울아산병원, Asan Medical Center Diagnosis: Camurati-Engelmann disease Discussion Disease definition: Autosomal dominant (TGF-B1), progressive diaphyseal dyspalsia Findings Bilateral, symmetrical cortical thickening of the diaphysis (periosteal and endosteal sides) Location: Tibia > femur > femur > fibula > humerus > ulna > radius Also affect mid-segment of the clavicle, calvaria, mandible, facial bone (intra-membranous ossification) In severe cases, affects skull base and cranial nerve palsies may develo Differential diagnosis(보내주신 감별진단만 semi-correct로 인정 합니다) 1.Ribbing disease 2.Erdheim-Chester disease References RadioGraphics 2011; 31:1865–1882

Title : Case 741

Age / Sex : 48 / F

Chief complaint : 무릎 통증, 보행장애, 지적장애, 청력 감소

가족력: (+)

What is your diagnosis?

Two weeks later, you can see the final diagonosis with a brief discussion of this case (Please submit only one answer).

Courtesy : 지충근, Choong Guen Chee, 서울아산병원, Asan Medical Center

Diagnosis:

Camurati-Engelmann disease

Disease definition: Autosomal dominant (TGF-B1), progressive diaphyseal dyspalsia

Findings

Bilateral, symmetrical cortical thickening of the diaphysis (periosteal and endosteal sides)

Also affect mid-segment of the clavicle, calvaria, mandible, facial bone (intra-membranous ossification)

Differential diagnosis(보내주신 감별진단만 semi-correct로 인정 합니다)

1.Ribbing disease

2.Erdheim-Chester disease

References
RadioGraphics 2011; 31:1865–1882

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