22 Underconstriction or Undertubulation (Wide

Diametaphysis)

CLINICAL IMAGAGINGAN ATLAS OF DIFFERENTIAL DAIGNOSIS

EISENBERG

DR. Muhammad Bin Zulfiqar PGR-FCPS III SIMS/SHL

• Fig B 22-1 Gaucher's disease. The distal ends of the femurs show typical underconstriction and cortical thinning (Erlenmeyer flask appearance).33

• Fig B 22-2 Niemann-Pick disease. Thin cortices and a lack of normal modeling of the distal femurs simulate the pattern in Gaucher disease.4

• Fig B 22-3 Thalassemia. Pronounced widening of the medullary spaces with thinning of the cortices. Note the absence of normal modeling due to the pressure of the expanding marrow space. Localized radiolucencies simulating multiple osteolytic lesions represent tumorous collections of hyperplastic marrow.

• Fig B 22-4 Fibrous dysplasia. Smudgy, ground-glass appearance of the medullary cavities with failure of normal modeling.

• Fig B 22-5 Multiple exostoses. Bilateral involvement of the distal femurs and proximal tibias.

• Fig B 22-6 Metaphyseal dysplasia. Frontal views of (A) the knee and (B) the proximal humerus show defective modeling leading to extreme widening of the metaphyseal areas of the visualized long bones. The cortices are markedly thin in the metaphyseal area.34

• Fig B 22-7 Multiple enchondromatosis. Multiple globular and expansile lucent filling defects involving virtually all the metacarpals and the proximal and distal phalanges

• Fig B 22-8 Progressive diaphyseal dysplasia. Dense endosteal and periosteal cortical thickening causes fusiform enlargement of the midshaft of the femur.

• Fig B 22-9 Osteopetrosis. Dense, uniform, symmetric increase in the density of the femur with failure of proper modeling.

• Fig B 22-10 Healing rickets. Widening of the metacarpals associated with diffuse periosteal reaction. There is still some bony demineralization and residual cupping and fraying of the distal radius and ulna.