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AJNR - American Journal of Neuroradiology

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Disappearing Calvarium in Gorham Disease: MR Imaging Characteristics with Pathologic Correlation

Chung-Ping Lo^a, Cheng-Yu Chen^a, Shy-Chyi Chin^a, Chun-Jung Juan^a, Chun-Jen Hsueh^a and Ann Chen^b

^a Department of Radiology, Tri-Service General Hospital and National Defense Medical Center, Taipei, Taiwan, Republic of China
^b Department of Pathology, Tri-Service General Hospital and National Defense Medical Center, Taipei, Taiwan, Republic of China

View larger version (114K): [in a new window]   FIG 1. Lateral-projection conventional radiograph of the skull shows a large, sharply demarcated defect over the left parietal bone.

View larger version (93K): [in a new window]   FIG 2. A, Axial spin-echo T1-weighted (TR/TE/NEX: 402/12.1/3) and B, T2-weighted (4275/96/3) MR images show a focal scalp depression with loss of subjacent calvarial marrow signal intensity (arrowheads). C and D, Axial and coronal contrast-enhanced fat-saturated T1-weighted images obtained at the margins of the skull defect show reticular-pattern of enhancement.

View larger version (70K): [in a new window]   FIG 3. 3D reconstructed CT scan obtained by using a bone algorithm viewed from the left lateral delineates well the large skull defect. FIG 4. Tc-99 m MDP bone scintigraphy reveals no bone uptake in the resorbed site (arrow) but increased uptake around the margin (arrowheads). FIG 5. Photomicrograph of the bone specimen shows the small capillary-like vessels (arrows) and spindle-shaped fibroblasts (arrowheads) (hematoxylin and eosin stain, x 400)