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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 27:274-277, February 2006
© 2006 American Society of Neuroradiology

CASE REPORT
HEAD AND NECK

Oromaxillofacial Osseous Abnormality in Sturge-Weber Syndrome: Case Report and Review of the Literature

D.D.M. Lin^a, P. Gailloud^a, E.F. McCarthy^b and A.M. Comi^c

^a Division of Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, Md
^b Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Md
^c Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Md

Address correspondence to Doris D.M. Lin, Division of Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD 21287

SUMMARY: We report a case of a 17-month-old child affected by Sturge-Weber syndrome who had unusually rapid overgrowth of the left frontal, temporal, orbital, and maxillary regions. CT angiography illustrated osteohypertrophy with periostitis and associated soft tissue hypertrophy directly corresponding to the distribution of the cutaneous port-wine stain. Extended maxillectomy was performed because of rapid growth and clinical debilitation, with surgical pathology revealing juvenile ossifying fibroma.