Oromaxillofacial osseous abnormality in Sturge-Weber syndrome: case report and review of the literature.
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.
Division of Neuroradiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
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Lin
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Gailloud
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McCarthy
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D003937:Diagnosis, Differential; D005146:Facial Asymmetry / Q000175:diagnosis* / Q000601:surgery; D018214:Fibroma, Ossifying / Q000175:diagnosis / Q000601:surgery; D005624:Frontal Bone / Q000473:pathology / Q000601:surgery; D006801:Humans; D006984:Hypertrophy; D007091:Image Processing, Computer-Assisted; D021621:Imaging, Three-Dimensional; D007223:Infant; D008297:Male; D008437:Maxilla / Q000473:pathology / Q000601:surgery; D008441:Maxillary Neoplasms / Q000175:diagnosis / Q000601:surgery; D019767:Maxillofacial Abnormalities / Q000175:diagnosis* / Q000601:surgery; D009056:Mouth Abnormalities / Q000175:diagnosis*; D009915:Orbit / Q000473:pathology / Q000601:surgery; D012888:Skull Neoplasms / Q000175:diagnosis / Q000601:surgery; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000601:surgery; D013701:Temporal Bone / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed; D015050:Zygoma / Q000473:pathology / Q000601:surgery