CT of infantile myofibromatosis of the orbit with intracranial involvement: a case report.

An infant boy presented at birth with a large mass protruding from the left orbit that significantly displaced the left globe laterally and inferiorly . Biopsy of the mass was performed the next day, yielding the diagnosis of infantile myofibromatosis. Therapy was considered but the parents opted for no treatment, although they agreed to yearly CT follow-up examinations. The CT study at the time of birth showed an enhancing 5.2 x 5.4 em mass centered over the left orbit (Fig . 1 A). The left globe was deformed and displaced inferiorly and laterally by the mass. The mass entered the ethmoid region through the lamina papyracea, the sellar region through the optic foramen, and the middle cranial fossa through the greater wing of the sphenoid. The mass also showed intracranial extension into the suprasellar region and anterior cranial fossa (Fig . 1B). CT examinations performed at yearly intervals showed significant regression of the mass. The study at the age of 2 years revealed residual enhancing tumor surrounding the left globe but predominantly contained within the left orbit (Fig. 2). The globe had regained a normal position. By the age of 4 years the tumor showed minimal remaining mass with only a thin rim of enhancement (Fig. 3). Review on bone windows revealed interval regrowth of a portion of the posterior and medial orbital walls with residual defects in the greater sphenoid wing and ethmoid . Sclerosis and cortical thickening of the ethmoid had an appearance mimicking meningioma.