Neurofibromatosis, left ptosis, cutaneous neurofibromas, plexiform neurofibroma . Brain: There is left sphenoid wing dysplasia with a defect in the left lateral orbital wall and associated expansion and protrusion of the middle cranial fossa into the left orbit, prominence of the extra-axial cerebrospinal fluid spaces overlying the left temporal lobe, and 5 mm of midline shift towards the left. There are punctate foci of high T2 signal in the subcortical white matter of the inferior left frontal lobe and midbrain posterior to the left red nucleus. There is no evidence of intracranial hemorrhage, mass, or acute infarct. The optic chiasm appears to be intact. The ventricles are not dilated. There appears to be an anomalous and enlarged proximal right opthalmic artery, which projects into the right sphenoid sinus. Orbits: There is an infiltrative heterogeneously enhancing mass in the left eyelid with apparent extension into the lacrimal gland and superior extraconal region, which measures approximately 10 x 35 mm in cross-section. In addition, there is diffuse enhancement measuring up to 2 mm in thickness along the lateral extraconal region of the left orbit, extending posteriorly to the orbital apex and into the superior orbital fissure. There are several additional nodular enhancing lesions in the skin and subcutaneous tissues of the left lateral periorbital and temporal regions, as well as overlying the left masseter muscle and parotid gland. There is left proptosis, compression of the left optic nerve, and deformity of the lateral rectus muscle associated with the sphenoid wing dysplasia and protruding intracranial contents, but no discernible tumor within the optic nerve. In addition, there is enlargement of the left globe diffuse, compatible with buphthalmos. The right orbit appears unremarkable. There is a subcentimeter enhancing focus in the right sublingual space.
1. A left eyelid plexiform neurofibroma measures up to approximately 35 mm and appears to infiltrate the lacrimal gland and superior extraconal space and what may represent additional tumor measuring up to 2 mm in thickness along the lateral extraconal region of the left orbit extending posteriorly to the orbital apex and into the superior orbital fissure.2. Several other left facial and scalp lesions are compatible with peripheral nerve sheath tumors as well.3. Left sphenoid wing dysplasia with proptosis associated with expansion of the middle cranial fossa and prominence of the subarachnoid spaces.4. Mild left buphthalmos.5. Apparent anomalous and enlarged proximal right opthalmic artery, which projects into the right sphenoid sinus and may in part represent vascular dysplasia, possibly with fusiform aneurysm. Dedicated angiographic imaging may be useful for further evaluation.6. A subcentimeter enhancing lesion in the right sublingual space may represent a neurofibroma.7. Nonspecific punctate foci of high T2 signal in the subcortical white matter of the inferior left frontal lobe and midbrain posterior to the left red nucleus may represent stigmata of neurofibromatosis type 1, such as "FASI".8. During the study the patient experienced a mild contrast reaction manifested by cough, pruritis, and nasal congestion, but no shortness of breath and symptoms resolved in few minutes. No treatment was administered. The patient was discharged in stable condition and instructed to seek medical care should symptoms recur.