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Comment. Tuberous sclerosis, otherwise known as Bourneville disease, is an autosomal dominant syndrome with an incidence of 1 case per 6000 to 29900 people per year. Despite its capability of following classic Mendelian patterns, approximately two thirds of cases are sporadic and seem to arise from new somatic mutations. Genetic research has implicated mutations at 2 gene loci, 9q34 (TSC1) and 16p13 (TSC2), in the pathogenesis of tuberous sclerosis. These genes are thought to be tumor suppressors. Historically, the clinical criteria for diagnosing tuberous sclerosis include facial adenoma sebaceum, mental deficiency, seizures, and retinal astrocytomas (hamartomas), although not all of the criteria need to be met to make the diagnosis. In addition to skin and retina, hamartomas have been identified throughout the body in locations such as the iris, brain, bones, kidneys, lungs, heart, and tongue. Bony abnormalities in patients with tuberous sclerosis have been previously described in the skull, long bones, pelvis, and metacarpal and metatarsal bones, with the hands and feet the most commonly involved. These lesions can be focal or diffuse; tend to be round, oval, or flame shaped; and range in size from a few millimeters to several centimeters. These bony lesions typically manifest as small cortical cysts or areas of dense periosteal sclerosis, the result of fibrous replacement of the normal trabecular bone pattern, and can produce an undulation in the bony contour. Bony abnormalities can be found in the cranium in up to 45% of patients with tuberous sclerosis. Radiographs of the skull may reveal sclerotic areas of dense bone formation, often due to hyperostosis of the diploë trabeculae of 1 or both tables of the cranial vault. The calvaria is thinned, marrow spaces are replaced by fat, and the periosteum is thickened. After an extensive review of the peer-reviewed English-language literature using MEDLINE, we found only 1 report describing an osseous abnormality of the facial skeleton and orbit in a patient with tuberous sclerosis and none with histopathologic confirmation of a bony hamartoma in this location. Breningstall et al reported a case of a sclerotic lesion of the sphenoid bone in a 9-year-old girl with tuberous sclerosis who had left-sided proptosis. Skull radiographs and computed tomography revealed marked sclerosis and thickening of the left wing of the sphenoid bone, which was attributed to fibrous dysplasia. However, no histopathologic analysis was provided. To our knowledge, this case of a large bony hamartoma arising from the inferior orbital rim in a patient with tuberous sclerosis provides the first clinicopathologic confirmation of a bony hamartomatous lesion involving the facial skeleton in the setting of tuberous sclerosis. Physicians treating patients with tuberous sclerosis should be aware of this presentation.

Total Opacification of Intraocular Lens Implant After UncomplicatedCataract Surgery: A Case Series—Correction