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Bony Hamartoma of the Inferior Orbital Rim in a Patient With Tuberous Sclerosis
Arch Ophthalmol. 2004;122:780-782.
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Named by Bourneville, one of its earliest discoverers, tuberous sclerosis is a heritable neurocutaneous syndrome that is classically manifested by the Vogt triad of mental deficiency, epilepsy, and adenoma sebaceum.1 Despite the classic triad of findings, tuberous sclerosis is a protean disorder characterized by the presence of hamartomas (benign neoplasms composed of cellular elements normally present in tissue) in multiple organ systems, including the brain, kidneys, heart, spleen, lungs, and eye.2-3 Osseous involvement is known to occur in the skull, long bones, pelvis, and metacarpal and metatarsal bones. We report a case of tuberous sclerosis that manifested as a bony mass arising from the inferior (maxillary bone) orbital rim. Histopathologic examination of the excised lesion revealed a hamartoma composed of mature bone. Based on our MEDLINE review of the English-language literature, this is the first reported case of a bony hamartoma arising from this location of the facial skeleton in . . . [Full Text of this Article]Report of Case.
Comment.
Ari Abel, MD;
David T. Brockbank, MS;
Martha Farber, MD;
Dale R. Meyer, MD
Albany, NY
Corresponding author: Dale R. Meyer, MD, Lions Eye Institute of Albany Medical Center, 35 Hackett Blvd, Albany, NY 12208.
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