Key points Definition: Pathologic entity caused by abnormal hypertrophy of the ocular hyaloid system. Classic clue: Child presenting at or soon after birth with leukocoria, micropthalmia, fluid-fluid level, no calcifications, and persistent Cloquet’s canal is virtually pathognomonic. In normal embryologic…
Key points Definition: A coloboma is caused by a rare congenital defect with failure of the embryonic intraocular fissure to fuse during the first trimester of fetal development resulting in an elongated or malformed globe. Colobomas may arise in any…
Key points Definition: Rhabdomyosarcoma (RMS) is a relatively rare sarcoma, frequently found in children aged between 1 and 5 years. Classic clue: A 5-year-old old child is referred by a pediatrician with a history of possible recent trauma, developing rapid…
Key points Definition: Esthesioneuroblastoma (ENB) is a rare neuroendocrine malignancy of neural crest origin arising from olfactory epithelium near the cribriform plate and the nearby nasal cavity. ENB may invade the orbit, causing ophthalmic signs and symptoms. Synonym: Olfactory neuroblastoma.…
Key points Definition: Isolated metastasis to the optic nerve is rare and is often overlooked or misdiagnosed initially. Classic clue: Typically, a middle-aged female presents with “a little blurred vision,” further inquiry identifies pain, proptosis, reduced visual acuity, and bitemporal…
Key points Definition: Leukemia presenting as an orbital mass portends a more serious prognosis as these patients continue to show significantly lower survival rates than other leukemia patients. Synonym: A subset of orbital leukemia includes: granulocytic sarcoma, chloroma, or myeloid…
Key points Definition: Retinoblastoma (RB) is a rapidly developing intraocular cancer arising from immature retinal cells. Classic clue: A calcified posterior globe mass in an infant or young child is an RB until proved otherwise. RB is the most common…
Key points Definition : Ocular melanoma is the most common intraocular malignancy in adults. Synonyms : Orbital melanoma, uveal melanoma, choroidal melanoma. Classic clue: A “collar button” enhancing intraocular mass in adults. Computed tomography (CT) shows: Ocular melanoma is hyperdense.…
Key points Definition: Ocular adnexal lymphoma (OAL) is one of the most common orbital tumors in adults, accounting for 50% of orbital malignancies. Synonym: Orbital lymphoma (OL). Classic clue: An older patient presenting with an orbital mass causing progressive exophthalmos,…
Key points Definition: Usually found in middle-aged adults, orbital schwannomas (OS) are well-defined, encapsulated, slowly growing benign tumors arising from peripheral nerve Schwann cells, and comprise 1% to 2% of orbital tumors. Classic clue: Middle-aged patient presenting with optic neuropathy,…