Superior Segment Hypoplasia (Topless Disc Syndrome)

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This condition may be mistaken for glaucoma due to the inferior arcuate visual field defect and RNFL thinning on OCT. On exam there is no cupping of the optic nerve (usually), and the superior half of the optic nerve is missing or sometimes looks “lopped off,” leading to the name “topless disc syndrome.” The key question that should be asked in the medical history is if the patient’s mother has diabetes mellitus. Unlike glaucoma, this condition does not need treatment.