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Risk?
There is a higher risk of DON in men, and in older patients of either gender. The mean age at presentation of DON is 56–57 years [13, 27]whereas for GO without DON it is 49 years [21, 27]. Other risk factors include diabetes mellitus which constitutes an additional 10-fold risk for DON [74]. Smoking is associated with a greater risk of more severe orbitopathy and may confer a higher risk for DON.
What Other Assessments Are Useful in Evaluating Possible Dysthyroid Optic Neuropathy?
In addition to the clinical assessments described in the section “How Do You Decide whether a Patient Has Dysthyroid Optic Neuropathy?”, several ancillary tests can also help to identify DON. These include visual-evoked potentials, contrast sensitivity and imaging.
Abnormalities in both latency and amplitude of visual-evoked potentials can be supportive in the diagnosis of DON; however, several issues affect their value in practice. Firstly they can be affected by thyroid dysfunction, which is frequently present at the time when DON develops [13]. In addition, many laboratories have no normal data for patients over 60 years old, making it hard to interpret findings in those who are already the most difficult to diagnose due to confounding pathology [4]. Hence, they should be interpreted with caution in patients with no other evidence of DON.
Contrast sensitivity, which measures spatial resolution at all levels of contrast, appears to be a sensitive indicator of DON [73]; however, it is less readily available and still subject to confounding pathologies.
Imaging has a very valuable place in supporting the diagnosis of DON. Coronal images on CT or MRI demonstrate apical crowding in the majority of patients. This is defined as the effacement of perineural orbital fat in the posterior orbit. The combination of apical crowding with evidence of fat herniation through the superior orbital fissure seen on axial images is thought to have a specificity of 91% and a sensitivity of 94% for DON [75].
In another study, radiological criteria which were significantly predictive of DON were apical crowding (p < 0.001) and medial rectus volume (p = 0.005) or diameter (p = 0.003), whereas proptosis and orbital angle (p = 0.895) were not [76]. Nugent et al. [77] noted mild or no apical crowding in 17% of DON patients, while 13% had severe apical crowding but no clinical evidence of DON. More recently, the objective quantification of apical orbital crowding based on square area measurements was shown to be a more efficient diagnostic tool than subjective quantification. In this study, the best-performing index was highly efficient at detecting DON, with a sensitivity of 91.7% and a specificity of 90.0% [78]. However, as with evoked potentials and contrast sensitivity, DON should not be diagnosed from imaging alone. Stretching of the optic nerve is less easy to diagnose without both axial and sagittal images: the latter are not generally available with CT.
Should These Tests Be Performed in All Patients at Every Assessment?
It is not necessary to perform any of these additional tests in all patients at every assessment. They can be very valuable when there are features suspicious for DON as described in “How Do You Decide whether a Patient Has Dysthyroid Optic Neuropathy?”, and, if abnormal, they may be useful for monitoring the response to treatment.
Are There Any Other Assessment Systems in Common Use?
The VISA system was developed a decade ago and is used predominantly in North America. The mnemonic stands for vision, inflammation, strabismus and appearance/exposure. The protocol for assessment of both inflammatory features and strabismus is less detailed than that of EUGOGO, and this may influence its relevance to scientific studies. However, its main use is in clinical patient management, and comparison of grading between 2 experienced observers showed good correlation relevant to clinical management decisions [79]. Both VISA and EUGOGO systems provide not only a diagnostic classification, but also an assessment with practical implications for guiding the management of patients, something valuable compared to the NOSPECS classification [68].
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