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REVIEW ARTICLE

Optic nerve gray crescent can confound neuroretinal rim interpretation: review of the literature

Sourabh Arora, MD, Jaspreet Rayat, MD, Karim F. Damji, MD, FRCSC

ABSTRACT RÉSUMÉ

The optic nerve gray crescent can be of clinical significance if unrecognized during assessment for glaucoma. It has a characteristic appearance of a slate gray area of pigmentation within the disc margins and commonly appears along the inferotemporal or temporal neuroretinal rim areas. This type of disc rim pigmentation can create the impression of neuroretinal rim thinning, and thus lead to the misdiagnosis of glaucoma or glaucoma suspectwith attendant implications for overtreatment or unnecessary close monitoring of such patients. The gray crescent is more common in African Americans than whites (prevalence rate 27% vs 7%) and is bilateral in at least 58% of cases. It has been reported in association with Kjer optic atrophy type 1. Suggested causes of the gray crescent include an accumulation of melanocytes, or retinal pigment epithelium cells partially located in the optic nerve head region if Bruch s membrane extends internal to the peripapillary scleral ring. Other causes of pigmentation that may resemble gray crescent are conus pigmentosus and variations of peripapillary atrophy. When a gray crescent is present, clinicians should endeavour to identify the true anatomical disc margins via the scleral lip and, if necessary, evaluate the patient further with imaging and visual field studies.

Le croissant gris dans la tête du nerf optique peut avoir une importance clinique s il n est pas reconnu pendant lévaluation du glaucome. Il a lapparence caractéristique dune zone de pigmentation grise-ardoise dans les marges de la papille et apparaît le plus souvent le long des zones de la jante neuro-rétinienne inférotemporales ou temporales. Ce type de pigmentation de la marge discale peut créer une impression d amincissement de la jante neuro-rétinienne et mener ainsi à un diagnostic erroné du glaucome ou de « soupçon de glaucome » avec implications systématiques de surtraitement ou de surveillance étroite non nécessaire de tels patients. Le croissant gris est plus fréquent chez les noirs (prévalence de 27 % vs 7 %) et bilatéral dans au moins 58 % des cas. Il a été signalé en association avec latrophie optique Kjer de type 1. Les suggestions détiologie concernant le croissant gris comprennent une accumulation de mélanocytes ou de cellules d 'épithélium pigmentaire de la rétine, située partiellement dans la région de la tête du nerf optique si la membrane Bruch s étend à lintérieur de lanneau scléral péripapillaire. Dautres causes de pigmentation qui peuvent ressembler au croissant gris sont le « cônus pigmentosus » et une variété datrophies péripapillaires. Lorsquun croissant gris est présent, les cliniciens peuvent sefforcer didentifier les véritables marges discales anatomiques par la lèvre sclérale et, si nécessaire, évaluer davantage le patient avec des imageries et des études du champ visuel.

Various features of the optic nerve head are commonly considered in the context of glaucoma, including measure- ment of disc size and evaluation of the neuroretinal rim for colour, as well as focal changes such as thinning, notching, sloping, cup-to-disc ratio, and disc hemorrhages. The gray crescent(GC) is an important and underrecognized phys- iologic variant rst described by Dr. Bruce Shields 1 in 1980; it can be dened as slate gray pigmentation on or within the neuroretinal rim. This type of disc rim pigmentation can create the impression of neuroretinal rim thinning, and thus lead to the misdiagnosis of glaucoma or glaucoma suspectwith attendant implications for overtreatment or unnecessary close monitoring of such patients. 2 , 3 We present a case of GC detected through our teleglaucoma program, and review the literature surrounding GC and select entities involving pigmentation within or around the disc margin.

C ASE R EPORT

A 37-year-old African male was referred from an optom- etrist for abnormal optic nerves.He had a spherical

From the Department of Ophthalmology, University of Alberta, Edmonton, Alta

Originally received Aug. 1, 2013. Final revision Jan. 27, 2014. Accepted Feb. 5, 2014

Correspondence to Karim Damji, FRCSC, Royal Alexandria Hospital, 2317, 10240 Kingsway Avenue, Edmonton AB T5H 3V9; kdamji@ualberta.ca

238 CAN J OPHTHALMOL —VOL. 49, NO. 3, JUNE 2014

equivalent refraction of 2.75 OD, 2.50 OS and best corrected vision of 20/30 OU. The IOP measured by noncontact tonometry was 13 mm Hg OD and 12 mm Hg OS. The vertical cup-to-disc ratio was 0.75 OU. No visual eld information was provided. Based on this referral information, particularly the nerve description, we believed

the patient could be at risk for glaucoma; thus, we assessed the patient through our hospital-based teleglaucoma system. 4 The patient had no ocular symptoms, prior ocular history, or family history of glaucoma, and was taking no ocular or systemic medications. Central corneal thick-

ness was 543 OD and 536 µ m OS. Slit-lamp examination

was unremarkable, with intraocular pressure (IOP) 16 OD

and 15 mm Hg OS by Goldmann applanation tonometry.

Stratus optical coherence tomography (OCT) was of high quality (signal strength ¼ 10 OU) and revealed average retinal nerve bre layer (RNFL) thickness of 113 m m OD

and

111 m m OS, with no focal RNFL thinning evident.

The

disc diameters were assessed as large by OCT: 2.41

mm

OD and 2.41 mm OS. Frequency doubling technol-

ogy eld testing was reliable and normal OU. Evaluation

Can J Ophthalmol 2014;49:238–242 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.

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Gray crescent: Review Arora et al.

Gray crescent: Review — Arora et al. Fig. 1 —A, OD with larger arrow pointing to

Fig. 1 —A, OD with larger arrow pointing to underestimated disc margin and smaller arrow marking the actual disc margin with associated gray crescent temporally. B, Another case with a gray crescent OD covering the temporal disc margin.

of stereoscopic digital fundus photos of the right optic nerve revealed a large disc, vertical cup-disc ratio of 0.7, and a temporal GC with some adjacent peripapillary atrophy (PPA; Fig. 1 ). The left nerve was also large, with vertical cup-to-disc ratio of 0.7 and mild inferior thinning noted. There was a more prominent temporal GC OS with some adjacent PPA. In light of the normal IOP, CCT, visual eld test, RNFL ndings on OCT, and GC temporally, the patient was thought to have physiologic rather than pathologic cupping, and hence the patient was considered unaffected for glaucoma. We recommended the patient continue to see an optometrist for annual evaluations, and if any concerns arose with regard to structure or function of the nerves, we could reassess.

D ISCUSSION AND L ITERATURE R EVIEW

Originally described by Shields 1 in 1980, the GC is an often underrecognized anatomic optic nerve variant. Relevant to this discussion is a de nition of the area of the optic nerve head, which Jonas 5 , 6 described as all areas inside the peripapillary scleral ring. Outside of the scleral ring is the peripapillary region, which is characterized by various other abnormalities discussed later. 7 The scleral ring is an anterior extension of sclera appearing as a thin white rim marking the disc margin. According to Shields, 1 , 2 the GC is characterized by increased pigmentation of slate gray colour within the substance of the optic nerve head. Jonsson et al. extended the de nition to the occurrence of a pigmented crescent that appeared to be located on or within the neuroretinal

rim tissue, that is, inside the scleral ring. 3 In some instances, the true disc border can be seen, although partially obscured by the GC ( Fig. 2 ). The clinical signicance of GC arises when it is mistaken for neuroretinal rim thinning and suspicious of glaucoma. Nonetheless, the diagnosis of glaucoma is often based on an integration of all available information including his- tory, IOP, nerve examination, imaging, and visual eld testing. In addition, taking a fundus photo to serve as a baseline may help for assessing progression/change. In the initial report by Shields, 1 a consecutive series of 29 patients with GC were described with a mean age of 35

of 29 patients with GC were described with a mean age of 35 Fig. 2 —Gray

Fig. 2 —Gray crescent OD with visible underlying disc margins.

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Gray crescent: Review Arora et al.

years and a relatively even split of males to females. However, 25 of the 29 patients were African American, whereas 4 were white. The 4 caucasian patients with GC all had dark complexions, and 1 had ocular melanosis. Thus, it was concluded that GC was more common in individuals with increased skin pigmentation. Thirty of the 58 eyes were myopic, with a mean spherical equivalent of 1.91. Four of these 29 patients were being treated for glaucoma, and 3 more were suspects. The GC was bilateral in 22 of the 29 patients. It was common for the crescent to be present in the temporal (24/51) or inferotemporal (21/ 51) regions. For large discs, the cup often touched the inner margin of the GC. The majority of these patients had been referred for assessment of glaucoma on the basis of elevated cup-to-disc ratio and elevated IOP. Impor- tantly, there was no corresponding visual eld loss in any of the eyes, even those in which there appeared to be complete cupping in a quadrant. 1 The Reykjavik Eye Study examined the epidemiology of GC among 1012 eyes in a white population within the age range most susceptible to glaucoma. 3 The GC was present in 22% of eyes examined. It was signi cantly more common in females (27% vs 17% in males), in hyperopic eyes, and in eyes w ithout PPA. Fifty-eight percent of cases were bilateral. It was associated with a large optic disc and was usually located in the temporal quadrant (37%), 360 degrees around the nerve (16%), or nasally (15%). It was rarely inferior (1.4%) or superior (1.4%). The vertical and hor izontal disc diameters were elevated in eyes with GC. The cup-to-disc ratio was also signi cantly increased. The occurrence of the GC was not associated with IOP elevation. There was no differ- ence in prevalence of GC in glaucomatous or non- glaucomatous eyes (OR 1.05). Although there appears to be a discrepancy in terms of characteristics of GC among whites versus African

Americans, a study by Higginbotham et al. 2 discussed this further. Consecutive patients of white or African American ethnicity were enrolled. Among African Americans, the incidence rate was 27%, whereas among whites it was 7%. There was no association with age, sex, refractive error, IOP, or glaucomatous optic neuropathy. The lower reported prevalence rate of the GC among white patients in this study compared with Jonsson et al.s study 3 was thought to be explained by the fact that Jonsson et al. included conus pigmentosus in their diagnosis of the GC, with the former being seen in the majority of their patients. There have also been reports of association between GCs and optic atrophy type 1 (OPA1, also known as Kjer type optic atrophy). 8 , 9 On ophthalmoscopic examination, OPA1 shows diffuse or temporal pallor of the optic discs, alongside occasional optic disc excavation. The neuro- retinal rim may also show some pallor, often associated with a temporal pigmentary GC. Votruba et al. noted a temporal GC in 31% of patients with OPA1. 10 Fournier et al. 11 studied patients with dominant optic atrophy and found all 9 patients had a GC. In 72% (13/18) of eyes, it was located temporally.

Differentiation from conus pigmentosus

Although the GC has been dened as an area of pigmentation on or within the neuroretinal rim, some ophthalmologists suggest a distinct second form of disc pigment, which has been termed conus pigmentosus of the optic disc 5 , 12 (Fig. 3A). The latter is described as an area of hypertrophy of the retinal pigment epithelium that covers the peripapillary scleral crescent and can enter the intra- papillary disc region (hypertrophy of retinal pigment epi- thelium closer to the surface of the disc). The authors of a recent study stated the appearance of conus pigmentosus is darker black, more irregular, and more supercial on the disc in comparison with a GC. 2 In their study, conus

disc in comparison with a GC. 2 In their study, conus Fig. 3 — A, OD

Fig. 3 — A, OD with conus pigmentosus superotemporally. B, OS with pigment outside the disc margin.

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Fig. 4 —OS with peripapillary atrophy. pigmentosus was found in 1 or both eyes of

Fig. 4 —OS with peripapillary atrophy.

pigmentosus was found in 1 or both eyes of 42 (54.5%) African Americans and 31 (20.9%) whites; the difference was statistically signicant (p o 0.0001). They also noted several patients had both types of disc pigmentation. We also identied a case of pigmentation outside the disc margin to contrast this with conus pigmentosum (Fig. 3B ).

Differentiation from peripapillary atrophy

PPA occurs when pigment and/or atrophy is seen outside the disc edge or peripheral to the scleral ring. 3 , 7 These areas are described as either zone beta (beside the optic nerve head) or zone alpha (away from or peripheral to zone beta). The alpha zone appears more prominent in the temporal disc margin than other quadrants. Because the alpha zone of PPA is also characterized by an irregular pigmentation, and because the boundary between the alpha zone and the surrounding tissue usually follows a semilunar line, partially parallel to the peripapillary scleral ring, one must be aware not to confound the GC with the alpha zone 5 , 6 (Fig. 4). In Higginbotham et al. s 2 study, 40 of 49 eyes (81.6%) with a GC also had zone alpha PPA just adjacent to the crescent or in the same quadrant. However, there was no correlation between the presence of zone beta PPA and GC. In a study by Jonsson et al., 3 PPA was found in 65% of their study population, and concomitant GC occurred in 19% of cases. There was a statistically signi cant inverse relation between circumferential extent of PPA and prevalence of GC. Lastly, for those without PPA, 30% had GC. Interestingly, the presence of GC is not associated with age or glaucoma, whereas PPA increases in prevalence with age and glaucoma. 5 In particular, beta PPA is thought to occur in 25% of normal eyes, and in a higher percentage of glaucomatous eyes. 7 Tezel et al. 13 , 14 demonstrated that presence and the size of PPA are related to the development of subsequent optic disc or visual eld damage in patients with ocular hypertension.

Cause of the gray crescent

Although no pathologic studies have been reported, Shields 1 suggested that the pigmentation may be an

Gray crescent: Review Arora et al.

accumulation of melanocytes, given the increased frequency among African Americans. According to Jonas, 6 because it is relatively dark, it may be associated with retinal pigment epithelium cells. These cells sitting on and forming Bruch s membrane may partially be located in the optic nerve head region if Bruchs membrane extends internally to the peripapillary scleral ring. 6 Recent research by Jonas et al. 15 , 16 identied the histologic importance of considering alpha, beta, gamma, and delta zones in understanding the cause of pigmentary disc changes and how they can be best classied. Based on high denition OCT studies, there is some support for the notion that Bruchs membrane can extend further into the nerve beyond the border of the disc that is identied clinically; whether this is the case with gray crescents remains to be determined. 17 The results of uorescein angiogram were similar in the 5 cases of GC studied. 1 Super cial vessels of the optic nerve head extended over the GC, whereas the deep uorescence of the optic nerve head was obscured to varying degrees by the crescent. This may indicate that the pigmentation is near the region of the lamina cribrosa. Higginbotham indicated that stereo examination of the optic nerve head may be useful in determining the level of disc pigment to distinguish between the GC and conus pigmentosus, with the latter having more super cial pigmentation. Also in the differential diagnosis of irregular pigmentation would be juxtapapillary choroiditis, choroi- dal degeneration, oblique disc insertion from myopia, melanosis, or melanoma. We presented a classic case of a GC in an African male, thought to be a glaucoma suspect based on cup-to-disc ratio but ultimately was a patient with physiologic cupping and unaffected by glaucoma. The GC can lead to overestimation of cupping if it is assumed that the underlying tissue is not neuror etinal rim but is peripapil- lary tissue. It typically appears as a slate gray area of pigmentation, commonly bilateral, along the temporal or inferotemporal disc margin s, and is distinct from conus pigmentosus and PPA.

Disclosure: The authors have no proprietary or commercial interest in any materials discussed in this article.

Acknowledgements : The authors acknowledge Dr. Jost Jonas, who reviewed the article and provided insights into histologic and anatomical aspects of the GC.

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Gray crescent: Review Arora et al.

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