Glaucoma without cupping

I S S U E H I G H L I G H T

Glaucoma without cupping

Jerome Sherman, O.D.,a,b Sherry J. Bass,a O.D., and Samantha Slotnick, O.Dma

=State University of New York, State College of Optometry and bThe Eye Institute and Laser Center, New York, New York

Background: Although glaucoma can exist wi th normal intraocular pressures (IOPs), clinicians still rely on the presence of a large cup to "flag" suspects, regardless of IOP. whereas a small cup at the same pressure level is often ignored. High-tech instruments offer a new dimension of evaluation in the objective assessment of structure when subjective tests of function and/or ophthalmoscopic observations are equivocal.

Case Reports: Thirteen cases are presented and show evidence of glaucoma based on glaucomatous visual-field defects, often wi th steadily rising intraocular pressures and retinal nerve fiber layer loss. Surprisingly, these patients maintained small C/O ratios. Accordingly, ophthalmoscopy and/or disk topography classified these disks as normal.

Conclusions: Although unrecognized in virtually the entire world's ophthalmic literature, normal cupglaucoma is a real clinical entity. At least half the normal cup glaucoma cases presented herein have disk drusen (obvious, subtle, or occult), while others are highly myopic and/or have documented IOP spikes. Several of the cases defy classification and explanation at the present time.

Key Words: Case reports, cupping, GDx, glaucoma, HRT, intraocular pressure, ocular hypertension, OCT, optic nerve head

T he misdiagnosis of glaucoma has visual as well as emotional, financial, and litigious consequences. Cup- ping of the optic nerve head and high intraocular pres-

sure have always been classic hallmarks of glaucoma and are frequently used to make the diagnosis of this disease. However, the presence of an elevated intraocular pressure is no longer considered an absolute harbinger of g1aucoma.l Certainly, glaucoma exists in patients with normal intraocular pressures, and glaucoma never develops in the major- ity of patients with ocular hypertensi~n.~ However, cup size, shape, and depth continue to alarm the eye care practitioner. Most clinicians feel that a "big" cup is a bad sign, while a "small" cup is safe.

The ophthalmoscopic observation of whether a cup is phys- iologically large or glaucomatous is sometimes difficult and takes on even greater significance when there may be other inconclusive signs of disease. This decision must be pred- icated on a careful and comprehensive evaluation of the optic nerve head. Such an evaluation is an integral part of the diagnosis and management of glaucoma, and there are many optic nerve head characteristics that must be examined carefully. However, many practitioners will admit that nothing commands more suspicion than a large, deep cup. For many practitioners, cup size drives decisions that deter- mine diagnosis of glaucomatous disease and can direct care.

Clinicians have witnessed the birth of a multitude of high- tech diagnostic instruments that were developed to quantify structural aspects of the optic nerve, retina, and retinal nerve fiber layer (RNFL).3 Some of these instruments include

Sherman J, Bass S, Slotnick S, Glaucoma without cupping. cla~~ification~ based on normal and glaucomatous databases Optometty z004;75:677-708. in which the statistical analysis indicates a category into

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V O L U M E 7 5 l N U M B E R 1 1 1 N O V E M B E R 2004 O P T O M E T R Y

ISSUE HIGHLIGHT

which the patient falls, thereby having the potential to aid clinicians in the assessment of an abnormality at an initial visit and in the determination of change over time, the hallmark of glaucoma. In addition, these tests, which are objective, have added a whole new dimension to glaucoma detection by removing the variability factors associated with subjective testing. However, the b Case 1

determination of cupping- Filure 1 ,

whether determined subjectively by stereo photography or objectively by some of the higher-tech instruments-can be misleading.

This article provides a retrospective analysis of exemplary cases. As such, no specific protocol was adhered to, and not every patient had every available test. In several cases, the patients were followed for more than a decade and the diagnosis was made before the availability of many of the high-tech devices. Thus, procedures such as the Heidelberg Retinal Tomograph (HRT) (HRT-Heidelberg Engi- neering, Inc., Carlsbad, California), GDx (Laser Diag- nostic Technology, San Diego, California), and corneal pachyrnetry were not obtained in some cases. But all of the patients had at least the "routine" glaucoma evaluation, including Goldmann tonometry, automated visual fields, gonioscopy, and fundus photography. A few of the patients were seen by nearly a dozen clinicians over a period of many years, and individual bias with regards to the orig- inal diagnostic workup is certainly possible. The authors may have chosen to treat some patients earlier and more aggressively, but the outcome of different therapeutic approaches will never be known. In the final analysis, the lack of a specific protocol for all patients and the possible bias on the part of select clinicians does not alter the results.

Included here are 13 cases that demonstrate the false sense of security that a small cup affords.

All the following cases, except Case 6 (KK) and Case 12 (PE), are patients who were evaluated at either the SUNY State College of Optometry Glau- coma Institute or at the Eye Institute and Laser Center in Manhattan. (Case 6 has not been evaluated by any of the authors, but one of us US] was asked to evaluate all available data by the patient's son, an optometrist.) The following cases were chosen because each case supports the concept

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OPTOMETRY

. Fundus photographs of the optic disks (A, right eye; B, left eye) demonstrate 10 ratios and optic disk drusen in each eye.

that glaucoma can exist without appreciable cupping. The first several cases have observable surface disk drusen with glaucoma and are included to sensitize the reader to the results of fields, HRT, and GDx in such cases. Select cases of subtle and even occult disk drusen are then explored, as well as normal cup glaucoma in high myopia andlor in patients with IOP spikes. We have included some cases of glaucoma without cupping in this article that defy classification.

In order to confirm the diagnosis of glaucoma, several modern technological innovations are used. The nature of each technology is discussed briefly as it applies in each case. A previously published article reviews these technologies in greater detail.4

Case Reports Case 1 (WG): Exfoliative glaucoma without cupping and with obvious optic disk drusen in a white man An 80-year-old white man, with no family history of glaucoma, was followed for two years as an ocular hypertensive based on elevated intraocular pressures (IOPs) that ranged from 20 to 26 mmHg in each eye. Visual acuities were 20120 OU and biomicroscopy revealed mild exfoliation on the lens surface in both eyes. Gonioscopy revealed open angles OU. Cupldisk ratios were noted to be 0.1 OU and there were both ophthalmoscopic and ultrasonographic evidence of optic disk drusen OU (see Figure 1).

Visual field testing had initially revealed nasal and inferior field loss in both eyes, thought to be due to either optic disk drusen or perhaps primary open-angle glaucoma (POAG). These field defects were slowly progressive over time (see Figure 2).

VOLUME 75lNUMBER 1 l/NOVEMBER 2004


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[ i ~ e 2 Case l. Visual fields reveal nasal and inferior loss in both eyes. The superior nasal defect in the right eye may have progressed.

Figure 3 Case I. A more recent HRT II still fails to reveal a cup in the O.D. and demontrates only a very small cup in the O.S. The Moorfields regression analysis is normal in both eyes.

V O L U M E 7 5 f N U M B E R 1 I I N O V E M B E R 2004 O P T O M E T R Y


WO years after his initial visit, the patient was diagnosed as having POAG because of the increasing IOPs, the observation of exfoliative material (which, arguably, could change the diagnosis to exfoliative glaucoma), and significant ongoing field loss. Treatment was initiated in both eyes. Disk topography, as assessed with the Heidelberg Retinal Tomograph was classified as highly normal OU, with Clas- sification scores of + 17.78 O.D. and + 8.47 O.S. (a classification score of + 1 to -1 is classified as borderline, c -1 is glaucomatous, and > + 1 is normal).

A more recent disk topography was obtained with the HRT I1 and remains normal and unchanged (see Figure 3). Note the absence of a central red zone in the image in the upper left. In these color disk topographic images, the cup is depicted as red, the disk as green, and the transition zone as blue. Thus, the HRT demonstrates no cup in the right eye and a very small cup in the left eye.

In contrast, GDx (Laser Diag- nostic Technology, San Diego, [i lure 4 Case 1. GDx rwealssignificant nerve fiber layer loss in both eyes, left greater than right.

California) retinal nerve fiber layer (RNFL) analysis measure- ments revealed significantly attenuated nerve fiber layers in both eyes (see Figure 4). A normal RNFL is thickest above and below the disk5 and is depicted in the color plot as bright colors. In this case, the superior and inferior RNFL are quite thin and are shown as more blue than red, especially in the left eye. In the 'NERVE FIBER ANALYSIS' caser wnous pnotograpny reveals minimal cupping in each eye (A, right eye: B, left eye). (bottom third of GDx printout], Figure 5 normal findings are in green, borderline findings in yellow, and abnormal find- ber" below 30 is considered normal, 30 to 50 is ings in red. The neural net number or "the Num- borderline, and over 50 is considered abnormal). ber" is a computer-generated index of overall The Numbers in this case are grossly abnormal, abnormality that varies from 1 to 100 (a "Num- with 76 O.D. and 93 O.S.

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A lure 6 Case2. The visual field reveals a nasal loss in the right eye, while the left field is normal,

In patients who demonstrate field loss in the presence of both disk drusen and high IOPs (and other risk factors, such as exfoliation), it is often not possible to attribute the field loss to either disk drusen or to glaucoma. Many clinicians con- clude that the field loss is due to both di~orders.~ With profound NFL loss (such as depicted with the GDx in this case), treatment to lower the elevated IOPs is indicated.

The diagnostic profile presents ophthalmoscopic observations of no cups and lumpy disk borders, high pressures, RNFL-type visual-field defects, topographic absence of cupping, and objective evidence of RNFL reduction. These factors support the diag- noses of disk drusen and glaucoma.

sented initially at age 55, her visual acuity was 20120 in each eye, external examination was unremarkable, IOPs measured by applanation tonometry were 18 mmHg O.D. and O.S., and her cupldisk ratio was estimated as being 0.15 OU. Visual-field testing did not reveal any defects in either eye. During the course of the 10-year follow-up examinations, intraocular pressures were noted to gradually increase. Over this same course of time, cupping remained stable and visual fields remained near-normal. At one of the later visits, IOPs were noted as being 30 mmHg in both the O.D. and O.S., and on a follow-up visit, hit a high of 31 mmHg, at which time treatment was initiated. At that time, her cupldisk ratios were noted as being 0.25 in each eye, and no other disk anomalies or peripapillary changes were noted (see Figure 5).

Case 2 (EG): Glaucoma without cupping with subtle disk drusen in a black woman A recent white-on-white perimetry revealed

nasal loss in the right eye only (see Figure 6 ) A 67-year-old black woman was followed for over and a short-wavelength automated perimetry a decade because of a strong family history of (SWAP) visual-field test was noted as being bor- glaucoma (both mother and sister). When she pre- derline.

VOLUME 75lNUMBER 111NOVEMBER 2004 OPTOMETRY

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fiu~re 7 Case2 HRT confirms normal cupping O D . and O S

Optic nerve head parameters were quantified by HRT, which revealed essentially no cupping and normal disk parameters in each eye. Both disks were somewhat larger than normal size (normal is 2.25 mm2), at 2.6 mm2 O.D. and 2.7 mm2 O.S. The overall classification on the HRT was "normal," with a +9.0 composite score O.D. (high probability of being normal), and a + 3.6 composite score in the left eye (see Figure 7).

The ranked segment analysis (RSA) curves for the parameters of rimldisk ratio, rimldisk area ratio, rim volume, and RNFL were also in the normal range for each eye. RNFL analysis was performed using scanning laser polarimetry with the GDx. Numerous statistically significant abnormalities were noted in various parameters in both eyes, and the overall neural net "Numbers" were 57 in the right eye and 60 in the left eye. These results were indicative of significant nerve fiber layer attenuation in both eyes.

At this point, treatment was initiated. The patient failed to return for two years. At the next visit, IOPs were elevated to 35 and 34 mmHg, and the patient admitted to noncompliance. B-scan ultra-

sonography failed to reveal conclusive disk drusen.

A GDx with Variable Corneal Compensator (VCC) with the 2003 software package demonstrated moderate RNFL loss in both eyes with a Nerve Fiber Indicator (NFI) of 43 and 48 (see Figure 8). (In the new version of the GDx with VCC, the Number has been replaced with the NFI and is based on a new database. The NFI separates normal from abnormal RNFLs, in which NFI > 30 is a strong indication of a RNFL defect associated with glaucoma, and NFIe 30 is probably normal.)

Based on the strong family history-the escalating IOPs with noncompliance, the questionable increase in CID ratio (from 0.15 to 0.25), the recent nasal visual-field loss in the right eye, and the grossly abnormal RNFL demonstrated on the GDx-it was concluded that this patient probably has primary open-angle glaucoma without the appearance of substantial cupping. Subse- quent white-on-white visual-field testing has still failed to demonstrate any consistent field loss in the left eye, while the nasal loss in the right eye persists.

OPTOMETRY VOLUME 75lNUMBER 111NOVEMBER 2004


com~arison to the size of the disk: Re-evaluation of the disk photographs revealed slightly lumpy disk borders, highly suggestive of disk drusen, most marked inferiorly in each eye. We believe this patient has subtle disk drusen that went unde- tected on earlier examinations. A more recent B-scan ultrasono- gram failed to detect disk drusen, probably because the drusen were not yet calcified in this case.

Case 3 (DS): Glaucoma without cupping in a black man A 75-year-old black man first presented for a routine eye examination at age 61. At that time, health history included mild systemic hypertension and diabetes, with a questionable family history of glaucoma. Examination of patient records over a 14-year period revealed a slight increase in cupldisk ratios from 0.2 O.D. and 0.3 O.S. to perhaps 0.25 O.D. and 0.35 O.S. (see Figure 9), but early disk photographs were never obtained. The right cup appears to be slightly larger horizontally than

A l l r e l c.1.2. t iU i w ~ t h Var~able Corneal bmpensator reveals ret~nal nerve tlber layer loss W I ~ vertically, whereas the opposite elevated Nerve Fiber lnd~cators (NFls) 0.0. and O.S. is observed in the left eye. A

Fi l l re 1 Case 3. Fundus photographs of the optic disks (A, right eye; B, left eye) demonstrate small C/D ratios in each eye with the left cup somewhat larger vertically. Note the peripapillary pigmentary changes that are suggestive of glaucoma.

not appear to be present.

Intraocular pressures during this time period increased from the high teens to the low 30s in each eye, at which time treatment was instituted. Field loss was noted on both Humphrey and Octopus perimeters O.S., with diffuse depression noted O.D. on Octopus perimetry only (see Figure 10).

As a general rule, patients with large disks have large physiological cups. This case is unusual in At a recent visit, the IOPs were 30 mmHg that the degree of cupping was quite small in O.D. and 28 mmHg O.S., despite the patient's

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Fium 11 Case 3. HRT demonstrates a minimally positive classification (normal) in the 0.0. and a minimally negative classification (glaucoma) in the left eye. However, a "Classification" between t1.00 and -1.00 is generally considered to be borderline.

insistence on compliance with medication. -0.94 O.S. Each of the four ranked segment HRT revealed a classification of "borderline," analysis curves were classified as normal (see with a composite score of + 0.94, O.D. and Figure 11).

O P T O M E T R Y V O L U M E 7 5 l N U M B E R 1 1 1 N O V E M B E R 2004

ISSUE H I G H L I G H T

Case 3. GDx reveals significant retinal newe fiber layer loss in each eye with a neural net number of 70 O.D. and 76 O.S. Note that both the Fiuure 12 . ~nfer~or . and superior sectors are shaded in the left 'Deviation from Normal' plot in the left eye and thus are significant.

demonstrated on the GDx, the diagnosis of POAG without cupping was supported. Fundus evaluation, fields, HRT, and GDx all indicate slightly more glaucomatous damage in the left eye than in the right eye. Although the cupldisk ratios increased slightly over a decade and a half, the crux of glaucoma, the present C/D ratio is stil l small relative to the mean in the black popu- lation. A reassessment of the disk Auure 13 " 4. Fundusphotographs of the optic disks (A, right eye; B, left eye) reveal small C/D photographs failed to reveal any

ratlos (0.2 0.0. and 0.3 O.S.) in each eye. evidence of disk drusen. How- ever, peripapillary changes-per-

A GDx was also performed. Numerous parame- haps glaucoma-related-were present in both ters in each eye were noted as being abnormal eyes. with statistical significance. The Number was 70 in the right eye &d 76 in the left eye, both grossly 4 m: GIaCIl d f l U t apping abnormal (see Figure 12). with subtle optic disk dtusen in a whi i man Based on the possible family history of glaucohm, A 56-year-old white man with no a family history the slow increase in IOPs from the high teens to of glaucoma was followed as a glaucoma suspect the low 30s, the mild increase in cupping, the due to IOPs near 30 mmHg in both eyes on sev- field loss documented numerous times on two dif- eral occasions. Cupldisk ratios were recorded as ferent perimeters, and the attenuated RNFL as 0.2 O.D. and 0.3 O.S. (see Figure 13).

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V O L U M E 7 5 l N U M B E R 111NOVEMBER 2004 OPTOMETRY

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White-on-white visual fields within the central 30 degrees were normal in each eye, but threshold fields between 30 and 60 degrees were reported as abnormal. SWAP visual fields on two separate occasions demonstrated mild field loss in each eye. The managing clinician decided not to treat at the time because of the small cups and questionable field defects.

During a family visit, the patient was examined by his optometrist son. Because of the elevated IOPs and equivocal field results, the son decided to obtain a GDx. There was an astonishing loss of RNFL thickness in each eye and most parameters were abnormal, with a high degree of statistical significance. The Num- bers were 90 O.D. and 86 O.S. (see Figure 14).

As a consultant in this case, one of us US) recommended treatment. Copies of the disk photographs were also requested and reviewed. Subtle disk drusen, as evidenced by a slightly lumpy appearance of the disk margins, were observed, but never confirmed by B-scan ultrasonography. This subtle disk observation has been encoun- tered in about a dozen other glaucoma patients and suspects. With treatment. IOPs have since decreased to the low-teens. Fiuure 14 Case 4. GDx reveals significant retinal nerve fiber layer (RNFL) loss in both eyes with a

neural net number of 90 O.D. and 86 O.S., highly indicative of significant RNFL loss.

This case documents a profound loss of the RNFL in the absence of both pathological cupping and field loss with central (30-degree) white-on-white perimetry. Since this patient was seen by several doctors and fundus photographs were not obtained by the treating physician, the subjective assessment of C/D ratio at each visit cannot be confirmed. According to b h- tographs reveal small C/D rat~os in each eye (A, right eye; the literature' an estimated 4% to AUure id , I& eye]. NO& the slightly lumpy disk border nasally 0 s . 11% of glaucoma patients demon-

686

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fiuure 16 k 5 . Visual fields demonstrate absolute nasal loss O D and superonasal loss 0s .

strate field defects outside of 30 degrees before defects within 30 degree^.^,^ SWAP may detect some field loss associated with the RNFL loss, but the patient only had two SWAP fields performed. Some experts suggest that only a third SWAP be used for diagnostic purposes, since many normal individu- als perform poorly on the first, and even the second SWAP field.lOJ1 Given the repeated episodes of elevated IOPs in the absence of any other optic nerve head anomaly that could explain the profound loss in the RNFL, this is probably a case of POAG without cupping in the presence of subtle disk drusen, which can cause field loss.12 Careful observations of the optic nerve head may allow the clinician to identify subtle disk drusen that perhaps are initially missed.

Case 5 (MZ): Glaucoma without cupping in a young white woman

b.i.d. OU. The patient reported a long-standing history of poor vision in the right eye, previously diagnosed as amblyopia. However, she also believed the vision in her right eye was progressively deteriorating. Entering best-corrected visual acuities (VAs) were Hand Motion at 6 ft. O.D. and 20120 O.S. A right constant esotropia was evident, with a mild right relative afferent pupillary defect. Intraocular pressures were 26 mmHg O.D. and 35 mmHg O.S., but the patient claimed she was compliant with her treatment of eye drops. Gonioscopy revealed open angles in all quadrants OU, and some anomalous vessels in the angle, which were believed to be con- genital. Fundus evaluation revealed CID ratios of 0.3 in each eye, with distinct borders and normal pink rim tissue (see Figure 15).

No findings of diabetic or other retinopathy were observed, but mild vascular tortuosity was noted. Visual fields were at tem~ted on several

A 31-year-old white woman, with a 10-year history occasions with erratic, unreliable results, but the of Type I1 diabetes and a family history of glau- last visual field appeared acceptable. It revealed coma, presented for a second opinion on a previ- an absolute nasal hemianopic loss and relative ous diagnosis of glaucoma, made one month earlier. temporal loss in the right eye and an absolute The referring doctor had prescribed Betoptic 0.5% superior nasal loss in the left eye (see Figure 16).


I S S U E HIGHLIGHT

HRT demonstrated normal optic nerve head topography O.S., with classification within normal limits (see Figure 17). Several attempts at obtaining an HRT from the right eye failed as a con- sequence of the poor fixation associated with the very reduced visual acuity.

I Case Mi?

Due to the reported progressively deteriorating vision in the right eye, a magnetic resonance image (MRI] of the anterior visual pathway was ordered, which was negative. A consulting neurologist found no evidence of a non-glaucomatous optic neuropathy and was of the opinion that the field loss must be due to glaucoma alone. A consulting glaucoma specialist agreed with the diagnosis of glaucoma OU, but had no explanation for the normal-appear- ing disks. The deteriorating vision in the amblyopic eye was probably due to the longstanding glaucoma, despite the absence of cupping. An alpha-agonist (Bri- monidine] was added to the treatment regimen because of the consistently elevated intraocular pressures on monotherapy. Fiuure 17 Case 5. HRT confirms normal disk topography O.S. and cupping within normal limits.

(Because of poor visual acuity and fixation 0.0.. an acceptable HRT image could not be

Although we are convinced this obtained.)

patient has glaucoma (based in part on IOPs as high as 35 mmHg, even while under treatment), we will continue to monitor for a contributing non-glaucomatous optic neuropathy, which we have not been able to detect as of this writing. This case of glaucoma without cupping is somewhat unusual in that an absolute nasal defect is present in each eye and yet no abnormal cupping is detectable ophthalmoscopically or with disk topography. occult disk drusen, as this three-dimensional R ~ r e 18 Case 5. Three-dimensional image of the left optic disk,

HRT rendering suggests (see Figure 18), provide a rendered by HRT, suggests nasal disk drusen (arrow).

possible explanation for this lack of cupping. But the disk drusen, if present, are nasal, and therefore do revealed mild ocular hypertension in the left eye

not correspond to the nasal field defect. (19 mmHg O.D., 22 mmHg O.S.). Health history was positive for a mild stroke at age 53, which caused weakness on one side of his body (patient

Case 6 (HS): Glaucoma without cupping in a white man ms,e of which side,, with full resolution aHer A 78-year-old white man, whose brother and two 3 years. Visual acuities were corrected to 20120 in sisters have glaucoma, first became a glaucoma sus- each eye. Pupils were normal. Cupldisk ratios were pect at age 68, when a routine eye examination small (0.3 OU). Baseline 30-degree threshold white-



Fiuure 11 Case 6. Visual fields early on reveal superior Bjerrum depressions in both eyes; in later fields, O.S. defect deteriorates, deepens superiorly. and spreads to the inferior field. Increased field loss O.S. was accompanied by development of tAPD O.S.

ated. The patient failed to return as requested, until 4 years later. The degree and nature of the cupping remained unchanged; IOPs remained mildy elevated (20 mmHg O.D., 23 mmHg O.S.). Gonioscopy revealed angles open to the ciliary body in both eyes, with no peripheral anterior synechiae and a mild degree of pigmenta-

Filure 20 Case 6. Fundus photographs demonstrate small C/D ratios (0.3) in each eye tion. Visual fields again (A, right eye; 6, left eye). revealed superior Bjerrum

depressions in both eyes. on-white visual fields revealed bilateral superior Because the degree of cupping was small and Bjerrum depressions in both eyes (see Figure 19). stable from earlier visits, no diagnosis of glau-

coma was made at this visit. The patient was A follow-up field 1 month later failed to show told to return in 2 months for a morning IOP consistent results, and no treatment was initi- check.

6 8 9

V O L U M E 7 5 l N U M B E R 1 I I N O V E M B E R 2004 O P T O M E T R Y


The patient was again lost to followup and returned on his own 2 years later, claiming that an examination elsewhere 2 weeks earlier had revealed elevated intraocular pressures. At this visit, visual acuities were still correctable to 20120 OU, but now an afferent pupillary defect was noted (grade 2 + ) in the left eye. IOPs measured 25 mmHg O.D. and 32 mmHg O.S. Cup- ping was round and cupldisk ratios were stable at 0.3 in each eye; neuro-retinal rims were ophthalmoscopically normal. Threshold visual fields at that visit revealed evidence of a superior depression OU, with no progression from previous fields. Because the IOPs were on the rise, the patient was treated. Because of the history of a mild stroke, the patient was referred to a neurologist who obtained blood tests, including an ESR, which were normal. Duplex Doppler scan of the carotids revealed a moderate stenosis of the right carotid. Bilateral flow was seen in the vertebral arter- ies of the neck, but velocity was Fi!ure 21 Case 6. GDx reveals significant retinal nerve fiber layer loss in both eyes, wi th neural

greater on the right side com- net numbers of 65 0.0. and 74 O.S. (The macula scan, not shown, failed to demonstrate

pared to the left. Transcranial any residual corneal birefringence.)

Doppler was recorded as normal. MRIs of the brain and orbits, with and without abnormal in each eye and revealed a neural net contrast, were unremarkable. number of 65 O.D. and 74 O.S. (see Figure 21).

The patient was followed over the next few years with no change in cupping. Intraocular pressures were maintained in the high teens with treatment. Over the following 2 years, however, visual-field defects were noted to progress O.S., despite the fact that cupping remained small. The positive APD persisted and was believed to be attributa- ble to glaucoma, since that eye had a larger degree of field loss. However, cupldisk ratios remained equal and stable with horizontal cupping (see Fig- ure 20).

The patient's medication was changed from Tim- optic b.i.d. OU to Alphagan b.i.d., O.S. only. Scan- ning laser polarimetry (GDx) was grossly

A Retinal Thickness Analyzer (RTA) was done on the O.S. only and also objectively documented retinal thinning superiorly. Alphagan was then instituted in the right eye as well, and Xalatan was added to the left eye because the IOP was climbing into the low 20's O.S. Because of the small degree of cupping, a B-scan ultrasound was performed for evidence of disk drusen, but disk drusen were not observed. If disk drusen are calcified, B-scan ultrasonography will typically pick them up because calcium is a strong reflector of sound. However, in our clinical experience, B- scan ultrasonography cannot typically detect uncalcified disk drusen. An HRT was performed to quantify disk topography parameters.

690

O P T O M E T R Y V O L U M E 7 5 l N U M B E R 1 I I N O V E M B E R 2004

ISSUE HIGHLIGHT

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Figure 22 Case 6. HRT II reveals normal disk parameters in each eye. (Note that the right eye image is inferior in quality to the left eye image in that the Topographic Standard Deviation is much greater.)

L Figure 23 Case 7. Normal optic nerve heads with a myopic fundus appearance (A, right eye:

B, left eye).

Composite scores of -0.26 O.D. and + 0.84 O.S. were assigned, suggesting a "borderline" classification of normal. The ranked segment analysis curves were all classified as normal in both eyes. A recent HRT I1 was also obtained; it revealed 6 of 6 normal disk zones in each eye and the Moorefield's regression analysis was normal OU (see Figure 22).

The subjective and objective evidence of normal cupping, along with subjective (visual field) and objective evidence of RNFL attenuation (GDx and RTA), as well as IOPs documented at 32 mmHg, all support the diagnosis of glaucoma without cupping.

Case 7 (MS): Early glaucoma without cupping with central acuity loss A 66-year-old white woman in

good health presented with symptoms of vision reduction in her right eye, first noticed 6 months before this visit. She claimed she had previously sought the services of a retinal specialist, who examined her retina and performed fluorescein angiography and Optical Coherence Tomography (OCT) . Since all testing performed by the retinal specialist was found to be normal,

VOLUME 75lNUMBER 111NOVEMBER 2004 OPTOMETRY


Figure 24 Case 7. A, The 24-2 visual field (on the lefl reveals a general reduction in sensitivity in the right eye, but is essentially normal; B, the 10-degree Humphrey visual field (on the righQ reveals a central superonasal depression in the right eye 2 degrees from fixation

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Case 7. Octopus 10Q visual fields reveal central depressions superior and superonasal to fixation 0.0. (right side). The O.S. visual field (left side) Figure 25 IS normal.

O P T O M E T R Y V O L U M E 7 5 l N U M B E R 1 I I N O V E M B E R 2 0 0 4

ISSUE HIGHLIGHT

I However, because of the reduced I visual acuity O.D., a 10-degree

dl* c m a visual-field test using a

I / Humphrey perimeter, in which points are tested 2 degrees apart, was administered. Testing revealed a central depression superonasal to fixation in the

, right eye (see Figure 24, B) and .- I no depression in the left eye.

/ A 10-degree field test of the right

Case 7. The rnacular scotorna on the Octopus loP visual field test Figure 26 bass of the OCT, which demonstrates normal rnacular structure.

he could not find an explanation for the loss of vision. A subsequent visit to a neuro-ophthalmologist also failed to elucidate an etiology for the reduced acuity. The neuro-ophthalmologist then referred the patient to one of us (JS) for Visual Evoked Potential (VEP) testing and RNFL analysis. At the present visit, she claimed her vision remained unchanged for the past half year. Best- corrected visual acuity through a -9.00 D sphere O.D. was 20170- and through a -7.00 sphere O.S. was 20130. No improvement with a pinhole was noted. Examination revealed mild nuclear and posterior cataracts in both eyes, with a small PSC slightly greater in the right eye. Angles were open by gonioscopy with grade 3 + pigment in all four quadrants. Goldmann tonometry revealed IOPs of 34 mmHg O.D. and 32 mmHg O.S. Fundoscopy revealed normal optic nerve heads, with CID ratios of 0.3 O.D. and 0.2 O.S. (see Figure 23).

Large areas of peripapillary atrophy were evident in both eyes, as well as retinal thinning in the posterior poles of both eyes. Central 24-degree visual fields revealed a general reduction in sensitivity, but were essentially normal (see Figure 24, A).

eye using the Octopus perimeter, in which points are tested 0.7

1 1 degrees a&rt within the central ( 4 degrees, revealed a dense sco-

tom; radiating both superior (and slightly temporal) and superonasal from fixation (see

Visually evoked potential testing, which was performed at the request of the referring neuro-

is not explained on the' ophthalmologist, revealed reduced amplitudes in both eyes; the responses from the right eye

were reduced by 50% compared to the left eye. The previously performed OCTs were reviewed for evidence of a macular hole; optic sections through the macula O.D. were normal (see Fig- ure 26).

Scanning laser tomography performed with the HRT I1 classified the O.D. nerve as normal and the O.S. nerve as borderline, with a possible thinning of the nasal rim (see Figure 27).

Based on the IOPs measured at this most recent examination, a diagnosis of primary open-angle glaucoma was justifiable, and it was concluded that the reduction in vision in the right eye was probably due to glaucoma, since macular involvement was ruled out. The patient admitted she had been told her intraocular pressures were "quite high" in past examinations, but she was never treated for glaucoma, possibly because of an absence of cupping. Although central field loss along with reduced visual acuity is not common in early chronic open-angle glaucoma, it has been reported.13 The patient was diagnosed with glaucoma and treatment was initiated to lower her IOPs. As documented by this and the

VOLUME 75fNUMBER 11fNOVEMBER 2004 OPTOMETRY

I S S U E HIGHLIGHT

Figure 27 Case 7. Normal optic nerve head appearance 0.0. and borderline appearance O.S. with HRT II, despite glaucomatous intraocular pressure and O.D. central field loss.

Figure 28 Case 8. A, Goldmann visual fields performed in the left eye were initially full years earlier; 6, Goldmann visual fields of the left eye 10 years later reveal a classic inferior nasal step originally attributed to "myopic degeneration" (right figure).

6 9 4

O P T O M E T R Y V O L U M E 7 5 l N U M B E R 1 I I N O V E M B E R 2 0 0 4


29 Case 8. A, Func 6, a fluorescein wi th virtuallv no

lus photography reveals a C/D ratio of no more than 0.1 in the left eye; performed in 2004 reveals a relatively well-perfused optic nerve head cuo and no moss retinal abnormalitv.

vision. He reported bumping into people and objects not only in dim envi- ronments, but also occasionally in well-lit rooms.

He had a history of a traumatic retinal detachment in his teens in the right eye with no light per- ception (NLP); best-corrected visual acuity in the left eye through a contact lens was 20150 O.S. A Goldmann visual field of the left eye, performed in the mid-1980's in order for the patient to obtain a driver's license, had been normal (see Fig- ure 28, A). The man was referred in the mid-1990's for consulta- tion because of increasing diffi- culty with both day and night

Filure 30 Case 8. A Dicon QMP field from 0 to 60 degrees. Note the numerous zero-decibel threshold levels. Central visual acuity O.S. probably remains because of several less- depressed points (white check marks) superior-nasal to fixation.

following case, glaucoma may be under- diagnosed in high myopes. The myopic disk is often indistinguishable from the myopic, glaucomatous disk, and, in our clinical experience, IOP spikes in high myopes are not uncommon.

Case 8 (OT): Advanced glaucoma without cupping in a 32-diopter myope A 32-year-old Hispanic man with an extremely high degree of myopia ( > -30 D) was followed for 15 years to monitor his myopic degeneration.

Best-corrected visual acuity th;ough a -30 D contact lens was still 20150 in the left eye. Intraocu- lar pressure in the left eye was in the mid-teens. Electroretinograms (ERGs) were obtained to rule out an overall retinal degeneration such as retini- tis pigmentosa sine pigmento. The ERGs were in the low normal range, commensurate with extremely high myopia.

Over the following year, the IOP in the left eye increased to the low 20's. Evaluation of the left fundus revealed posterior pole thinning consistent with myopic degeneration, a large conus temporal to the optic nerve head, and a cup-to-disk ratio

I I of 0.1 in the left eye without pallor (see Figure 29, A). A 3-mirror lens examination also failed to reveal any significant cupping. Goldmann visual- field testing of the left eye at that time revealed a large inferior field loss with a nasal step (see Fig- ure 28, B), but the field loss was thought to be due to "myopic degeneration" and not glaucoma, perhaps because of only mildly elevated IOPs at the time and the lack of glaucomatous cupping. Since the earlier Goldmann visual-field test was normal, the development of an inferior nasal step documented progressive loss. One year later, the VA dropped to 20180 in the left eye. The IOP in that eye was still 20 mmHg and the cupping remained unchanged. An automated visual field at that visit was performed using the Dicon QMP #11 pro- gram (see Figure 30). As with the Goldmann visual field, an inferior nasal step was demonstrated. Overall, the field appeared to worsen, but it is difficult to compare a Goldmann kinetic field with an automated static field.

695

V O L U M E 7 5 / N U M B E R 1 1 1 N O V E M B E R 2004 O P T O M E T R Y

ISSUE HIGHLIGHT

One month later, the patient presented with another drop in central visual acuity O.S. (201100) and intraocular pressures of 42 mmHg O.D. and36 mmHg O.S., which remained in the 30's over subsequent visits. Gonioscopy again revealed open angles and the patient was started on treatment, despite the clinical finding of a 0.1 CID in the left eye. A CT scan, ordered to rule out other b Filure 31 Case 9. Fundus photography reveals CID ratios of 0.2 in each eye (A, right eye;

non-glaucomatous optic neu- B, left eye).

ropathies, was normal (an MRI was not ordered due to insurance issues). Over IOPs during this time ranged from the high- the next several years of followup, however, his teens to the mid-20's. Cup-to-disk ratios had IOP O.S. remained in the low- to mid-teens and always been recorded as 0.1 in each eye and 30- his CID ratio remained unchanged in the left eye. degree threshold visual fields were always nor- In such a case as this, using hi-tech instruments mal. After the 13th year of followup, he was such as HRT, OCT, GDx, and RTA may not be submitted as a potential Ocular Hypertensive helpful, because there are no normative databases Treatment Study (OHTS) subject, but was dis- with this refractive error for any of these hi-tech missed from the study based on the finding of instruments; in addition, fixation may be a prob- subtle visual-field defects that were "diagnostic" lem in patients with reduced acuity. of glaucoma. Returning to our facility for care,

his cup-to-disk ratios were recorded as 0.2 (see At the time of his most recent evaluation, best- Figure 31), and treated intraocular pressures corrected visual acuity had dropped to hand were 28 mmHg in the right eye and 30 mmHg motion in the left eye, but his IOP was 16 mmHg. in the left eye. Fluorescein angiography revealed a well-perfused disk with (at most) a 0.1 CID ratio and no gross Visual-field testing demonstrated minor inferior retinal abnormality (e.g., a vascular occlusion) (see loss in the left eye only (see Figure 32). Subse- Figure 29, B). A record review of the last two quent fields consistently reveal a similar inferior years revealed occasional noncompliance with and slightly nasal loss in the left eye only. medications and pressure spikes above 40 mmHg on several occasions. The cup-to-dish ratio, however, Heidelberg Retinal Tomograph I1 disk studies remained unchanged at 0.1 O.S. At the present revealed normal cupping and neuro-retinal rim in time, this patient has no measurable visual field both eyes (see Figure 33). and, with hand motion vision O.S., plus the pressure spikes into the 40fs, we believe this patient Nerve fiber layer analysis with the GDx revealed has end-stage glaucoma. The authors have seen significant nerve fiber layer attenuation in both several similar cases of occult glaucoma in very eyes. A GDx VCC revealed an NFI of 43 in the high myopes with spikes in IOPs. Nearly all these right eye and 69 in the left eye (see Figure 34, A). patients have maintained small, non-glaucomatous The deviation map demonstrates numerous sta- cups. We believe this case highlights the visual tistically significant abnormal RNFL points supe- consequences a patient can experience due rior and inferior nasal to the disk in the left eye. to the failure to diagnose glaucoma because The greatest deviation is in the superior RNFL of the absence of observable cupping. that corresponds to the minor inferior field defect

in the left eye.

Case 9 (JA): Glaucoma without cupping in a former OHTS Study patient

An OCT I11 also demonstrated an abnormal RNFL in each eye (see Figure 34, B), but this patient was

A 74-year-old Middle Eastern man was followed tested on a unit before the release of the nor- as an ocular hypertensive patient for 13 years. mative database.



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Auure 33 Case 9. Heidelberg Retinal Tomograph I1 confirms normal cupping and neuroretinal rim in each eye.

697

V O L U M E 7 5 l N U M B E R 1 1 I N O V E M B E R 2004 O P T O M E T R Y

I S S U E HIGHLIGHT

Treatment was instituted after the first GDx demonstrated marked RNFL loss, and the patient generally maintains IOPs in the upper teens, with no change in cupping, no visual- field progression, and no progression in nerve fiber layer attenuation. As previously mentioned, IOPs reached 28 mmHg O.D. and 30 mmHg O.S. on one occasion, despite reported compliance.

On a recent evaluation, both a 3- mirror lens fundus examination and B-scan ultrasonography were performed. Disk drusen could not be identified with either procedure. However, the disk borders appeared to be slightly lumpy, so subtle or occult disk drusen cannot be ruled out. This case represents glaucoma without cupping and no clear evidence of disk drusen. Since the central fields are normal in the right eye but the RNFL is abnormal with both the GDx VCC and the OCT 111, one could classify the right eye as "pre-perimetric" glaucoma without cupping.

Case 10 (PE): Glaucoma without cupping in an angle closure patient with undiagnosed disk drusen A 66-year-old white man woke up with light sensitivity and moderate discomfort in his left eye, but refused to present for an evaluation due to inclement weather and health-related issues, including a bad back. The patient had been using Ble- phamide for about a week for blepharoconjunctivitis. Previous records revealed 20120 visual acuity in each eye, open angles, Goldmann pressures of 12 mmHg OU, and cup-to-disk

Case 9. A, GDx VCC (top) reveals significant retinal nerve fiber layer (RNFL) attenuation ratios of 0.1 OU. Rather than Fiuure 34. ~n each eye (NFI = 43 O.D. and 69 0,s.). The most abnormal zone is the superior RNFL in

come in himself, he sent his wife the left eye; B, The OCT RNFL (bottom) is also abnormal in each eye.

698

OPTOMETRY VOLUME 75fNUMBER 11INOVEMBER 2004


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although patent, was inadequate. A second laser LPI was performed, which appeared to control the IOPs. Ultrasound Biomicroscopy (UBM) was never obtained, and it is unclear whether the patient had a plateau iris or (the very rarely reported) variable anterior cham- ber depth with corresponding pressure spikes.

The visual fields have consis-

Case 10. Visual fields reveal an inferior nasal arcuate loss with a nasal step in the left tently been normal in the right

Fiuure 35 eye. The right visual field was completely normal. eye and consistently abnormal in the left eye. The dense inferior

to pick up different eye drops. The patient, a computer expert, searched the Web and determined that his new symptom of light sensitivity was due to an iritis. Reluctantly, the doctor gave the wife topical steroid drops and a dilating agent for use in the left eye for what the doctor thought might be a reaction to the sulfa in Blephamide or perhaps for the self-diagnosed iritis. But the patient's condition worsened and he came in several days later. IOPs at that time were measured at 20 mmHg in the right eye and 52 mmHg in the symptomatic left eye. The doctor discontinued the medicines and began treatment with Alphagan and Xalatan in the left eye only. Without the dilating agent and with the glaucoma drops, the IOP dropped to 9 mmHg O.S. the next day. Several days later, however, the left eye once again became red and painful, in spite of IOPs in the teens. Fields revealed an inferior nasal arcuate loss with a nasal step in the left eye only (see Fig- ure 35).

The patient went to another doctor for a second opinion. Once again, an elevated IOP in the left eye was found and the angles were judged as being open and classified as grade I11 OU with the biomicroscope. A dilated examination revealed no cupping, and the patient was told to return in 1 week for followup and repeat visual fields. But the patient returned the next morning with pain and redness in the left eye. A glaucoma specialist was consulted who performed gonioscopy, a procedure not performed by either of the last two doctors. The angles were judged to be very narrow and a laser peripheral iridotomy (LPI) was performed in the left eye. Another bout of high IOPs led to a referral to a university-based glaucoma specialist, who believed that the first LPI,

arcuate scotomas and nasal step have perhaps deepened slightly O.S. Stereo disk photographs and HRT were obtained on several occasions. Nei- ther a 3-mirror lens evaluation of the disk, nor stereo photographs, nor HRT revealed any cupping whatsoever. The baseline HRT was inter- preted by the glaucoma specialist as a "minimal cup" in both eyes (see Figure 36).

Without any cupping detected in the left eye superiorly (to correspond to the dense inferior field loss O.S.), the university-based glaucoma specialist referred the patient to a retinal specialist for con- sultation. The retinal specialist suspected a superior branch artery occlusion, but a fluorescein angiogram revealed no evidence of an artery occlusion or any other retinal disorder.

Although two glaucoma specialists and a retinal specialist examined the patient, none noted the existence of subtle optic nerve head drusen OU (see Figure 37). Another specialist believed that anterior ischemic optic neuropathy (AION) in a disk at risk was the diagnosis.

Spikes in IOPs-due to angle ~ l o s u r e , ' ~ narrow- angle, pigmentary glaucoma, or high myopia- appear to result in axonal loss without cupping. l5 This case of glaucoma without cupping probably represents both angle closure and subtle disk drusen as the reason for "normal cup" glaucoma. It is also an example of the absolute failure of both subjective and objective disk assessment to predict the profound visual-field loss. In contrast, objective RNFL assessment with either the GDx or the OCT, neither of which have been performed to date, would predictably correspond to the subjective field loss. This case supports our



A u r e 36 Case 10. Heideelerg Retinal Tornograph i confirms no cupping in each eye

contention that disk drusen when present, may be missed and, similarly, an HRT may be misleading (such as depicted in Figure 36, which demonstrates no cupping) because of the disk drusen.

Case 11 (AT): Glaucoma without cup- Filure 37 Case 10. Note the absence of cupping and the slightly lumpy disk borders (A, right eye;

ping in an Asian man with B, left eye).

Posner-Schlossman syndrome A 61-year-old Asian man first presented to the University Opto- metric Center as the parent of a first-year optometry student. Examination revealed small cups with peripapillary atrophy, mod- estly elevated IOPs, and a family history of glaucoma (mother) (see Figure 38).

A follow-up examination was Case 11. Note the small cups (A, 0.3 0.0. and B, 0.35 O.S.), in the presence of peri- ordered to re-check IOPs and Figure 31 papillary atrophy.

perform baseline visual fields. At this visit, IOPs had decreased years. Central corneal thickness was only mar- from 22 to 19 mmHg O.D. and from 21 to 17 ginally thicker than average (550 microns). SITA mmHg O.S. Pressures persisted in this range at Standard 30-2 visual fields were full O.D., but all follow-up examinations over the next two showed inferior nasal defects O.S. (see Figure 39).

O P T O M E T R Y V O L U M E 7 5 l N U M B E R 1 I J N O V E M B E R 2004

ISSUE HIGHLIGHT

,I ., 1 . 1 , 11

. rnQLY I *...l01(1.. ....... an inferior nasal defect O.S.

I Filure 39 Case 11. Threshold Visual Fields are essentially full O.D., but show

H~lre 40 Case 11. An atypical glaucoma presentation with nasal zones flagged on HRT II, O.D. and 0s.

70 1

VOLUME 75lNUMBER 1 IINOVEMBER 2 0 0 4 OPTOMETRY


While on vacation, AT went to an eye clinic in Florida with pain in his left eye. IOPs were recorded at 21 mmHg O.D. and 39 mmHg O.S., with a few cells and keratic precipitates on the endothelium O.S. Treatment was initiated with a steroid and a beta-blocker to lower the IOP O.S. lleatment was later modified to Cosopt b.i.d. O.S. in March of 2003. Even with treatment, pressure spikes as high as 45 mmHg O.S. have since been recorded.

More extensive glaucoma-oriented testing revealed symmetric nasal areas of optic rim thinning in each eye with HRT 11, a somewhat unusual presentation in glaucoma (see Figure 40). The GDx VCC identified extensive RNFL loss O.S., with an NFI of 89 in the left eye (see Figure 41). The GDx also shows superior RNFL O.S. loss exceeding inferior loss, corresponding to the inferior nasal field defect O.S. (see Figure 42).

On closer evaluation of the anterior segment of the patient's left eye, dilated vessels and chemosis revealed a possible mixed mechanism for glaucoma in which both a raised episcleral venous pressure and a probable diagnosis of Posner-Schlossman syndrome exacerbated the chronic open-angle glaucoma. No disk cupping was detected at the time of treatment, nor has disk cupping progressed during the treatment period. At this time, the inferior RNFL thinning O.S. has yet to become evident on visual-field testing in the central 30 degrees, but can be appreciated in the 30- to 60-degree visual field (see Figure 42).

Family history, IOPs as high as 45 mmHg O.S. with an open angle, moderate glaucomatous field loss, and anterior segment drainage complications in the absence of optic nerve head cupping support a diagnosis of glaucoma without cupping O.S. The pronounced nerve fiber layer loss found on GDx verifies the corresponding field defects in the O.S. A careful review of the fundus photographs fails to reveal any evidence of disk drusen contributing to the lack of cupping.

Case 12 (SC): Glaucoma without cupping in an elderly black woman A 71-year-old black woman presented for an initial eye examination in 1991. At that time, IOPs were in the mid-teens OU, ClDs were 0.3 OU,

Case 11. GDx VCC corresponds with visual fields O.S. Normal Figure 41 ret~nal nerve fiber layer 0.0. corresponds to clean fields 0.13.

and the patient reported a negative family history of glaucoma. Medical history was positive for mild systemic hypertension, which was under control with treatment. Subsequent yearly examinations revealed a gradual escalation of IOP to the high-teens, reaching 33 mmHg O.D. and 29 mmHg O.S. in 2002, at which time treatment was initiated with Xalatan q.h.s. OU. Central corneal thickness was 520 pm O.D. and 525 pm O.S. Angles were open to the ciliary body OU on gonioscopy. Best-corrected visual acuities were slightly reduced, to 20130 O.D., O.S., OU, due to grade 2 + anterior and posterior cortical cataracts OU. The quality of the current fundus photographs was thus diminished, but reflected stable cupping over 11 years at 0.3 O.D., O.S. (see Fig- ure 43).

SITA-Standard 24-2 pattern deviation visual fields revealed superior and inferior arcuate scotomas O.D. and an inferior arcuate scotoma O.S. The total deviation plots revealed diffuse loss OU, probably secondary to the cataracts as well as glaucoma (see Figure 44). A glaucomatous supero-nasal quadrantic-type field defect O.D. was evident on a Humphrey 10-2 visual field (see Fig- ure 45).

7 0 2

O P T O M E T R Y V O L U M E 7 5 l N U M B E R 1 1 1 N O V E M B E R 2 0 0 4


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[iuUre 42 Case 11. Diagnostic synergy 0s . between peripheral field loss and retinal nerve fiber layer loss.

Case 12. Fundus photographs reveal 0.3 C/Us Fiuurc 43 quahty , . I S due to lack of media clarity. i t eye; B, left eye). Diminished

HRT I1 disk topography was essentially normal OU, with only the inferior temporal zone OS flagged as borderline by Moorfields Regression Analysis (see Figure 46); infero- temporal rim loss corresponds (somewhat poorly) to supero-nasal field loss. Here, disk drusen are unlikely, as disk topography would tend toward a "supernormal" appearance in their presence. B-scan ultrasonography also failed to identify disk drusen as a potential factor contributing to the observed field loss in the absence of cupping.

The GDx VCC revealed marked RNFL thinning OU, with an NFI of 98 O.D. and 61 O.S. (see Fig- ure 47). The pronounced inferior loss depicted on the left deviation map is somewhat consis-

tent with the borderline HRT finding in the inferior-temporal zone O.S.

The combined findings of escalating IOPs over an 11- year period with glaucomatous field loss OU and no ostensible increase in cupping over the same time period support a diagnosis of glaucoma without cupping. The GDx images help &splay the extent of RNFL

damage and the borderline HRT in the O.S., but the visual fields and pressure rise are suf- ficient for diagnosis.

Case 13 (PA): Glaucoma without cupping in a black woman A 49-year-old black woman was initially labeled as a glaucoma suspect by one of us (JS) in 1986 because of her strong family history of glaucoma (brother) and IOPs in the mid- to high twenties. Medical history revealed systemic hypertension for nearly a decade. Cup- ping was estimated as 0.35 OU. Gonioscopy revealed angles to be open to the ciliary body OU. The patient failed to return for visual fields after her general examination, and was next evaluated by a different clinician nearly



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Case 12. SITA-Standard 24-2 pattern deviation visual fields reveal arcuate field loss OU. Diffuse loss on the total deviation plots was also AIJIIR 44 ev~dent, . probably secondary to both the cataracts and glaucoma.

fiuure 45 Case 12. Central 1C-Z visual fields reveal a superior-nasal glaucomatous quadrantic-like field defect 0.0

704

O P T O M E T R Y V O L U M E 7 5 l N U M B E R 1 I I N O V E M B E R 2004

ISSUE HIGHLIGHT

fiiure 46 Case 12. HRT lldisk topography is within normal limits O D and borderline 0s.. with the inferior-temporal zone flagged.

I

Filure 47 Case 12. GDx VCC analysis reveals a grossly abnormal RNFL OU.

8 years later. Treatment with Timoptic was initiated in 1993 when IOPs increased to 30 mmHg and nasal field loss was documented with threshold fields in each eye. In 1994, another clinician examined her and discontinued the beta blocker, presumably because of the normal disks. IOPs with treatment were 22 and 23 mmHg at that time. Over the next several months, IOPs fluctuated through the mid-20s and reached 32 and 38 mmHg after 6 months of non-treatment. At this point, angles were assessed again and found to be open to the ciliary body OU, and treatment was rein- stituted. The CID ratio at this time was assessed as 0.35 OU (see Figure 48).

In 1997, a GDx was obtained when the technology became available. The nerve fiber layer revealed a moderate diffuse loss, with numerous parameters flagged as abnormal. The Number was 41 in the right eye and 46 in the left eye (see Figure 49).

Visual fields in 1999 appeared unchanged, and revealed a mean deviation loss of -17 db in the

VOLUME 75lNUMBER 1 IINOVEMBER 2004 OPTOMETRY


right eye and -8 db in the left eye (see Figure 50).

This case represents the last in this series of glaucoma without cupping. Family history, IOPs as high as 38 mmHg with open angles, and moderate-to-severe field loss support a diagnosis of glaucoma without cupping. The GDx-certainly not needed for RIJU~E 4 I B, left eye].

Case 13. Note the small cups ana well-aennea OISK Doraers In eacn eye (A, rlgnr eye; the diagnosis-supports the pronounced nerve fiber layer I 1 ioss in each eye. A careful ieview of the fun- r u n ~ w o n - ~ ~

GDxm S,mmetry Analysis , ~ E ~ m ~

dus photographs fails to reveal any evidence of A P ID # disk drusen as the cause. In contrast to several , ,, - ,-, ,, , ~ n t DUD won 1997

previous cases, this patient consistently had high pressures when not treated, but no spikes in the IOP have ever been documented. At the last evaluation, IOPs dropped to below 20 I I mmHg with combined beta blocker and alpha- ,-\,- =

m

gan therapy.

Summary Just as there are myriad etiologies of glaucoma, there are numerous signs of glaucoma as well. Cupping is but one sign of damage from this disease. However, since the range of cupldisk ratios is from 0.0 to 0.9 in the normal popu- lation, l6 the correct identification of both normal and glaucoma patients, based on cupping alone, becomes complicated. Since cupping can be misleading, the eye care practitioner needs to consider other diagnostic modalities, as well as other examination results (the ultimate hallmark of glaucoma is visual field change over time). Glaucoma can and does exist without cupping.

Summary points from these cases are as follows: 1. Glaucoma without cupping is often due to

disk drusen (obvious, subtle, or even occult).

2. Glaucoma without cupping also occurs in patients with pressure spikes, such as in angle closure, plateau iris, pigmentary glaucoma, etc.

3. Glaucoma without cupping in high myopes (Cases 7 and 8) is probably due to unde- tected pressure spikes in some (but not all) such cases. But some cases of glaucoma

Case 13. The retinal nerve fiber layer is abnormal in both Fiuure 41 .,,, without cupping still remain an enigma (Cases 9, 12, and 13).

4. Early reports of glaucoma without elevated pressures were initially received with great skepticism. Today, normal tension glaucoma is readily accepted as a true clinical entity. Pre-penmetric glaucoma, or glaucoma prior to field defects, is slowly becoming a recog- nized entity. With the advent of new clinical instruments (GDx, OCT, RTA), which can

706

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[iulre 50 Case 13. These threshold fields are markedly abnormal and typic

assess the retinal nerve fiber layer inde- pendently of the optic nerve head and visual fields, the documentation of both pre-perimetric glaucoma and glaucoma without cupping is now a clinical reality.

Three decades ago, clinicians were taught that glaucoma could only be diagnosed in patients with high pressures, characteristic field defects, and optic nerve cupping. Now we are begin- ning to realize that glaucoma can occur without elevated pressures , without overt visual-field defects, and without evident cupping. Widespread use of imaging instruments that can assess nerve fiber layer loss should document just how common such previously unrecognized subgroups of glaucoma are in clinical practice. Such imaging instruments should also be used to monitor change over time and to help guide appropriate diagnosis and treatment.

Acknowledgments and Disclaimers We wish to acknowledge Herminder S. Boparai, 0.0.. Jeffrey M . Roth, 0.0.. and Michelle Battaglia, B.A. for their invaluable assistance in the editing and prepa- ration of this manuscript for publication. The authors are not paid consultants to any of the companies mentioned in this article; none of the authors has any financial or proprietary involvement with any

:al of the fields measured on numerous occasions over the years.

companies mentioned; and none of the authors owns stock or has stock options in any of the companies mentioned. Dr. Sherman has declined stock options in Laser Diagnostic Technology. Dr. Sherman does lecture frequently for Laser Diagnostic Technology (LOT) and has prepared a CO of cases for Zeiss. Dr. Bass lectures occasionally for LOT, also. Dr. Sherman's private office. The Eye Institute and Laser Center in New York, has all of the instruments mentioned in the article, and all were paid for by the Eye Insti- tute. Dr. Sherman has received grant funds from LOT to the Research Foundation of the State of New York, but none of his salan/ has been paid by the various grants.

References 1. Sponsel WE. Tonometry in question: can visual screen-

ing tests play a more decisive role in glaucoma diagnosis and management? Surv Ophthalmol 1 9 8 9 ; 3 3 (Supp l ) :291-300.

2. Kass MA, Heuer DK, Higginbotham EJ, et al. The Ocu- lar Hypertension Treatment Study: a randomized trial determines that topical ocular hypotensive medication delays or prevents the onset of primary open-angle glaucoma. Arch Ophthalmol2002;120:701-13.

3. Zangwill LM, Bowd C, Berry CC, et al. Discriminating between normal and glaucomatous eyes using the Hei- delberg Retinal Tornograph, GDx Nerve Fiber Layer Ana- lyzer, and Optical Coherence Tornograph. Arch Ophthalmol 2001;119;985-93.

4. Bass SJ, Sherman J. Optic disk evaluation and the use of hi-tech in the diagnosis of glaucoma. Optometry 2004; 75:277-96.

5. Jonas JB, Nguyen NX, Naumann GO. The retinal nerve fiber layer in normal eyes. Ophthalmology 1989;96:627-32.

6. Ritch R, Mudumbai R, Liebmann JM. Combined exfoliation and pigment dispersion: paradigm of an overlap syndrome. Ophthalmology 2000; 107: 1 0 0 4 - 8 .

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ISSUE HIGHLIGHT

7. Varma R, Tielsch JM, Quigley HA, et al. Race-, age-, gen- der-, and refractive error-related differences in the normal optic disk. Arch Ophthalmol 1994; 112: 1068-76.

8. Caprioli J, Spaeth GL. Static threshold examination of the peripheral nasal visual field in glaucoma. Arch Ophthalmol 1985; 103:1150-4.

9. Ballon BJ, Echelman DA, Shields MB, et al. Peripheral visual field testing in glaucoma by automated kinetic perimetry with the Humphrey Field Analyzer. Arch Oph- thalmol 1992; 110: 1730-2.

10. Johnson CA. Diagnostic value of short-wavelength automated perimetry. Cum Opin Ophthalmol 1996;7:54-8.

11. Johnson CA, Sample PA, Cioffi GA, et al. Structure and function evaluation (SAFE]: I. criteria for glaucomatous visual field loss using standard automated perimetry (SAP) and short wavelength automated perimetry (SWAP). Am J Ophthalrnol2002; 134: 177-85.

12. Samples JR, van Buskirk M, Shults WT, et al. Optic nerve head drusen and glaucoma. Arch Ophthalmol 1985; 103: 1678-80.

13. Pickett JE, Teny SA, O'Connor PS, et al. Early loss in central visual acuity in glaucoma. Ophthalmology 1985; 92:891-6.

14. Douglas GR, Drance SM, Schulzer M. The visual field and nerve head in angle-closure glaucoma. Arch Oph- thalmol 1975;93:409-11.

15. Caprioli J. Correlation between disk appearance and type of glaucoma. In: V m a R, Spaeth GL, eds. The optic nerve in glaucoma. Philadelphia: Lippincott, 1993:91-8.

16. Jonas JB, Gusek GC, Naumann GO. Optic disk, cup and neuroretinal rim size, configuration, and correlations in normal eyes. Invest Ophthalmol Vis Sci 1988;29:1151-8. (Correction: Invest Opthalmol Vis Sci 1991;32:1893.)

Corresponding author:

Jerome Sherman, O.D. State University of New York

College of Optometry 33 West 42nd Street

New York, New York 10036

708

OPTOMETRY VOLUME 75lNUMBER 1 11NOVEMBER 2004

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Glaucoma without cupping

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