6
Major Articles Efficacy of Spectacles in Persons With Albinism Jill Anderson, MD, a Jane Lavoie, CO, a Kim Merrill, CO, a Richard A. King, MD, PhD, a,b,c,d and C. Gail Summers, MD ad Introduction: Patients with albinism have varying degrees of reduced vision, strabismus, iris transillumination, nystagmus, fundus hypopigmentation, and foveal hypoplasia. High refractive errors are common, but reduced vision persists due to nonrefractive factors, causing reluctance by some clinicians to prescribe spectacles. We sought to evaluate the effect of spectacle correction of refractive error on clinical findings and recorded compliance with refractive corrections, as little detailed data exist. Methods: We prospectively examined 35 consecutive patients with albinism for whom glasses had been prescribed to determine if objective improvement in recognition visual acuity (VA), strabismus, anomalous head posture (AHP), fusion, or stereoacuity occurred with refractive correction. Parents or patients reported compliance with glasses wear (excellent: 75% of awake hours; good: 50-75% of awake hours; fair: 26-50% of awake hours; poor: 25%). Results: Median age was 9.5 years (range: 3 to 30). Median refractive correction was 1.875 D spherical equivalent (range: 9.75 to 8.88 D). Glasses wear was initiated at a median age of 14 months (range: 3 months to 14 years). Mean binocular VA at distance was 20/80.9 corrected and 20/107.6 uncorrected (P 0.001). Mean VA at near was 20/28.4 corrected and 20/41 uncorrected (P 0.001). Mean strabismic deviation was 7.2 PD with glasses and 10.0 PD without glasses at distance (P 0.006) and 10.8 PD with glasses and 14 PD without glasses at near (P 0.042). Mean AHP at distance was 8.3 degrees with glasses and 7.3 degrees without glasses at distance (P 0.327) and 4.7 degrees both with and without glasses at near (P 0.308). Twenty-one patients had fusion with or without glasses, two had fusion only with glasses, and one patient had fusion only without glasses. The other patients did not have any detectable degree of fusion. Twenty-seven individuals had no stereoacuity with or without glasses, five had gross stereoacuity of 3000 seconds of arc both with and without glasses, and three had gross stereoacuity only while wearing glasses. Compliance was excellent in 29 patients, fair in four, and poor in two. Conclusion: This prospective study showed a significant improvement in corrected VA and alignment in persons with albinism, despite overall subnormal acuity. Some individuals also experienced improvement in binocular alignment and AHP. Compliance with spectacles was generally good. Therefore, refractive correction should be encouraged in persons with albinism as improvement in visual function is likely to occur. (J AAPOS 2004;8:515-520) A lbinism is an inherited disorder of melanin pigment production with a heterogenous phenotype. While the diagnosis of albinism is typically based on clin- ical features, advances in molecular genetics have permit- ted classification that now includes 10 different genetic loci. Cutaneous hypopigmentation in albinism ranges from complete absence of melanin and inability to tan to a minimal reduction in skin and hair color. 1 The ocular phenotype includes reduced visual acuity, nystagmus, stra- bismus, irides that transilluminate, hypopigmented fundi, reduced or absent stereopsis, and the invariable finding of foveal hypoplasia. Optic misrouting detected by visual evoked potentials with monocular stimulation has also been shown to be constantly associated with albinism. 2,3 Detecting misrouting with visual evoked potentials (VEP) after monocular stimulation is particularly useful when a definite diagnosis cannot be made due to variation from the expected phenotype. 2 High refractive errors are common in albinism, with myopia, hyperopia, and astigmatism being described. 4 Re- duced vision usually persists despite refractive correction due to foveal hypoplasia and nystagmus. 5 Because of pre- sumed poor potential for visual improvement, many eye care professionals do not prescribe glasses. In this prospec- From the Departments of Ophthalmology, a Medicine, b Institute of Human Genetics, c and Pediatrics, d University of Minnesota, Minneapolis, Minnesota. Presented at the 29th Annual Meeting of the American Association for Pediatric Ophthal- mology and Strabismus, Big Island, Hawaii, March 23-27, 2003. Supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, New York. Submitted September 4, 2003. Revision accepted August 30, 2004. Reprint requests: C. Gail Summers, MD, Department of Ophthalmology, Mayo Mail Code 493, 420 Delaware Street SE, Minneapolis, MN [email protected] Copyright © 2004 by the American Association for Pediatric Ophthalmology and Strabismus. 1091-8531/2004/$35.00 0 doi:10.1016/j.jaapos.2004.08.008 Journal of AAPOS December 2004 515

Efficacy of spectacles in persons with albinism

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Page 1: Efficacy of spectacles in persons with albinism

thm

ation,duceds. Weordeded 35ementd withwakeyearsassestance20/41es atHP atgreess, twoe anygrosswhile

: Thisinism,AHP.ed in

Major Articles

Efficacy of Spectacles in Persons WiAlbinis

Jill Anderson, MD,a Jane Lavoie, CO,a Kim Merrill, CO,a Richard A. King, MD, PhD,a,b,c,d

and C. Gail Summers, MDad

Introduction: Patients with albinism have varying degrees of reduced vision, strabismus, iris transilluminnystagmus, fundus hypopigmentation, and foveal hypoplasia. High refractive errors are common, but revision persists due to nonrefractive factors, causing reluctance by some clinicians to prescribe spectaclesought to evaluate the effect of spectacle correction of refractive error on clinical findings and reccompliance with refractive corrections, as little detailed data exist. Methods: We prospectively examinconsecutive patients with albinism for whom glasses had been prescribed to determine if objective improvin recognition visual acuity (VA), strabismus, anomalous head posture (AHP), fusion, or stereoacuity occurrerefractive correction. Parents or patients reported compliance with glasses wear (excellent: �75% of ahours; good: 50-75% of awake hours; fair: 26-50% of awake hours; poor: �25%). Results: Median age was 9.5(range: 3 to 30). Median refractive correction was 1.875 D spherical equivalent (range: �9.75 to �8.88 D). Glwear was initiated at a median age of 14 months (range: 3 months to 14 years). Mean binocular VA at diswas 20/80.9 corrected and 20/107.6 uncorrected (P � 0.001). Mean VA at near was 20/28.4 corrected anduncorrected (P � 0.001). Mean strabismic deviation was 7.2 PD with glasses and 10.0 PD without glassdistance (P � 0.006) and 10.8 PD with glasses and 14 PD without glasses at near (P � 0.042). Mean Adistance was 8.3 degrees with glasses and 7.3 degrees without glasses at distance (P � 0.327) and 4.7 deboth with and without glasses at near (P � 0.308). Twenty-one patients had fusion with or without glassehad fusion only with glasses, and one patient had fusion only without glasses. The other patients did not havdetectable degree of fusion. Twenty-seven individuals had no stereoacuity with or without glasses, five hadstereoacuity of 3000 seconds of arc both with and without glasses, and three had gross stereoacuity onlywearing glasses. Compliance was excellent in 29 patients, fair in four, and poor in two. Conclusionprospective study showed a significant improvement in corrected VA and alignment in persons with albdespite overall subnormal acuity. Some individuals also experienced improvement in binocular alignment andCompliance with spectacles was generally good. Therefore, refractive correction should be encourag

persons with albinism as improvement in visual function is likely to occur. (J AAPOS 2004;8:515-520)

pigme. Whon cperm

t gen

ges fromtan to ae ocularus, stra-

ed fundi,nding of

by visualhas alsoinism.2,3

ls (VEP)l when aion from

sm, withed.4 Re-

orrectione of pre-any eye

Genetics,

atric Oph

ess, Inc.,

ayo Mail.edualmology

A lbinism is an inherited disorder of melaninproduction with a heterogenous phenotypthe diagnosis of albinism is typically based

ical features, advances in molecular genetics haveted classification that now includes 10 differenloci.

From the Departments of Ophthalmology,a Medicine,b Institute of HumanPediatrics,d University of Minnesota, Minneapolis, Minnesota.Presented at the 29th Annual Meeting of the American Association for Pedimology and Strabismus, Big Island, Hawaii, March 23-27, 2003.Supported in part by an unrestricted grant from Research to Prevent BlindnYork, New York.Submitted September 4, 2003.Revision accepted August 30, 2004.Reprint requests: C. Gail Summers, MD, Department of Ophthalmology, M493, 420 Delaware Street SE, Minneapolis, MN 55455.summe001@umnCopyright © 2004 by the American Association for Pediatric OphthStrabismus.1091-8531/2004/$35.00 � 0

doi:10.1016/j.jaapos.2004.08.008

Journal of AAPOS

entile

lin-it-

etic

Cutaneous hypopigmentation in albinism rancomplete absence of melanin and inability tominimal reduction in skin and hair color.1 Thphenotype includes reduced visual acuity, nystagmbismus, irides that transilluminate, hypopigmentreduced or absent stereopsis, and the invariable fifoveal hypoplasia. Optic misrouting detectedevoked potentials with monocular stimulationbeen shown to be constantly associated with albDetecting misrouting with visual evoked potentiaafter monocular stimulation is particularly usefudefinite diagnosis cannot be made due to variatthe expected phenotype.2

High refractive errors are common in albinimyopia, hyperopia, and astigmatism being describduced vision usually persists despite refractive cdue to foveal hypoplasia and nystagmus.5 Becaussumed poor potential for visual improvement, m

c and

thal-

New

Code

and

care professionals do not prescribe glasses. In this prospec-

December 2004 515

Page 2: Efficacy of spectacles in persons with albinism

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Journal of AAPOSVolume 8 Number 6 December 2004516 Anderson et al.

tive study, we sought to determine whether glassethe clinical findings in persons with albinism antermine an estimate of compliance with spectacltion of refractive error.

METHODS

The project was approved by the institutionaboard at the University of Minnesota. Afterconsent was obtained from subjects or their pguardians, we prospectively recruited 35 consectients with albinism as they came in for their fvisits. Although these patients had been previoand glasses had been prescribed, the data were coa prospective manner with forms used to collecsired clinical findings for this study. Diagnosis wby an ophthalmologist and a geneticist based ophenotype. Abnormal decussation of the retrostridetected with VEP, normal electroretinographydetection of mutations on genes causing albiniused when the diagnosis could not be confirmed bexamination. Patients were included if a cycloplegtion had been performed within the past year anognition acuity could be measured. In generawere prescribed for refractive error in at least onewas �3.50 D myopia, 3.00 D hyperopia, and/oastigmatism in children less than age 2, and �myopia, 2.00 D hyperopia, and 2.00 D astigmchildren greater than age 2 after cycloplegia wipentolate 1.3%. Refractive errors smaller than thiued to be corrected as the individuals matured everefractive error decreased below the above guidthe time of this study, if they had been wearglasses at least half of their awake hours. Onlytients included in this study had glasses that didour criteria in at least one eye. Exclusion criteriainability to perform binocular recognition acuity

One of two certified orthoptists performedtions first while patients were wearing their glathen repeated examinations when patients were ning their glasses. With fixation on linear symboaccommodative target at 20 feet and 14 inchescorded best-corrected binocular visual acuity (Vbismus with prism and alternate cover testing, analous head posture (AHP) measured with a gonPatients were allowed to use their preferred headwhen VA, stereoacuity, and AHP were measalignment was measured in primary position. Recacuity was measured with Snellen charts, but meawas allowed with HOTV matching or Allen figuSnellen acuity could not be recorded. Alternatesting was rapidly performed to minimize the inamplitude of nystagmus that can make accuratement of strabismus more difficult. We recordeusing the Worth-4-dot test (Lombart Instrumenfolk, VA) at 20 feet and 13 inches and stereoacu

the Titmus (Stereo Optical Co., Inc., Chicago, IL) tes

redde-ec-

iewedor

pa--upeen

inde-adeicalers/orereicalac-ec-seshatDDin

lo-tin-eir

s ateirpa-eet

ded

na-andar-anre-

tra-m-ter.urebutionenten

verin

re-ionor-ing

14 inches, both with and without glasses. Parentients reported compliance with glasses wear as(�75% of awake hours), good (50-75%), fair (26-poor (�25%).

LogMAR acuity scores were analyzed and conSnellen equivalents for data reporting. Absoluteand exotropia values were used to determine tstrabismus. Similarly, we used absolute degreeszontal AHP and did not tabulate direction of turnrank P value was used to assess statistical signStatistical significance was assigned for P � 0.05

RESULTS

The studied group included 19 patients with oculous albinism type 1, 12 patients with oculocutanenism type 2, and 4 patients with ocular albinismrecruited between 1/02 and 7/03 (Table 1). Thincluded 18 males and 17 females. The mediaexamination was 9.5 years (range: 3 to 30 yeamedian age at beginning to wear refractive corre14 months (range: 3 months to 14 years). All haprescribed for at least 2.5 years except patients 334, who had worn glasses for 2 months to 1 ymedian refractive correction was 1.875 D sphericalent with a range from �9.75 to �8.88 D. Acorrection ranged from 0 to 6.75 D. Three patiewearing refractive correction that did not meet aabove listed criteria in either eye. Three patielenses that darkened with exposure to UV lighwore other types of filtered lenses.

Twenty-eight patients were tested using Snelle6 with HOTV, and 1 with Allen figures. Mean bvision significantly improved with glasses (Tabledistance VA was 20/80.9 with glasses compare107.6 without glasses (P � 0.001). Mean nearsignificantly improved, measured at 20/28.4 witcompared to 20/41.0 without glasses (P � 0.0individual had worse VA with glasses at distanceThree individuals had improved VA with glasses o1 octave with distance fixation and seven individimprovement of at least 1 octave with near fixatioing was performed using the present glasses butand cycloplegic refractions were done and thedifference from present glasses was only 0.50 Dand/or cylinder.

Mean strabismus of 7.2 PD ET (range: 20 PDPD XT) measured with distance fixation was sigless with glasses compared with 10.0 PD ET (rangET to 14 PD XT) without glasses (P � 0.006). Stwith near fixation was also significantly improv14.0 PD ET (range: 80 PD ET to 14 PD XT)glasses to 10.8 PD ET (range: 35 PD ET to 25with glasses (P � 0.042). Although the meanments in alignment may not seem large in acglasses improved the alignment at either distanc

t at fixation by at least 10 PD in 12 cases. However, alignment

Page 3: Efficacy of spectacles in persons with albinism

Glasses

.25 � 91

.75 � 89

.75 � 90

.00 � 90

.00 � 86

.00 � 90

.25 � 90

.00 � 90

.75 � 82

.00 � 99

.50 � 109

.75 � 78

.75 � 85

.00 � 90

.25 � 95

.75 � 90

.00 � 85

.75 � 102

.75 � 87

.75 � 97

.00 � 90

.25 � 95

.75 � 100

.75 � 86

.00 � 92

.50 � 88

.50 � 80

.25 � 95

.50 � 120

.75 � 70

.00 � 83

.00 � 84

.25 � 175

.50 � 15

.75 � 115

.00 � 60

.00 � 55

.25 � 90

.00 � 180

.75 � 175

.50 � 70

.75 � 90

.25 � 95

.75 � 79

.25 � 90

.00 � 70

.75 � 80

.75 � 80

.25 � 80

.00 � 91

.25 � 90

.25 � 88

.00 � 90

.00 � 90

.75 � 90

.50 � 90

.25 � 100

Journal of AAPOSVolume 8 Number 6 December 2004 Anderson et al. 517

Table 1. Patient Characteristics and VA results

IDType of

AlbinismAge

(mos)

Binocular VA Distance Binocular VA Near

PresentWith

GlassesWithoutGlasses

WithGlasses

WithoutGlasses

1 OCA 1B 72 20/70 20/70 20/20 20/30 �2.50 � 1�3.25 � 1

2 OCA 1B 150 20/100 20/125 20/25 20/30 �1.00 � 4�1.75 � 4

3 OCA 1B 52 20/150* 20/150* 20/25* 20/50* �0.25 � 2Plano � 3

4 OCA 1A 60 20/160 20/200 20/200 20/200 �1.75 � 2�0.25 � 3

5 OCA 1B 114 20/70 20/80 20/25 20/50 �1.25 � 1�1.75 � 2

6 OCA 2 120 20/80 20/100 20/50 20/50 Plano � 4Plano � 3

7 OCA 1A 60 20/160 20/160 20/40 20/70 �3.00 � 3�3.50 � 3

8 OCA 1B 120 20/50 20/400 20/20 20/40 �10.50 � 5�11.75 � 6

9 OCA 1B 66 20/70* 20/125* 20/20* 20/40* �4.00 � 3�4.50 � 1

10 OCA 2 72 20/60 20/60 20/20 20/25 �4.50 � 2�3.25 � 2

11 OCA 1B 84 20/100 20/100 20/20 20/40 �6.00 � 2�6.50 � 2

12 OCA 2 84 20/125 20/125 20/20 20/25 Plano � 3�0.75 � 3

13 OCA 1B 90 20/80 20/80 20/50 20/70 Plano � 4�1.00 � 3

14 OCA 1B 132 20/125 20/160 20/50 20/200 �7.00 � 3�7.25 � 3

15 OA 1 48 20/200* 20/200* 20/25* 20/60* �1.25 � 3�1.00 � 2

16 OCA 1A 60 20/100* 20/200* 20/50* 20/80* �7.00 � 5�7.00 � 4

17 OCA 2 360 20/50 20/50 20/20 20/50 �3.00 � 0�2.50 � 0

18 OCA 2 168 20/100 20/300 20/40 20/200 �11.50 � 3�12.25 � 5

19 OA 1 132 20/70 20/125 20/25 20/40 �8.75 � 4�5.25 � 3

20 OCA 1B 120 20/40 20/40 20/25 20/25 �1.50 � 1�1.75 � 0

21 OCA 1B 144 20/30 20/30 20/25 20/25 �1.25 � 1�1.00 � 3

22 OCA 2 204 20/60 20/80 20/20 20/20 �1.25 � 4�1.25 � 4

23 OCA 2 180 20/60 20/60 20/20 20/20 �1.25 � 1�1.25 � 1

24 OCA 2 192 20/40 20/50 20/20 20/30 �1.25 � 2�2.75 � 0

25 OCA 1A 132 20/100 20/150 20/20 20/30 �1.50 � 5�2.00 � 4

26 OCA 1B 264 20/50 20/60 20/25 20/25 �8.00 � 3�8.50 � 6

27 OCA 2 60 20/200 20/200 20/40 20/40 �3.00 � 5�3.25 � 5

28 OCA 2 132 20/100 20/125 20/40 20/40 �3.50 � 2�2.50 � 2

29 OCA 2 276 20/60 20/100 20/25 20/40 �2.00 � 6

�0.25 � 3.25 � 112
Page 4: Efficacy of spectacles in persons with albinism

in setion, ttients

spherwith

tropid exoter examberincl

t. Ofnts win whand te corrin whoneifferepatie

e patihange5-10 Pr grea

ents w�9.75

of whiched by 10

measure-d for thee slightlyees with-P at nears withoutor with-ses. Oneelated tothis pa-

etectableno ste-

conds ofad 3000

as excel-The twos old andpatients

cians. Ofot differ

nses. Nof filtered

Glasses

0 � 90e5 � 1050 � 205 � 895 � 900 � 900 � 850 � 905 � 86ee

y testing

ned RankP Value

�0.001�0.001

0.3270.3080.0060.042

nts.

Journal of AAPOSVolume 8 Number 6 December 2004518 Anderson et al.

with glasses was worse than without correctionpatients (two patients by 2 PD with distance fixapatients by 5 PD with near fixation, and three pa8-10 PD with distance and near fixation).

Subgroup analysis relating refractive errorequivalents and strabismus showed 10 patientsesotropia or exotropia, 14 with hyperopia and esowith myopia and esotropia, 4 with hyperopia anpia, and 1 with myopia and exotropia. We furthined the esotropic group as it had the larger nupatients. The refractive error ranges that followthe spherical equivalents of both eyes of a patienpatients with hyperopia, there were four patierefractive errors between �5.00 and �8.875 Dtwo showed improvement in ET by 20 PDshowed improvement by 5-10 PD with refractivtion; three patients between �3.25 and �5.00 Done had improvement of the ET by 20 PD,improvement by 5-10 PD, and one had minimal din alignment with refractive correction; and sevenbetween �0.25 and �2.75 D. Of these seven, onhad increased ET by 5-10 PD, one showed no calignment, three showed improvement in ET byand two showed improvement in ET by 10 PD owith refractive correction. Of the esotropic patimyopia, there were three between �6.75 and

Table 1. Continued

IDType of

AlbinismAge

(mos)

Binocula

WithGlasses

30 OCA 1B 48 20/60*

31 OA 1 108 20/150

32 OA 1 108 20/150

33 OCA 1B 36 20/30**

34 OCA 2 52 20/125*

35 OCA 1B 138 20/40

*VA measured with HOTV matching.**VA measured with Allen figures. All others represent Snellen acuit

Table 2. Clinical Characteristics of Study Population (n � 35

VA at distant gaze*VA at near gaze*AHP at distant gaze (Degrees)AHP at near gaze (Degrees)Strabismus at distant gaze (Prism diopters)Strabismus at near gaze (Prism diopters)*Values are the logarithmic means converted to the Snellen equivale

with no change in the angle of ET when glasses were us

venwoby

icalouta, 6ro-m-of

udetheithichwoec-ich

hadncentsent

inD,terithD

and three patients between �0.25 and �1.75 D,two had minimal change in ET and one improvPD with correction.

No statistically significant difference betweenments made with and without glasses was founother studied variables. AHP measurements werhigher with glasses at 8.3 degrees versus 7.3 degrout glasses with distance fixation (P � 0.327). AHmeasured 4.7 degrees with glasses and 4.7 degreethem. Twenty-one patients had fusion both without glasses, while two had fusion only with glaspatient only had fusion without glasses, probably rthe variable angle of the strabismic deviation intient. The other patients did not have any ddegree of fusion. Twenty-seven individuals hadreoacuity with or without glasses, five had 3000 searc both with and without glasses, and three hseconds of arc only while wearing glasses.

Parents reported compliance with glasses wearlent for 29 patients, fair for 4, and poor for 2.patients with poor compliance were both 11 yeardid not like their appearance with glasses. Threewere fit with UV-darkening lenses by their optithese, all three had good compliance. This does nfrom the compliance in patients without these lepatient in this study was wearing other types o

istance Binocular VA Near

PresentWithoutGlasses

WithGlasses

WithoutGlasses

20/60* 20/20* 20/25* �4.00 � 0.5�4.00 spher

20/250 20/25 20/25 �2.00 � 1.2�2.75 � 2.0

20/250 20/25 20/25 �2.25 � 2.2�2.75 � 2.7

20/50** 20/30** 20/30** �3.50 � 1.5�2.50 � 1.5

20/125* 20/40* 20/60* �4.50 � 3.5�5.50 � 3.2

20/70 20/20 20/20 �1.25 spher�1.75 spher

.

WithGlasses

WithoutGlasses

Sig

20/80.9 20/107.620/28.4 20/41.0

8.3 7.34.7 4.77.2 ET 10.0 ET

10.8 ET 14.0 ET

r VA D

)

ed, lenses.

Page 5: Efficacy of spectacles in persons with albinism

tely 1lly mystagmormation,is av

s arermalould

r. Inents wheir c

anceatisticis knoognitre teso expifferey eitave bthat dsses wen teslossed wh

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protroeqveralorizonome

re whe mwith

14.0veme, glas

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not serabismnot, i

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s had ans and oneses were

y signifi-be clin-dependsmay use

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reoacuityents whoreoacuitystereoa-

dividualson while

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ith actualnized orefractiveents thatrtant to

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ciated with

Journal of AAPOSVolume 8 Number 6 December 2004 Anderson et al. 519

DISCUSSION

Albinism occurs with a frequency of approxima18,000 in the United States.6 Diagnosis is typicaafter referral to an eye care professional when nappears and/or vision does not begin to develop nDespite the routine measure of cycloplegic refractle information on the usefulness of glasses wearable for these patients. This may be because glasseprescribed due to inevitable persistence of subnosion and a question as to whether the child wcompliant with correction of high refractive erroprospective study that collected data on 35 patialbinism who had glasses prescribed, we report tical findings and their compliance with glasses.

The improvement in visual acuity at both distnear with glasses was found to be the most stsignificant of any variable studied (P � 0.001). Itthat grating acuity may overestimate eventual recacuity6 and none of the patients in our study wewith this method. The data were collected by twenced orthoptists. There did not appear to be a din the amount of improvement documented borthoptist. It is possible that the children may hless attentive as the examination progressed andfrom the portion of the examination without glaaffected by this. However, the ages of the childrwere advanced enough to suspect that attentionnot a factor contributing to the differences observpatients wore or did not wear their glasses.

Despite the improvement in visual acuity withthe overall acuities remain subnormal. This is mcaused by the invariable association of foveal hyalthough nystagmus may also contribute. Threeals (patients 8, 31, 32) had previous Kestenbaumdures and two others (patients 14 and 29) had retorial placement of horizontal rectus muscles. Sethors have reported that large four-muscle hrectus recessions may improve visual acuity in stients.7,8,9

Improvement in strabismus when glasses wewas also found to be statistically significant. Tdeviation was 7.2 PD with glasses and 10.0 PDglasses at distance, and 10.8 PD with glasses andwithout glasses at near. Although the mean improin alignment may not seem large in actual PDimproved the alignment at either distance or neaby at least 10 PD in 12 cases. There was a trengreater improvement in the angle of esotropia inwith high hyperopia when glasses were used, inson to those patients with smaller amounts of hbut this finding was not invariable.

In general, the anomalous head postures didto be improved by glasses wear. Because the stimproved with glasses but the head posture did

possible that the anomalous head postures were m

inade

uslly.lit-ail-notvi-be

thisith

lin-

andallywniontederi-ncehereenataeretedwasen

ses,elysia,du-ce-ua-au-tal

pa-

orneanoutPDntssesionardntsari-pia,

emus

t is

often caused by abnormalities other than incomibismus. Individuals with nystagmus will often deAHP to damp their nystagmus. Seven patientincrease in head posture between 5 and 10 degreepatient had an increase of 13 degrees when glasworn. While analyzed data were not statisticallcant, the difference in these individual cases mayically significant. We recognize that head postureon visual interest and speculate that some personsan increased head posture to find their null povisual acuity is improved with glasses.

There were two individuals who gained fusglasses, but one also lost fusion with glasses. Nonethree patients had any detectable amount of steeither with or without glasses. Because three patifused with the Worth-4-dot test gained gross ste(3000 seconds of arc) with glasses and none lostcuity with glasses, it is likely that at least some inwill experience an increase in binocular functiwearing glasses.10

In our study, we assessed compliance by aspatients or parents to rate the compliance by perctime glasses were worn. The responses showwhelming compliance with glasses wear. This retentially could have overestimated compliance wglasses wear as individuals or parents either recogdesired an improved level of visual function with rcorrection. However, the perception by the parthe child is tolerating the glasses is also impoensure their continued efforts to reinforce the bethe child. In addition, we selected individuals woften had been wearing glasses for several yearswho had been intolerant of glasses in the past mtransferred their eye care prior to this study, butour database of patients with albinism would nothat this is a large source of bias. Our general imis that children with albinism who have refractiwithin the limits noted above are usually complglasses wear. Such may be related to the accessexcellent fitting of pediatric frames in our patienthe tendency for patients with albinism to experduced photosensitivity when filtering lensesscribed.

In conclusion, glasses wear in persons with albisignificantly improve visual acuity, but subnormacuity persists. However, children with albinismerally compliant with glasses wear when presccording to the guidelines described in this articletive correction should be encouraged as part ofagement plan. In general, compliance with spectshould be expected to improve vision and alignm

REFERENCES1. Oetting WS, Brilliant MH, King RA. The clinical s

albinism in humans. Mol Med Today 1996;2:330-5.2. Creel DJ, Summers CG, King RA. Visual anomalies asso

ore albinism. Ophthalmic Paediatr Genet 1990;11:193-200.

Page 6: Efficacy of spectacles in persons with albinism

ed mutal in t

f albin

age qu990;11

l Soc 1

s surgery inus 1997;34:

horizontal8:1302-5.

ions for the1991;109:

h albinism:

Journal of AAPOSVolume 8 Number 6 December 2004520 Anderson et al.

3. Guillery RW. Why do albinos and other hypopigmentlack normal binocular vision, and what else is abnormcentral visual pathways? Eye 1996;10:217-21.

4. Abadi R, Pascal E. The recognition and management oOphthalmic Physiol Opt 1989;9:3-15.

5. Abadi RV, Dickinson CM, Pascal E, et al. Retinal imin albinos: a review. Ophthalmic Paediatr Genet 180.

6. Summers CG. Vision in albinism. Trans Am Ophthalmo

XCIV:1095-155.

antsheir

ism.

ality:171

996;

7. Davis P, Baker R, Piccione R. Large recession nystagmualbinos: effect on acuity. J Pediatr Ophthalmol Strabism279-85.

8. Helveston E, Ellis F, Plager D. Large recession of therecti for treatment of nystagmus. Ophthalmology 1991;9

9. von Noorden G, Sprunger D. Large rectus muscle recesstreatment of congenital nystamus. Arch Ophthalmol221-4.

10. Lee K, King R, Summers CG. Stereopsis in patients wit

clinical correlates. J AAPOS 2001: 98-104.

An Eye on the Arts – The Arts on the Eye

Things become interesting when conversions occur. Moving toward a newfaith is usually attended by the sense of a change from a state of ignorance,blindness, deludedness to one of knowledge, vision, the truth. St. Augustine,that great observer of the self, speaks of his “false conceptions of God.” In hisConfessions he tells God: “My conception of you was quite untrue, a merefalsehood. It was a fiction based on my own wretched state, not the firmfoundation of your bliss.” My eyes saw only material things, my mind saw onlytheir images; I did not know, I was ignorant, I was blind—the Confessions arestudded with these kinds of self-assessments concerning Augustine’s earlierfalse, deluded stated.

The profound spiritual transformation that Augustine underwent involvedan equally profound feeling of having arrived at the truth after decades of errorand after a battle with his own mind, which had insisted that the truth berevealed by rational thought. Among self-confessed former self-deceivers, hestands out for his analysis of the fear that attended his conversion, the fear oftaking the wrong path. He tells God:

I wanted to be just as certain of these things which were hidden from mysight as that seven and three make ten. . . . If I had been able to believe Imight have been cured, because in my mind’s eye I should have had clearervision, which by some means might have been directed towards youreternal, unfailing truth. . . . [M]y sick soul, which could not be healedexcept through faith, refused this cure for fear of believing a doctrine thatwas false.

Even when God had stood him face to face with himself to make him see howsordid, deformed, and squalid he was “so that I should see my wickedness andloathe it,” Augustine had resisted. “I had known it all along, but I had alwayspretended that it was something different. I had turned a blind eye andforgotten it.”

—Evelin Sullivan (from The Concise Book of Lying)