Volume 14 Number 6 / December 2010 Letters to the Editor 561

5. Nischal KK. Congenital corneal opacities—a surgical approach tonomenclature and classification. Eye 2007;21:1326-37.

doi:10.1016/j.jaapos.2010.09.001J AAPOS 2010;14:560-561.Copyright � 2010 by the American Association for Pediatric Ophthalmology and

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REPLY

To the Editor: The patient described in our case report1 hada 9q22 deletion and not a 9p22 deletion, as reported in themanuscript.We express our regret for the error in theman-uscript and appreciate our colleague’s diligence in pointingthis out. We would like to confirm that the case describedwas associated with Robinow syndrome, as discussed.BothPeters anomaly and sclerocornea are anterior segment

disorders with variable and possibly overlapping generalphenotypic features. However, there are distinct pathologicfeatures that separate these 2 entities.2,3 The hallmark ofPeters anomaly, as seen in the case described, is the centralabsence of the corneal endothelium, Descemet’s membrane,posterior corneal stromal thinning, and central cornealstromal lamellar disorganization. Also, iridocorneal andkeratolenticular apposition may be observed in the area ofabsent Descemet’s membrane. In contrast, the pathologicfeatures of sclerocornea are highlighted by the replacementof the normal lamellar arrangement of corneal collagen bycoarse irregularly arranged thick collagen fibers thatresemble scleral collagen. This rearrangement and changein collagen fiber size is likely influenced by cornealproteoglycans and their core proteins.4Histological examina-tion of both corneas in the neonate in this case report showednormal collagen lamellar arrangement except in the centralcornea of the left eye. In addition, although not always thecase, sclerocornea does tend to be bilateral2,3 and ourpatient had a normal right cornea.We would also like to clarify the comment made about

the funduscopic examination of the left eye. As mentionedin the original manuscript, this was not performed becauseof the central corneal opacity in the left eye but the rightfundus was unremarkable. It was only on histologicalexamination that the retina of the left eye was noted to bewithout pathology.The clinical findings of our case differ significantly to the

case report by Ying and colleagues5 mentioned with the9q22 deletion especially in terms of the skeletal and abnor-malities of the extremities. The infant demonstratedextensive skeletal and extremity abnormalities but ourneonate had a normal skeletal survey.In conclusion, we believe that our case report is unique

because it does describe a case of Peters’ anomaly associatedwith a new gene defect. However, it is clear that a defect in9q22 creates a similar spectrum of anterior segmentabnormalities with histologically distinct features.

Nancy Hanna, MDDeepak P. Edward, MD

Summa Health System, Akron, Ohio

Journal of AAPOS

References

1. HannaNN, Eickholt K, Agamanolis D, Burnstine R, EdwardDP. Atyp-icalPetersplus syndromewithnewassociations. JAAPOS2010;14:181-3.

2. Idrees F, Vaideanu D, Fraser SG, Sowden JC, Khaw PT. A review ofanterior segment dysgeneses. Surv Ophthalmol 2006;51:213-31.

3. Harissi-Dagher M, Colby K. Anterior segment dysgenesis: Peters’anomaly and sclerocornea. Int Ophthalmol Clin 2008;48:35-42.

4. Young RD, Quantock AJ, Sotozono C, Koizumi N, Kinoshita S. Sul-phation patterns of keratan sulphate proteoglycan in sclerocornea re-semble cornea rather than sclera. Br J Ophthalmol 2006;90:391-3.

5. Ying KL, Curry CJ, Rajani KB, Kassel SH, Sparkes RS. De novo inter-stitial deletion in the long arm of chromosome 9: A new chromosomesyndrome. J Med Genet 1982;19:68-70.

doi:10.1016/j.jaapos.2010.09.002J AAPOS 2010;14:561.Copyright � 2010 by the American Association for Pediatric Ophthalmology and

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ORALREADINGAFTERTREATMENTOFDENSE CONGENITAL UNILATERALCATARACT

To the Editor: Birch and colleagues1 tested monocular read-ing rate, accuracy, fluency, and comprehension in 18 chil-dren treated for dense unilateral congenital cataract andcompared them to 55 normal children. They reported that6 treated eyes with a visual acuity equal to or better than0.3 logMAR (20/40) developed “normal reading ability”;however, their data do not fully support this conclusion.

The 6 eyes with excellent vision recovery were comparedto the entire group of 55 normal children.They should havebeen compared in a case-control fashion to age-matchedchildren with the same degree of educational accomplish-ment.This does notmean the same grade level.One generalcriticism of this study is that subjects were tested at gradelevel and 1 grade higher, but there is a wide range of readingachievement within a grade level, ranging from a year aboveto a year below (Linda Smutz,MSCurriculum and Instruc-tion, personal communication). Mild educational lossesover the summer are typical and could have affected theperformance of children tested in August as opposed toMay or June. These effects would broaden the score rangesin normal children, particularly fluency above grade level,as can be seen in Figures 1 and 2 in the article. Consideringthe effort required to achieve 20/25 visual acuity in an infantwith unilateral aphakia, it is reasonable to suppose thatthese patients had particularly attentive parents who wouldalso ensure that their children performed scholastically tothe best of their abilities. The combination of a smallnumber of subjects in the disease cohort, a large interquar-tile range in normals, and subtle confounding factors makeit possible to find “no difference” when one exists.

Birch and colleagues also do not adequately address thecontradiction between their results and 3 earlier studies ofeyes with strabismic or refractive amblyopia and excellentvision recovery,2-4 which showed persistent readingdeficits. I would not predict aphakic amblyopia to have

562 Letters to the Editor Volume 14 Number 6 / December 2010

a better prognosis than refractive amblyopia. I would alsobe interested to read a report that considered which eyewas amblyopic, since right eyes make temporal saccadesto read and left eyes make nasal saccades. Finally, I ampuzzled by the general lack of interest2 in the relevantfunctional parameter, binocular reading ability.5

Sandra Brown, MDCabarrus Eye Center, 201 LePhillip Ct., NE, Concord

North Carolina

References

1. Birch EE, Cheng C, Vu C, Stager DR Jr. Oral reading after treatmentof dense congenital unilateral cataract. J AAPOS 2010;14:227-31.

2. Repka MX, Kraker RT, Beck RW, Cotter SA, Holmes JM,Arnold RW. Monocular oral reading performance after amblyopiatreatment in children. Am J Ophthalmol 2008;146:942-7.

3. Zurcher B, Lang J. Reading capacity in cases of “cured” strabismicamblyopia. Trans Ophthalmol Soc UK 1980;100:501-3.

4. Stifter E, Burggasser G, Hirmann E, Thaler A, Radner W. Evaluatingreading acuity and speed in children with microstrabismic amblyopiausing a standardized reading chart system. Graefes Arch Clin ExpOphthalmol 2005;243:1228-35.

5. Rubin GS, Munoz B, Bandeen-Roche K, West SK. Monocular versusbinocular visual acuity as measures of vision impairment and predictorsof visual disability. Invest Ophthalmol Vis Sci 2000;41:3327-34.

doi:10.1016/j.jaapos.2010.09.005J AAPOS 2010;14:561-562.Copyright � 2010 by the American Association for Pediatric Ophthalmology and

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REPLY

To the Editor:Dr. Brown raises several issues related to oneaspect of our study,1 the comparison of reading perfor-mance of the subset of treated eyes with 20/40 or bettervisual acuity outcomes with the performance of normalcontrol subjects. She argues that because there is a widerange of reading levels within a grade level we shouldhave matched the control group to the patient group onthe basis of educational accomplishment and time of yeartested.

We agree that a case-control designmay be appropriate toassess reading achievement, but that was not the goal of ourstudy. Our stated aim was to determine the “utility of thetreated eye for reading in the event of injury to the soundeye.”1 We operationally defined utility for reading as theability to read at grade level and at 11 above grade level.Each child provided his or her own control via statisticalcomparison of reading performance on 2 passages read bythe treated versus fellow eye. As can be seen in Figure 2,1

reading performanceongrade level and11 above grade levelpassages was remarkably similar for treated eyes with 20/40or better visual acuity and their fellow eyes. Also, Figure 2shows that the median rate, accuracy, and comprehensionscaled scores for treated eyeswere all near 4, indicative of flu-ent reading with excellent comprehension. As discussed inthe our article,1 the normal control group was included pri-marily because we used an abbreviated, nonstandardmethod

to administer the GORT-4; they provided a benchmark forthe abbreviated test method.

Dr. Brown suggests that there may be a contradictionbetween the present study and previous reports of persistentreading deficits associatedwith treated strabismic amblyopiadespite excellent visual acuity outcomes.2-4 However, unlikedeprivation amblyopia, strabismic amblyopia is associatedwith abnormal contour interactions,5 hyperopia and abnor-mal accommodation,6,7 and visuocognitive deficits.8-10 Anyor all of these characteristics of strabismic amblyopiamay affect the higher cortical function beyond letterrecognition that is required for fluent reading in ways thatare different from deprivation amblyopia.

Finally, Dr. Brown is puzzled by our lack of interest inbinocular reading ability as an outcome measure in thisstudy. Although binocular reading ability would certainlybe a worthwhile functional measure to study in this popu-lation, it would not have allowed us to evaluate the utility ofthe treated eye for reading in the event of injury to thesound eye.

Eileen Birch, PhDRetina Foundation of the Southwest

University of Texas Southwestern, Medical CenterDallas, TX

David R. Stager Jr, MDPediatric Ophthalmology & Adult Strabismus

Plano, TX

References

1. Birch EE, Cheng C, Vu C, Stager DR Jr. Oral reading after treatmentof dense congenital unilateral cataract. J AAPOS 2010;14:227-31.

2. Repka MX, Kraker RT, Beck RW, et al. Monocular oral readingperformance after amblyopia treatment in children. Am JOphthalmol2008;146:942-7.

3. Zurcher B, Lang J. Reading capacity in cases of “cured” strabismicamblyopia. Trans Ophthalmol Soc U K 1980;100:501-3.

4. Stifter E,BurggasserG,HirmannE, et al. Evaluating reading acuity andspeed inchildren with microstrabismic amblyopia using a standardizedreading chartsystem. Graefes Arch Clin Exp Ophthalmol 2005;243:1228-35.

5. Levi DM. Crowding—an essential bottleneck for object recognition:A mini-review. Vision Res 2008;48:635-54.

6. Ingram RM, Lambert TW, Gill LE. Visual outcome in 879 childrentreated for strabismus: Insufficient accommodation and vision depriva-tion, deficient emmetropisation and anisometropia. Strabismus 2009;17:148-57.

7. Atkinson J, Braddick O, Robier B, et al. Two infant vision screeningprogrammes: prediction and prevention of strabismus and amblyopiafrom photo- and videorefractive screening. Eye (Lond) 1996;10(Pt 2):189-98.

8. Atkinson J,NardiniM, Anker S, BraddickO,Hughes C, Rae S. Refrac-tive errors in infancy predict reduced performance on the movementassessment battery for children at 3 1/2 and 5 1/2 years. DevMedChildNeurol 2005;47:243-51.

9. Atkinson J, Anker S, Nardini M, et al. Infant vision screening predictsfailures on motor and cognitive tests up to school age. Strabismus2002;10:187-98.

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