significant ocular findings in siblings included any of the follow-ing: spherical refractive error � �3 D in either eye; astigmatism� 2 D in either eye; anisometropia � 1 D; and/or any strabismus.A �2 test was performed to ensure there were no significantdifferences across family size. Prevalence of above criteria wascomputed with confidence intervals weighted by family size.Results: In siblings 42.0% (33.0-50.9%) had significant ocularfindings: strabismus, 14.8% (8.3-21.3%) compared with 4% inthe general population; hyperopia � 3 D, 37.0% (27.3-46.7%);astigmatism � 2 D, 6.4% (2.0-10.8%); and anisometropia � 1,8.9% (3.1-14.7%). Discussion: Prior studies have relied on pa-tient/parent reported family history of strabismus to determinethe prevalence of strabismus among family members. This was aprospective study which further supports The American Acad-emy of Ophthalmology’s preferred practice pattern recommend-ing screening for all children with a family history of strabismus.Conclusions: Siblings of children with accommodative esotropiahave a higher prevalence of strabismus and amblyogenic riskfactors. Parents and insurers should continue to be informedregarding the importance of adhering to the referral policiesadvocated by the Academy.

The incidence of orthoptic complications in patients withepidermolysis bullosa. Kate A.V. Smith, Sophie M. Jones,Jemima Mellerio, Anna Martinez, Ken K. NischalIntroduction: Epidermolysis bullosa (EB) is a genetic disorderresulting in blistering of the skin and mucous membranes. Theoccurrence of ocular surface complications in EB patients is welldocumented. There are, however, no reports on the incidence oforthoptic complications in these patients. Methods: Fifty-fiveconsecutive EB patients underwent examination including visualacuity (VA), cover test, ocular motility, cycloplegic refraction,and fundus examination. Results: Thirty-five percent of patientshad reduced VA (0.3 logMAR or less) in at least one eye, and18% had bilaterally reduced VA. Twenty-nine percent of pa-tients had refractive errors; 22% hypermetropia, 6% myopia, and16% astigmatism (�1 DC). Eleven percent of patients had an-isometropia (�1 DS). Thirteen percent of patients had a constantor intermittent tropia; 9% exotropia and 4% esotropia. Twenty-five percent of patients had a significant phoria (�10�); 24%exophoria, 2% esophoria. Eleven percent had clinically signifi-cant convergence insufficiency. Discussion: This cohort of EBdemonstrated a high prevalence of reduced VA, strabismus, andrefractive errors compared with age-matched normals. The in-creased incidence of hypermetropia may be explained by theocular surface changes in some cases. Conclusions: In view ofthe improved survival of EB patients, visual morbidity is ofincreasing importance. This study highlights the need not onlyfor careful ocular surface surveillance of children with EB, butalso for careful orthoptic assessment.

Transsclerally sutured intraocular lens placement inpediatric Marfan syndrome. Michael C. StruckIntroduction: Surgical correction of subluxated lens in Marfansyndrome requires leaving the patient aphakic or implanting alens in the absence of capsular support. In the absence of capsularsupport the options include placement of an ACIOL or trans-scleral fixation of a PCIOL. We report our experience withPCIOL fixation in six patients with Marfan syndrome. Methods:This is a retrospective case review. Results: Eleven eyes of sixpatients were treated with lensectomy, vitrectomy, and primarytransscleral fixation of PCIOL. All children had dislocated lenses

or aphakia related to Marfan syndrome. Average age at surgerywas 8.8 years (39 to 179 months). Average follow-up was 21months (9-50 months). Best-corrected distance acuity rangedfrom 20/30 to 20/100 preoperatively (average, 20/60). Best-corrected postoperative vision ranged from 20/15 to 20/30 (av-erage, 20/20). All patients’ BCVA improved a minimum of threelines, and no patient lost vision. Complications included sutureerosion (1), vitreous wick (1), postoperative hyphema (1), andtemporary IOL capture (3). Five reoperations were performedfor complications secondary to IOL placement. No patient ex-perienced lens tilt or decentration, suture failure, CME, or ret-inal detachment. Conclusions: Transscleral suture fixation ofposterior chamber IOL in Marfan syndrome is successful inrestoration of visual acuity. There is a trend toward improvedbinocular function. All patients included in this study had failedstandard optical rehabilitation and required intraocular surgeryat the time of the implant surgery. Complications required areoperation rate of 45%.

Evaluation of vision-specific quality of life in albinism. C.Gail Summers, Beth R. Kutzbach, Kimberly Merrill, KathyHogue, Sara Downes, Ann M. HolleschauIntroduction: Office measurements of visual acuity do not nec-essarily reflect daily visual function and health status. We eval-uated vision-targeted health-related quality of life (QOL) inalbinism with a self-administered tool. Methods: Consentingparticipants completed the NEI Visual Function Questionnaire(NEI-VFQ-25) consisting of 25 questions about QOL and 12questions about well-being. Best-corrected binocular visual acu-ity (BCVA) and type of albinism were recorded. Results: Four-teen males and 25 females with albinism completed the study.Median age was 30 years (range, 18 to 79 years). Mean BCVAwas 20/95 (range, 20/20 to 20/320). Eighteen (46%) reportedthat they were currently driving. Participants perceived moderateproblems with their general vision (mean subscale score, 58.97).BCVA was moderately correlated with overall NEI-VFQ-25scores (r � 0.443). Most notable impairment was noted ondistance acuity, vision-specific mental health, and vision-specificrole difficulties subscales (mean subscale scores, 67.09, 67.07, and68.27, respectively). Differences by gender were insignificant.Ceiling effect �80% was noted for ocular pain, vision-specificdependency, and color vision. Discussion: Self assessment ofQOL with the NEI-VFQ-25 has been used in groups withspecific vision disorders. The vision-related results reportedherein are similar to those with moderate to severe diabeticretinopathy, although those with albinism reported better gen-eral health. Conclusions: The NEI-VFQ-25 is a valuablemethod to evaluate self-reported effects of vision-related QOL inalbinism.

Comparison of cycloacceptance versus postmydriatic testfor pediatric refraction. Meenakshi Swaminathan, ShaliniKaul, Thandalam S. Surendran, Sumita Agarkar, Sujata GuhaIntroduction: Cycloplegic refraction is an essential part of pe-diatric eye examination. Post mydriatic test (PMT) is used inolder children a few days following cycloplegic refraction, torefine the refraction subjectively. Aim: Accuracy of the subjectiverefraction performed soon after cycloplegic refraction, termedcycloacceptance, was compared with the PMT. Methodology:Prospective: from February 2004 to May 2006. At the SankaraNethralaya Orbis Pediatric Ophthalmology learning and trainingcenter, Chennai, India. Children ages 6 to 16, with no binocular

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anomalies and vision of 6/18 or better in each eye. Results:Seven hundred eight eyes of 354 subjects were analyzed. Theaverage of sphere (0.41) and spherical equivalent (0.00) aftercycloacceptance were compared with the average of sphere (0.23)and spherical equivalent (�0.20) at PMT. The difference wasfound to be statistically significant by paired t-test (0.02 and 0.04,respectively). The whole cohort was classified into low, moder-ate, and high myopia and hyperopia. The difference in sphereand spherical equivalent was significant only in hyperopia of alldegrees. Discussion: The difference in hyperopes may be attrib-uted to variation in accommodative status between the hyperopeand the myope. Conclusion: PMT may be considered in hyper-opes since there was low agreement between cycloplegic accep-tance PMT.

Amblyopic children identified by photoscreening respondwell to treatment. Ronald G.W. Teed, Christina M. Bui,David G. Morrison, Robert L. Estes, Chun Li, Sean P.DonahueIntroduction: Photoscreening programs have been shown toidentify children with amblyopia or amblyogenic risk factors.This study serves to evaluate the effect of therapy on amblyopicchildren identified through a community outreach photoscreen-ing program. Methods: Retrospective chart review of 510 chil-dren referred to one academic pediatric ophthalmology groupfrom a photoscreening program. Children diagnosed with am-blyopia were treated with standard therapy, including spectacles,patching, and/or atropine penalization. Successful treatment ofamblyopia was defined as two lines or greater of improvement invisual acuity, equal visual acuity among both eyes, or equalfixation preference in preverbal children. Results: Of the 510photoscreen-positive children, 110 were diagnosed with ambly-opia, 288 had AAPOS-defined risk factors without amblyopia, 82were false-positives without amblyopia or amblyogenic risk, and30 were excluded. Of the 110 with amblyopia, 84 (76%) weresuccessfully treated, with a mean follow-up time of 34 � 23months. Of the remaining 26 patients, 75% are still being ac-tively treated. Additional follow-up will be updated prior topresentation. Discussion: The treatment of amblyopia in chil-dren identified through photoscreening is successful in a majorityof cases. Conclusions: Photoscreening programs are not onlycapable of identifying at-risk children; in addition, those childrenwith amblyopia can be successfully treated. Implementation ofsuch vision screening programs can be a useful adjunct in theprevention of amblyopia-related vision loss.

Functioning avascular retinae—a report of two siblings. SriThyagarajan, Daniel M. Gore, Alki Liasis, Ken K. NischalIntroduction: We present two siblings who, despite havingcomplete retinal vessel absence, have functional retinae. Meth-ods: Retrospective case reports Results: Two sisters, aged 3 (SK)and 5 (AK), of consanguineous parents, presented with poorlycontrolled congenital glaucoma. AK had a right phthisical eyeand uncontrolled glaucoma in the left. She demonstrated de-graded electroretinograms (ERG) in the right eye but no con-sistent flash visual-evoked potentials (VEP). SK had uncon-trolled glaucoma and dense cataract in the left eye with moder-ately controlled glaucoma in the right eye. She demonstratednormal ERG in the right eye but no consistent responses in theleft eye. Both children had microspherophakia, marked iris hy-poplasia, and anomalous dentition. Homocystinuria was excludedserologically. AK had a right vitreolensectomy with subsequent

control of glaucoma with topical Cosopt®. SK underwent com-bined left trabeculectomy and trabeculotomy with mitomycin C,with subsequent lensectomy. The right eye glaucoma was con-trolled topically as for AK. Clinically SK was able to navigate infamiliar surroundings and play with toys close to her face. Bothchildren showed absence of retinal vasculature. Discussion:These are the first reported cases to the best of our knowledge offunctioning avascular retinae. AK has little or no vision second-ary to glaucoma, although ERG responses are present. SK hasfunctioning vision with normal ERG in the right eye. Conclu-sions: Physiologically avascular retinae occur in the mammalianworld in which the entire metabolic demands are met by thechoroidal circulation. These cases may represent a similar entityand in so doing contravene the accepted hypothesis that anavascular retina precludes functional vision in humans.

Lens extraction in children with Marfan syndrome. Elias I.Traboulsi, Sue CrowePurpose: To report the outcome and technique of lensectomyusing a clear cornea approach in 15 eyes of patients with Marfansyndrome. Methods: Chart review of patients with Marfan syn-drome who underwent surgery for subluxated lenses. Pre- andpostoperative visual acuity, phakic and aphakic refractions, andcomplications were recorded. Results: Patients ranged in agefrom 62 to 157 months. Best-corrected phakic visual acuity (noneof the children could wear aphakic correction) ranged from 20/50to 20/130. Postoperative visual acuity using glasses or contactlenses was 20/40 or better in all patients with a follow-up of 9 to48 months. One patient developed a retinal detachment after aninjury to the aphakic eye; this was repaired successfully witheventual visual acuity of 20/30. Discussion/Conclusions: Ananterior approach clear cornea lensectomy in children withMarfan syndrome appears to be safe with significant improve-ment of vision in all patients.

Magnitude of ocular problems in school children of lowsocioeconomic strata in Delhi. Kanak Tyagi, Gaurav Kakkar,Abhishek Dagar, Anil TaraPurpose: This study was aimed to assess the magnitude and typeof eye problems in school children of low socioeconomic strataand provide specialized services. Methods: We examined 45,075children aged 6 to 15 years from formal and nonformal (slum)schools of Delhi. The team was composed of one ophthalmolo-gist, three ophthalmic assistants, and one school coordinator, allsupervised by a pediatric ophthalmologist. The examination in-cluded recording visual acuity of each eye separately, assessmentof binocular function, and anterior segment examination, whichwas done by an ophthalmologist. Any child having visual acuityof less than 6/12 in one eye was subjected for cycloplegic refrac-tion. Direct ophthalmoscopy was done whenever necessary. Re-sults: 45,705 children were examined and 6594 (14.6%) hadocular problems. Of these, 3498 (53%) had refractive error andthe rest (3096; 47%) had other ocular disorders. The ocularproblems included eyelid problems in 6.23% cases, conjunctivitisand corneal problems in 9.28% cases, Vitamin A related con-junctival xerosis in 1% cases, cataract in 0.3% cases, strabismus in2.3% cases, and convergence insufficiency in 20.98% cases.Other problems were retinal problems, amblyopia, and pseu-dophakia in 3.92% cases. Glasses were provided to 2199 childrenand medicines provided to needy. Children who needed inter-vention were referred to Venu Eye Institute and Research Cen-tre. Conclusions: School screening programs mainly stress vi-

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