Title: Fitting Gas Permeable Contact Lenses on Aphakic Infants with Congenital Cataracts: Case Report

Transcription

1 Title: Fitting Gas Permeable Contact Lenses on Aphakic Infants with Congenital Cataracts: Case Report I. Case History Patient demographics: 9-month-old Caucasian female Purpose of visit/chief Complaint: Contact lens fitting on aphakic eye (OD) Ocular history: 1. Congenital Cataract, OD 2. Monocular nystagmus, OD 3. Dense Deprivation amblyopia, OD Medical history: 1. Spinal Muscular Dystrophy 2. h/o bilateral femur fracture 3. Club foot of both feet 4. Congenital vertical talus deformity 5. Congenital joint contractures 6. Feeding problem Ocular Medications: Vigamox BID OD; Atropine QD OD; Prednisolone Acetate BID OD; Tobradex BID OD Systemic Medications: Glycerin (bulk) solution Acetaminophen (Tylenol) II. Pertinent findings Visual Acuity/Fixation: OD: Central, Unsteady, Unmaintained. OS: Central, Steady, Maintained. (strong fixation preference detected per induced tropia test using 16 pd BD) Cycloplegic Refraction: OD: OS: x090 Pertinent Anterior Segment findings: Cornea OD: superior wound well apposed Lens OD: aphakic

2 IOP: 11/12 mmhg with icare Posterior segment findings: OD: c/d 0.10 round, macula flat, evenly pigmented OS: c/d 0.10 round, macula flat, evenly pigmented Peripheral retina flat and attached 360 OU All else unremarkable Patient was fit with a GP lens made of Menicon Z material (Menicon, Japan) using the Dyna Intralimbal design (Lens Dynamics, CO) Trials: BC/Power/Diameter and Observations # 1: 7.50/ +24/10.4: too steep ~2D central bubble # 2: 7.80/ +24/10.4: D steep still. Plano over-retinoscopy # 3: 8.00/ +24/10.4: inferior bubble with slight central bearing- flat # 4: 8.20/ +24/10.4: slight superior central bearing and inferior bubble Dispensed from spare set 7.85/+27.00/10.4 after successful I and R training completed by the parents. The intended over-refraction for aphakic children at this age is D to provide the best clarity for the infant at a near focal point. Final: DIL:MZ to be ordered: 7.90/+27.50/10.2 III. Differential diagnosis Primary/leading Congenital Cataract OD Fitting Options: Spectacles Soft contact lens Rigid gas permeable Scleral lenses Intra-ocular lens After discussing with the family the different treatment options, the parents elected to try rigid gas permeable lenses.

3 IV. Diagnosis and discussion Congenital cataracts are a treatable vision impairment that affects approximately per 10, in the United States and 3 per 10,000 in the United Kingdom 5. The etiologies of congenital cataracts are diverse with idiopathic being the most common cause. Other causes include familial, autosomal dominant, galactosemia, persistent hyperplastic primary vitreous, rubella, salt and pepper chorioretinitis, microphthalmos, and Lowe syndrome. The primary concern with congenital cataracts is severe deprivation amblyopia. V. Treatment, management Congenital Cataracts are treated with the surgical removal of the cataract in a timely manner to avoid deprivation amblyopia. There are relatively high risks associated with infants that undergo anesthesia and surgical procedures. Vishwanath et al and Khan et al demonstrate case reports which show a higher incidence of glaucoma occurrence in infants who underwent cataract extraction earlier than 4 weeks 10,18. However, Birch and Stager demonstrated that if the cataract extraction was delayed beyond 6 weeks, than the visual prognosis was worse 2. Immediately following cataract extraction, patients are either fit into spectacle correction, soft contact lenses, rigid gas permeable lenses or intra-ocular lenses 1. Spectacle corrections aren t usually chosen because of the poor optical performance, cosmesis, and aniseikonia with monocular aphakia. The infant aphakia treatment study is a prospective, randomized control trial aimed to evaluate and compare intra-ocular lenses and contact lenses. There was no significant difference between mean visual acuity of both groups at 1 year of age with grating acuity and 4.5 years with HOTV optotype acuity 6,8. There was 5-fold increase in additional intraocular operations in the IOL group, most of them due to opacities which developed in the first 3-6 months after IOL implantation 8. The theory behind the lower rate of lens reproliferation into the visual axis and pupillary membranes were thought to be because the anterior and posterior capsular bag fuse together prevents the lens material from migrating. The IOL provides a scaffold for the remaining lens epithelial cells to reproliferate, especially in infants 6. Glaucoma developed in 18% of the patients with no difference between pseudophakic and aphakic eyes 4. The risk of developing glaucoma or being a glaucoma suspect was 31%. There was a 3.2 times higher risk of developing glaucoma in the younger age group days old versus 48 days. However, the patients who had earlier surgery had a better median visual acuity. Ninetyfive percent of the glaucoma was primary open angle glaucoma.

4 Cataract extraction with a primary IOL implantation was 7% more expensive than treatment with contact lens ($27090 vs $25331) at age 5 years. However, the costs of supplies were more expensive with contact lens. The average number of contact lenses used by a patient was ten in the first year, nine in the second year, seven in the third year, and five in both the fourth and fifth year. Bibliography 1. Aausri MK et al. Management of Pediatric Aphakia with Silsoft Contact Lenses. The CLAO journal October; 25(4): Birch EE, Stager DR. The critical period for surgical treatment of dense congenital unilateral cataract. Invest Ophthalmol Vis Sci. 1996;37(8): Chen Y-CE et al. Long-term results of early contact lens use in pediatric unilateral aphakia. Eye & Contact Lens Jan; 36(1) Freedman SF et al. Glaucoma-related adverse events in the first 5 years after unilateral cataract removal in the infant aphakia treatment study. JAMA Ophthalmol. 2015; doi: /jamaophthalmol Holmes JM, Leske DA, Burke JP, et al. Birth prevalence of visually significant infantile cataract in a defined U.S. population. Ophthalmic Epidemiol 2003;10: The Infant Aphakia Treatment Study Group. A randomized clinical trial of HOTV optotype acuity at age 4.5 years and clinical findings at age 5 years. JAMA Ophthalmol. 2014;132(6): The Infant Aphakia Treatment Study Group. The Infant Aphakia Treatment Study. Arch Ophthalmol Jan; 128(1): The Infant Aphakia Treatment Study Group. A randomized clinical trial comparing contact lens with intraocular lens correction of monocular aphakia during infancy. Arch Ophthalmol. 2010;128(7): Kruger et al. Cost of intraocular lens versus contact lens treatment after unilateral congential cataract surgery in the infant aphakia treatment study of age 5 years. Ophthalmology 2015; 122: Khan AO, Al-Dahmesh S. Age at the time of cataract surgery and relative risk for aphakic glaucoma 11. Lambert et al. The infant aphakia treatment study: further on intra- and postoperative complications in the intraocular lens group. Journal of AAPOS April; 19(2): Lin et al. Update on pediatric cataract surgery and intraocular lens implantation. Curr Opin Ophthalmol. 2010; 21: Plager DA et al. Complications in the first 5 years following cataract surgery in infants with and without intraocular lens implantation in the infant aphakia treatment study. American Journal of Ophthalmology. 2014; 158(5): Rahi JS, Dezateaux C; British Congenital Cataract Interest Group. Measuring and interpreting the incidence of congenital ocular anomalies: Lessons from a National Study of Congenital Cataract in the UK. Invest Ophthalmol Vis Sci 2001;42:

5 15. Saltarelli DP. Hyper oxygen-permeable rigid contact lenses as an alternative for the treatment of pediatric aphakia. Eye & Contact Lens. 2008; 34(2): Sindt CW. Fitting infants and young children with GP lenses. Contact Lens Spectrum January. 17. Vishwanath M, Cheong-Leen R, Taylor D, Russell-Eggitt I, Rahi J. Is early surgery for congenital cataract a risk factor for glaucoma? Br J Ophthalmol. 2004; 88(7): VI. Conclusion Clinical pearls Both contact lenses and IOL implantations are viable options in the treatment of infant aphakia Treatment options should be thoroughly explained with the family, including benefits and risks of each option Optometrists should be equipped and prepared to fit aphakic babies Pediatric gas permeable fitting sets Blue LED micro flashlight (ex Ize Inova Microlight) Loose lens retinoscopy Compliance with patching and contact lens is equally important for a good outcome Early cataract removal usually results in better visual prognosis, however, it is linked with increased risk of glaucoma