Nothing to Disclose Course Goals

Nothing to Disclose

Pediatric Anterior Segment Conditions Marie I. Bodack,OD, FAAO, FCOVD, Dipl BVPPO Chief, Pediatric Primary Care @ Southern College of Optometry [email protected]

Course Goals

• To review commonly encountered pediatric anterior segment conditions and their treatment options. • To become comfortable with FDA age guidelines and dosing for ophthalmic medications including orals • To become comfortable in treating pediatric anterior segment disorders • To instill clinical ‘pearls’ for examining children

Capillary Hemangioma Capillary Hemangioma

• Benign, Soft Tissue Tumor composed of blood • Location: palpebral conjunctiva, upper lid vessels • Bright red mass (superficial) • Most common orbital tumor of childhood • Blanch on pressure • 1/200 births • May bleed spontaneously • Females:Males 3:2 • When crying may enlarge • 30% at birth, 100% by 6 months • Rapid growth in first 12 months of life • Most spontaneously regress by age 5/6 • 30% age 3 • 60% age 4 • 76% age 7

1 Capillary Hemangioma Capillary Hemangioma - Evaluation

• Retinoscopy • Concerns • Cardiac/neurological workup & CT/MRI of orbit • Amblyopia 25‐60% • If extensive facial dysmorphia = may be orbital • Occlusion of visual axis by lid • Posterior fossa malformation • Induced corneal astigmatism • Hemangioma • Arterial anomalies • Cardiac defects • Eye abnormalities

Capillary Hemangioma - Treatment • Correct refractive error • Treat amblyopia • Steroid injection • Regression in 2‐4 weeks • Oral Beta blocker (propanolol ‐1‐2mg/kg/day) • Mechanism: vasoconstriction, reduction in pro‐ angiogenic signals • Topical timolol maleate 0.25% bid Pope E et al, Topical Timolol Gel for • Surgical excision Infantile Hemangiomas: A Pilot Study. • If severe (cosmesis), amblyopia Arch Dermatol. 2010;146(5):564-565.

Ptosis • Unilateral or bilateral • Etiology • Congenital • Acquired • CNIII Palsy • Systemic • Myasthenia Gravis • Trauma • Mechanical • Chalazion/Hordeolum • Hemangioma www.aapos.org

2 Congenital Ptosis

• Signs • Levator function • Upper eyelid fold • Concerns • Amblyopia • Deprivation • Induced astigmatism

Horner Syndrome Neuroblastoma

• Triad • Most common extracranial solid tumor in • Anisocoria dim>bright infants/children • Etiology: disruption of sympathetic nerve • 8‐10% childhood cancers supply • Overall incidence 14/100,000 • Congenital • 85% < age 5 • Iris color lighter • Abdomen • Acquired • Adrenal gland • Neuroblastoma (children) • Can metastasize • Pancost tumor of lung (adults)

Ptosis Evaluation Ptosis - Treatment

• Observation • Congenital • Frontalis • Treat Amblyopia • Head position • If vision deprivation or cosmetic concerns • Work Up • Surgical correction • Palpebral aperture • Acquired • Upper lid fold • Treat condition • Pupils • Myasthenia • Pearl: Direct ophthalmoscope • Neuroblastoma • Iris color • Ice pack test • MRI abdomen

3 Chalazion/Hordeolum

• Treatment • Hot compresses • Topical Medications • Surgery? • Size • Duration • Risk of occlusion amblyopia • Anesthesia • Omega 3s • Oral Antibiotics?

Steroid Antibiotic Combination Chronic Treatment

• TobraDex (Dexamethasone 0.1%,Tobramycin 0.3%) •Omega 3 • Ages 2+ • gtts (generic) and ung • Adult dose 2‐1000 mg tid • TobraDex ST (Dexamethasone 0.3%, Tobramycin 0.05%) •Pediatric Availability • Ages 2+ • Zylet (Loteprednol 0.5%,Tobramycin 0.3%) • Chewable daily • Blepharitis and lid inflammation 0‐6 years • Maxitrol (Dexamexasone 0.1%,Neomycin,Polymixin B) • Ages 2+ • gtts and ung

Macai MS. The role of omega-3 dietary supplementation in blepharitis and meibomian gland dysfunction (an AOS thesis). Trans Am Ophthalmol Soc. 2008;106:336-56.

Oral Antibiotics

• Erythromycin Ethylsuccinate (EES) – 30‐50mg/kg/day q 6 h – Formulations: 200/400 mg/5ml • Dicloxacillin – 12.5‐50mg/kg/day q 6 h • Amoxicillin – 25‐45mg/kg/day q12 h – 20‐40 mg/kg/day q8h – Formulations: 125/200/250/400 mg/5ml • Tetracycline (Ages 8+) – 250 mg qid

4 Preseptal Cellulitis Preseptal Cellulitis

• Infection of eyelid anterior to septum • Examination • 5x more common than orbital • VA • Causes • Pupils • Lid/cutaneous infections • EOMS • Hordeolum/Impetigo • Slit Lamp • HSV/HZV • Treatment • URI/Sinusitis • Oral Medications • Differentials • Referral? • Allergic • Adenovirus (16%)

Oral Antibiotics Oral Antibiotics • Amoxicillin, Clavulanate (Augmentin) • 20‐40mg/kg/day q 8h • Formulations: 125/200/250/400mg/5ml •Sulfa Allergy Precautions • Cephalexin (Keflex) – 1st gen •No tetracycline < 8 years • 25‐50mg/kg/day q 6h • Formulations: 125/250/500 mg/5ml •PCN Allergy Precautions • Cefaclor (Ceclor) – 2nd gen st • 20‐40mg/kg/day q 8h • Cross‐reactive to cephalosporins (1 gen)? • Formulations: 125/187/250/375 mg/5ml • Cefdinir (Omnicef) – 3rd gen • 7 mg/kg/day q 12 hr • Formulations: 125/250 mg/5ml • Trimethoprim/Sulfamethaxole (Bactrim) • 8‐12/40‐60mg/kg/day q12h

Oral Antibiotics Dosing • PCN Allergy • Cross‐reactivity with cephalosporins • Follow FDA Guidelines • Need to know • Child’s weight in kg • Formulations of medication • 1 tsp = 5ml

Apter AJ et al. Am J Med 2006;119, 354 Campagna JD et al. J Emerg Med 2012;42:612

5 Example 5 y/o preseptal: Example 5 y/o preseptal: • 40 lbs = 18.14 kg • 40 lbs = 18.14 kg • Keflex • Augmentin – Dose 25‐50mg/kg/day q 12 h • 20‐40mg/kg/day tid – Formulations 125/250/400mg for 5 ml • Formulations 125/200/250/400mg for 5 ml • Determine min and max mg/day • Determine min and max mg/kg – 453.5 to 907 mg/day • 362.8 to 725.6 mg/day • Therefore : • Therefore : – Can do 250mg q 12 h (500mg daily) • Can do 125 (375 daily) or 200 (600 daily) – Can do 400mg q 12 h (800mg daily) – Can do 1.5 tsp (7.5ml) of 250mg (375) q 12 h (750mg • On Rx: daily) • Giving 5ml tid (15ml/day) for 10 days so dispense • On Rx: 150 ml – 5ml bid x 10 days = 100ml – 7.5ml bid (15ml/day) x 10 days = 150ml

Dermoid Cyst • Choroistoma • Congenital • Location: • Conjunctiva = Limbal dermoid • Firm • Color: variable • Concern • Associations • Goldenhar syndrome • Linear nevus sebaceous syndrome

Dermoid Cyst Dermoid Cyst - Treatment

• Lateral Canthus = Dermolipoma • Monitor • Ddx: prolapsed fat • Correct astigmatism • Amblyopia treatment • Surgical?

6 Allergic Conjunctivitis • Immediate hypersensitivity Type I reaction • Mast cell degranulation • Histamine and leukotriene release • Increased vascular permeability • Seasonal • Year round (Perennial)

Ocular Allergy – Antihistamines, Mast Cell Allergic Conjunctivitis Stablizers, & Combination – OTC • Signs: • Ketitofen 0.025% • Bilateral injection • Zaditor • Papillary reaction – bulbar conjunctiva • Alaway • Ages 3+ • Tearing • bid • “Allergic shiner” • Olopatadine 0.1 %0.2% (Pataday) • Symptoms • bid/qd • Ages

Antiallergy -Rx Antiallergy -Rx • Azelastine 0.05% (Optivar) • Cromolyn Sodium 4% • Olopatadine 0.1% (Patanol) (Crolom) • Loteprednol etabonate 0.2% (Alrex) • Olopatadine 0.7% (Pazeo) • Ages 4+ • 4‐6 times/day • daily • Safety information in pediatric patients • Vernal • Ages 2+ has not been established • Epinastine 0.05% (Elestat) • Lodoxamide 0.1% (Alomide) • Ages 2+ • Ages 2+ • • 2‐4 times a day up to 3 months Dosing: qid • Bepotastine 1.5% (Bepreve) • Vernal • Ages 2+ • Alcaftadine 0.25% (Lastacaft) Unless stated FDA approved for ages • Daily 3+ and bid • Ages 2+ • Emedastine 0.05% (Emadine) • Up to qid • Cetirizine 0.24% (Zerviate)

7 Vernal Conjunctivitis (VKC)

• IgE and T cell mediated • Association: asthma, eczema, seasonal allergic rhinitis • Males>Females (2‐3:1) • Onset <10 years (5‐6 years) • Spring • Symptoms

Vernal Conjunctivitis (VKC) Vernal Conjunctivitis - Treatment • Signs • Cobblestone Papillae • Ropy mucus discharge • Mast Cell Stabilizer • Injection • • Cobblestone Papillae Trantas Dots • Cornea • Steroid • Trantas dots • Shield Ulcer • Shield ulcer (5%) • Antibiotic drops • Oval • Superior • Plaque

Steroids • Lotoprednol etabonate 0.2% (Alrex) • Lotoprednol etabonate 0.5% (Lotemax) • Drops, ointment and gel • QID • Fluromethalone 0.1% (FML) • Fluromethalone 0.25% (FML Forte) • Ages 2+ • bid to qid • Prednisolone Acetate 1% (Pred Forte) • Use is supported by evidence • Difluprednate (Durezol) • 0 to 3 years of age s/p cat sx

Unless stated, no safety information for pediatric patients

8 Bacterial Conjunctivitis Bacterial Conjunctivitis

• 50% of cases of acute conjunctivitis • Symptoms • Gigliotti. 1981 J. Pediatrics • Signs • Pathogens • Bulbar/tarsal conjunctival injection • H. flu • Hemorrhagic with H. flu • S. pneumonia • Discharge • 39% concurrent ear infection • Treatment • < 5 years old • Culture? • H. flu • Topical Medication

Ointments vs. Drops? Antibiotics - Ointments • Ointment: • Erythromycin 0.5% (Ilotycin) – Neonatal prophylaxis • Easier Installation • Ciprofloxacin 0.3% (Ciloxan) – Up to 6 times/day • 2+ years • Longer lasting • Bacitracin (Ak‐Tracin, Bacticin) • tid x 2 days then bid x 5 – Safety in peds not established • Blurry vision days – 1‐3 times/day • Sulfacetamide 10%, 15% • Better for infants • Tobramycin 0.3% (Tobrex) • Rarely used • Drops: • 2+mos • 2‐3 times/day • No blur • Gentamicin 0.3% (Gentak) • More options – Safety in neonates not established • May sting – 2‐3 times/day – More toxic to cornea • Taste in mouth

Antibiotics - Drops Stevens-Johnson

• Life‐Threatening Skin Condition • Polymxin B+ • Aminoclycosides • Trimethoprim (Polytrim) • Tobramycin 0.3% Mucus Membranes • 2+mos • Gentamicin 0.3% • Etiology: Hypersensitivity to medications • Dose q3h (6x day) • Sulfacetamide 10%, 15% • Stings • Rarely used

9 Antibiotic Drops - Fluroquinolones • Ages (1+) Antibiotics–Azithromycin (AzaSite) • Q2h x 2 days then qid x 5 days unless stated • • Ciprofloxacin 0.3% FDA 1+ year old • Ofloxacin 0.3% • FDA bacterial conjunctivitis • Levofloxacin 0.5% • • Levofloxacin 1.5% – age 6+ Dosing • Gatifloxacin 0.3% (Zymar) • Off label use • Gatifloxacin 0.5% (Zymaxid) • Blepharoconjunctivitis • Moxifloxacin 0.5% (Vigamox) • tid x 7 days • Lid margin hyperemic • Moxifloxacin 0.5% (Moxeza) *4+mos • Conjunctival inflammation/injection • bid x 7 days • Sub‐epithelial infiltrates • Besifloxacin (Besivance) • tid x 7 days • Daily x 1 month; hot compresses • Resistance! • Alabiad CR, Miller D, Schiffman JC, Davis JL. Antimicrobial resistance profiles of ocular and nasal flora in patients undergoing intravitreal injections. Am J Ophthalmol. 2011 Dec;152(6):999‐1004

Viral Conjunctivitis • 20% cases acute conjunctivitis • Signs • Conjunctival injection • Follicles • Tearing • PAN • Testing? • Adenoplus® • serotypes 1, 3, 4, 5, 7, 8, 11, 14, 19, 31, 37 • Types • Simple adenovirus, PCF, EKC

Epidemic Keratoconjunctivitis (EKC) Viral Conjunctivitis - Treatment • Etiology: Adenovirus 8,19,37 • Signs • Mild cases • Lid • Tears • Pseudomembrane • Hygiene • Edema • Antibiotic/Steroid • Cornea • Epi/Subepi keratitis • EKC • Psedomembrane • Pseudomembrane/SEI – steroid • SEI • Isolation • Resolution • Extremely contagious • Hygiene!

10 Red Eye 12 y/o F

• History of red R eye treated by PCP, ER with tobramycin, ciloxan, vigamox • VA 20/60 OD PH 20/25, 20/20 OS • SLE as noted

Red Eye 12 y/o F Recurrent Red Eye

• Etiology • 8 WF referred by PCP for evaluation of • Treatment? red eye • Azithromycin 1g po x 1 dose • Saw PCP with 2 day hx of red eye • Doxycycline 100mg po bid x 7d • Rx ciprofloxacin ophthalmic qid • Erythromycin 500mg po qid x 7 d • 3 days later increased redness, light sensitive, unable to open eye • Pearl: Kids can have adult problems

Photo by Emily Shull, OD, Cincinnati Eye Institute Photo by Emily Shull, OD, Cincinnati Eye Institute

11 Herpes Simplex Virus (HSV) 1 Is HSV Different in Children? • 48% of children ‐ recurrence within 15 • Signs • Vesicular lesions on the lid months • Epithelial defect • 61% epithelial/stromal disease recurrent • Conjunctival injection stromal disease • Stromal opacities • Epithelial disease only? • Iritis • Stromal scarring (48%) • Symptoms • Amblyopia • Tearing • Pain • 26% both eyes ‐ concomitant or sequential • Photophobia • Misdiagnosis • None Chong et al. Am J Ophthalmol 2004: 138: 474-5 Liu et al. Ophthalmology 2012;119:2003

Is HSV Different in Children? HSV Treatment in Pediatrics • Lid lesions • Oral Acyclovir • Greater inflammatory response • Corneal dendrites • Amblyopia • Stromal disease • Epithelial disease • Taper PF to FML to Lotemax • Prophylaxis for recurrences • Pearl: You will have a Threshold dose • Prevent infectious disease if on steroid • Oral Acyclovir for chronic stromal disease • Active v. Inactive Schwartz&Holland. Ophthalmology 2000: 107, p 278-282 • Treat amblyopia

Topical Antivirals Antivirals (oral)

• Trifluorothymidine (Viroptic) • Acyclovir • FDA ages 6+ • All tid • Dosing: q2h up to 9 times/day • 18 mos – 3 years: 200mg (5ml) • Ganciclovir (Zirgan) • 3‐5 years: 300mg (7.5ml) • FDA ages 2+ • 6+ years: 400mg (10ml) • Dosing 5 times/day until heals then tid for 7 • Liu S, et al. Ophthalmology 2012;119:2003 days

12 Case Disposition

• Disposition • Pearl: • You can get flare ups

Corneal Abrasion

• Etiology: • Trauma • History • Exam pearl: • Work quickly • Get the information you need • Patient types

INOVA Blue LED Micro Flashlight Bluminator

Bear Hug Corneal Abrasion Treatment

• Topical Antibiotics • Pain medication (oral) • Cycloplegic • Bandage Contact Lens • Fox Shield • Follow Up • Daily

13 7 year old playing in a tree Exam Under Anesthesia • Local ED same day • Tree Branch remnant in inferior fornix • Dx: Corneal abrasion • 2mm Ulcer 20% depth • Rx: Polytrim qid • Treatment: • ED 3 days later • Amphotericin B (Ambisome) 1% q 1h • Drainage • Ceftazidime 50mg/ml q1h • Swelling • Vancomycin 25 mg/ml q1h • Mom “removed twig” • Unable to open eye patient, mom, MD, RN • Sent to Eye Clinic

Pearl Antifungal Drops

• You should be able to open the eyelid •Indication of a child with an abrasion. If you • Fungal infection can’t, further evaluation needed! • Organic matter (e.g. tree branch) • Non healing abrasion •Natamycin 5% • Dose q1h for 1 day then taper • Duration 4‐6 weeks • Safety in pediatric patients not established

Uveitis

• Etiology • Symptoms • Trauma • Injection • Idiopathic • Pain • Systemic disease • Photophobia • Sequelae: • White & quiet • Cataract eye • Glaucoma • No pain • Asymptomatic

14 Uveitis Treatment Topical Steroids • Prednisolone Acetate 1% (Pred Forte) • Cycloplegia • Referral to • Difluprednate • Topical Steroid rheumatology • Systemic treatment • Effective for uveitis • Aggressive to start • NSAIDs • 88% improvement in cells • Taper • Methotrexate • TNF inhibitors/Biologics • 50% eyes, 50% patients: • Work Up • Adalimumab (Humira) • IOP increase (≥10 mmHg & ≥24mmHg) • Blood tests • Abatacept (Orencia) • Etanercept (Enbrel) • 39% eyes, 43% patients • ANA • Infliximab (Remicade) • Cataract formation/progression • RF • HLA‐B27 Slabaugh MA et al. Efficacy and Potential complications of difluprednate use For pediatric uveitis. Am J Ophthalmol 2012;153: 232-58

2019 JIA Screening Topical Steroids Recommendations • Treat as you do in adult Type ANA Age at Duration Risk Frequency Onset of Disease Category of Eye • Don’t under treat!! Exam Oligoarthritis, + <7 AND ≤4 High 3 months • Monitor IOP polyarthritis (RF ‐), psoriatic, • Steroid responders undifferentiated

• If increase IOP add glaucoma drop (e.g. Oligoarthritis, ‐ ≥ 7 OR >4 Low or 6‐12 polyarthritis (RF ‐), Moderate months Timoptic 0.25% or 0.5%) psoriatic, • Contraindications undifferentiated • Wean off Systemic JIA, Low or 6‐12 polyarthrisis (RF ‐), Moderate months enthesitis related arthritis

Hyphema

• Blood in the anterior chamber • Etiology • Traumatic • Disease • Retinoblastoma • Sickle Cell*

•

15 Traumatic Hyphema Exam Hyphema Treatment

• VA • IOP • Bed rest! • Cornea • Retina • Or Lazy Boy Chair • Abrasion • Hemorrhages • Head elevated 30deg • Blood staining • Commotio retinae • No ASA or Ibuprofen • AC • B scan? • Fox shield • Cells • Gonio? • Iris • Sphincter Tear • Lens • Cataract

Hyphema Treatment Glaucoma • Beta Blockers • Cycloplegic –Timolol maleate 0.25, 0.5% (Timoptic, Timoptic XE, • Atropine 1% bid Istalol) • Steroids –Betaxolol 0.25% (Beoptic S) • • Pred Forte “Safe for pediatrics patients” • q1h or q2h initially • Alpha Adrenergic Agonists • Monitor IOP –Brimonidine 0.2% (Alphagan/P) • Steroid responders v. Traumatic Glaucoma • Ages 2+ • Extreme sleepiness (50‐83%) ages 2‐6, 25% 7+ • Follow up • Carbonic Anhydrase Inhibitors • Daily until hyphema is resolved then weekly –Brinzolamide 1% (Azopt) • As needed for other problems – No IOP lowering 4 wks to 5 mos • Admit to Hospital –Dorzolamide 2% (Trusopt) • “Safe in pediatric patients”

Glaucoma Glaucoma

• Prostaglandin analogs •Rho Kinase Inhibitors • Ages 16* •Netarsudil 0.02% (Rhopressa) • Brimatoprost 0.01/0.03% (Lumigan)* •No safety information <18 years old • Travaprost 0.004% (Travatan/Z)* •53% conjunctival hyperemia • Tafluprost 0.0015% (Zioptan)* • Not recommended in pediatric patients • Lantanoprost 0.005% (Xalatan) • No pediatric information • *Pigmentation changes

16 Glaucoma Combination Drugs Glaucoma Combination

• Brimonidine 0.2%, Timolol 0.5% • Brinzolamide 1% & brimonidine tartrate 0.2% (Combigan) (Simbrinza) • Ages 2+ • Contraindicated <2 year old • Dorzolamide 2%, Timolol 0.5% (Cosopt/PF) • Brimonidine • Ages 2+ • Netarsudil 0.02% & latanoprost 0.005% (Rocklatan) • No safety information <18 years old

Lawnmower injury Lawnmower injury

• 12 y/o WM mowing lawn and injured OS • Initial treatment • Went to ER • PF q1h, Alphagan tid, Vigamox qid • Dx: Corneal Abrasion, Hyphema, Borderline • 3 days after injury IOP • IOP 30 • MD added Cosopt bid • 5 days after injury • Presented with nausea, photophobia, malaise • IOP spike to 44 • Admit to hospital for IV Diamox • Vomiting • IOP still elevated

Baseball Injury Day 1 Follow up

• 17 year old male foul tip off bat • VA HM • Seen in ED • No motility restrictions • Hyphema 50% • No RAPD (by reversal) • Orbital floor fracture (tripod fracture) • Clot 90% of AC • Treatment • Blood on endothelium • Atropine bid • Sphincter Tear • Pred q2h • Conjunctival chemosis? • No nose blowing • IOP • Plastic surgery consult • 12,13

17 Day 2 Follow Up Pearl

• AC Stable • There can be an open globe without a • IOP 4 Seidel Sign • Suspect scleral show (brown coloration to • IOP nasal conjunctiva) • Conjunctival changes • Exploratory Surgery • Scleral rupture with uveal prolopse 15mm “L” shape starting near inferior limbus extending to the medial rectus insertion • Orbital floor surgery deferred

Iris Coloboma

• Failure of embronic fetal fissue to close • Other areas • Optic nerve • Retina • Choroid • Other signs • Nystagmus • Microphthalmia

Iris Coloboma • Isolated/part of a syndrome • Coloboma • Heart Defects • Atresia of chonae • Retarded growth • Genital hypoplasia • Ear malformations/hearing loss • Treatment Retina Image Bank • Glare • Refractive error

18 Iris Lisch Nodules Diagnostic Criteria NF-1 • Discrete lesions on anterior iris surface • 6+ café au lait macules > 5 mm pre‐/15mm post pubertal • Color • 2+ neurofibromas • Round • Plexiform neurofibroma – S shaped upper eyelid • Bilateral • Freckling in axillary or inguinal regions • Varying size • Distinct osseus lesion • Associated with Neurofibramatosis I (NF1) • First degree relative with NF‐1 • 5% < 3 year old • 2+ iris Lisch nodules • 42% 3‐4 year olds Lubs, NEJM, 1991;324:1264 • Optic Nerve glioma • 55% 5‐6 year olds • About 100% over 21 year olds

Neurofibramatosis - 1 (NF1) - Ocular • Optic Nerve Glioma • Most develop in 1st 6 years of life • 15‐20% of patients • 50‐75% asymptomatic! • Symptoms

Pediatric Cataracts Pediatric Cataract - Etiology

• Bilateral • Unilateral • Opacities of crystalline lens • Idiopathic (60%) • Idiopathic (80%) • Size • Hereditary without • Ocular anomalies (20%) systemic disease (30%) • Uveitis • Shape • Most AD but can be AR, • Traumatic (10%) X‐Linked • Location • Genetic, metabolic, systemic disease (5%) • Incidence • Galactosemia (AR), Down Syndrome, DM • 3/10,000 (<1 year) • Maternal infection (3%) • 4.5/10,000 (>1 year) • Rubella, CMV, Syphilis • Ocular anomalies (2%) • Visual development • Medication related • Steroid, Radiation

19 Pediatric Cataract Evaluation Pediatric Cataract Work Up

• History!!! • Unilateral may not • Bilateral cataracts ‐ require extensive more work up if no • Family history, duration, medications, systemic work up family history trauma, health, premature, • History and physical • Tests: • Urine for reducing • Thorough evaluation substances (after milk • VA or Fixation preference of lens feeding) ‐ galactosemia • TORCHS (Toxo, Rubella, • Urine for amino acids • Refractive error (Lowe’s syndrome – glc, CMV, HSV/HZV, cat, dev delay) Syphilis) Titers • IOP • TORCHS Titers • CBC • Dilation • BUN • Calcium, Phosphorus – • Lens assessment metabolic disorders

Infant Aphakia Treatment Study Congenital Cataract Treatment • 114 Infants (1‐6mos) with unilateral cataract surgery • Refractive Correction • IOL v. CL • Outcomes: • CLs after and IOL when older • Grating Acuity at 12 months • SilSoft • HOTV Acuity at 4.5 years • GPs • Results • Bifocal • VA • • Complications (81% v. 56%) Monitor VA • 11/57 Glaucoma in IOL, 9/57 Glaucoma in CL • Amblyopia treatment especially if unilateral • Conculsion “…children should be left aphakic and • Monitor for glaucoma treated with a contact lens until the family and surgeon decide that IOL implantation is indicated..” • Pearl: Lifetime glc suspect

Ectopia Lentis • Lens subluxation due to disorders that disrupt the microfibrils of the zonules • Usually will remain attached, but can detach • Symptoms: • May have reduced VA • Causes • Marfan’s Syndrome • Homocystinuria • Disorder of methionine catabolism • Systemic: developmental delays, blood clots • Complications: pupil block glaucoma • Test: Urine testing for homocysteine • Treatment: Vitamin B6, diet changes Photo courtesy of Daniele Saltarelli, OD

20 Ectopia Lentis Marfan’s Syndrome • Mutations in gene for fibrillin‐1 (FBN1) • Disorders that disrupt the microfibrils of • Systemic the zonules • Tall stature • Marfan’s • Aortic root dilatation • Mitral valve prolapse • Homocystinuria • Complications: cardiac • Usually zonules remain attached, but can • Criteria: detach • Myopia >‐3.00 • Ectopia lentis (non‐progressive) • Signs: www.papermasters.com • Family history • Lens dislocation (dilated exam) • (+) Genetic testing • Reduced VA • Treatment

Ectopia Lentis Treatment Summary • Appropriate MD referral if no prior diagnosis • Protective glasses for sports • Get the information you need • No contact sports if severe subluxation • Kids can have same problems as adults • Accurate refraction • Sometimes eye findings indicate a • Referral for surgery if poor VA due to systemic disease subluxation • Thank You • VA 20/60 or worse • Monocular diplopia • Lenticular prolapse • Average age 10.2 years

Surgical management of lens subluxation in Marfan syndrome, JAAPOS 2014;18:140-6.