Written Summary: Ophthalmology, 48:06 - PEDIATRIC OPHTHALMOLOGY

Main Written Summaries Listing | Ophthalmology: 2010 Listings

Volume 48, Issue 06

March 21, 2010

The following is an abstracted summary, not a verbatim transcript, of the lectures/discussions on this audio program. If, after reviewing this written summary, you would like to hear the contents and/or earn CME/CE credit, simply visit the Audio-Digest Foundation website

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Pediatric Ophthalmology

From the 20th Annual Jack Crawford Day — Pediatric Ophthalmology: Challenging the Myths
presented by the University of Toronto Faculty of Medicine

Educational Objectives

The goal of this program is to improve the diagnosis and treatment of ocular disorders in children. After hearing and assimilating this program, the clinician will be better able to:

1. Discuss the validity of common myths about thediagnosis, treatment, and progression of retinopathy of prematurity.

2. Choose appropriate techniques and tools for eye examinations in children with developmental disorders.

3. Recognize different types of visual impairment in developmentally delayed children and distinguish anterior from cortical visual loss.

4. Diagnose uveitis in children in the presence or absence of arthritis.

5. Determine the optimal therapeutic approach for children with uveitis.

Faculty Disclosure

In adherence to ACCME Standards for Commercial Support, Audio-Digest requires all faculty and members of the planning committee to disclose relevant financial relationships within the past 12 months that might create any personal conflicts of interest. Any identified conflicts were resolved to ensure that this educational activity promotes quality in health care and not a proprietary business or commercial interest. For this program, the faculty and the planning committee reported nothing to disclose. In their lectures, Drs. Coats and Najm-Tehrani present information that is related to the off-label or investigational use of a therapy, product, or device.

Acknowledgements

Lectures by Drs. Coats, Buncic, and Najm-Tehrani were recorded at 20th Annual Jack Crawford Day — Pediatric Ophthalmology: Challenging the Myths, held April 24, 2009, in Toronto, ON, and sponsored by Continuing Education and Professional Development, Faculty of Medicine, University of Toronto. The Audio-Digest Foundation thanks the speakers and Continuing Education and Professional Development, Faculty of Medicine, University of Toronto, for their cooperation in the production of this program.

Retinopathy of Prematurity: Debunking Common Myths

David K. Coats, MD, Professor of Ophthalmology and Pediatrics, Baylor College of Medicine, and Chief of Ophthalmology, Texas Children’s Hospital, Houston

Myth #1 “plus disease easily diagnosed and well defined by standard photograph”: most clinicians diagnose retinopathy of prematurity (ROP) by comparison to standard photograph from Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) study; however, one photograph unlikely to be representative of all cases; in study of performance among 22 experts who diagnosed plus disease by comparing photographs of posterior pole vessels to reference standard, sensitivities ranged from 30% to 100% and specificities also varied widely; speaker prefers combining standard photograph with working definition (ie, unequivocal dilation and tortuosity of arterioles and venules in ³2 quadrants in posterior pole); in future, computer analysis expected to guide definition of plus disease

Myth #2 “large babies do not develop serious ROP”: criteria for examining babies for ROP include <1500 g birth weight and <28 wk gestational age; among 328 infants treated for ROP in speaker’s practice, birth weights ranged from 375 to 1211 gm; threshold ROP in 26 infants whose birth weights ranged from >1250 to 1874 g has been reported; ROP in large infants occurs in developing nations; seriously ill babies also may develop serious ROP despite relatively large birth weight

Myth #3 “treatment must be administered when eye reaches high-risk prethreshold (type I disease defined by Early Treatment of Retinopathy of Prematurity [ET-ROP] study)”: speaker describes case example in which 3 clock-hours of mild stage III ROP developed slowly in infant, then regressed without treatment; studies provide guidelines for when to consider treatment, but judgment remains important (speaker treats some patients on basis of clinical judgment, despite not meeting criteria); document reasons for decision not to treat eligible patient, follow-up frequently, and make sure parents understand and agree with decision

Myth #4 “threshold disease should no longer occur”: speaker discussed example of infant who did not qualify for treatment on basis of criteria from ET-ROP study, but progressed to plus disease within 1 wk (ie, bypassed high-risk prethreshold stage); threshold disease can occur despite standard care

Myth #5 “children no longer go blind from ROP”: outcomes from ET-ROP study included 9.1% rate of detachment despite early treatment; another study showed decrease, but not elimination of, stage V ROP; speaker cautions that blindness remains possible outcome

Myth #6 “laser treatment benign”: complications, both systemic (eg, apnea, cardiac issues, difficulty weaning off ventilator) and ocular (eg, cataracts, glaucoma, accidental foveal ablation, vitreous hemorrhage, late giant retinal tears), may occur from laser treatment; cataracts result from persistent tunica vasculosa lentis, uptake of laser energy by vessels, and damage to lens; study showed 1% of patients treated with argon laser developed cataracts; although another study reported no cases of cataract in patients treated with diode laser; other complications (eg, angle closure glaucoma, pupillary block) reported; laser should not be applied indiscriminately

Myth #7 “ROP always follows predictable pattern of progression”: theoretically, ROP progresses from stage I to III; however, (especially with Zone 1 ROP) aggressive, posterior disease can occur at ³30 to 31 wk postmenstrual age and progress from severe stage within few days to 1 wk; impossible to predict course of disease in advance; neovascularization can grow anteriorly; if vascular-avascular junction treated too early, vessels pull back after disease regresses and retina requires retreatment; timing of retinal detachment —study showed children who had vitreous organization after treatment developed detachments weeks after treatment; important to perform lengthy follow-up of such patients

Conclusions: ROP not stagnant disease; treatment and disease itself evolve over time; speaker recommends avoidance of emotion-based medicine in favor of evidence-based medicine

The Eye Examination in
Developmentally Delayed Children

J. Raymond Buncic, MD, Professor of Ophthalmology, Hospital for Sick Children, University of Toronto, ON

Techniques: depend on child’s age, degree of alertness, type of central nervous system (CNS) involvement, and whether child on sedating drugs (eg, anticonvulsants); some disorders of CNS have predicable courses which affect visual outcome; generally rely on objective findings (eg, reflexes) more than subjective findings; ask parents how well child sees, and determine level of information desired by parents and by teachers (differentiate cognitive vs visual performance for purposes of education)

Observation: important to observe child’s interaction with others, exploration of environment (eg, use of saccadic horizontal or abnormal eye movements); avoid touching patient; examine patterns of fixation (eg, children with central scotomas look above individual to use inferior visual field); explain that anomalous head positions visually useful for child (should not be corrected); emergence of forced preferential looking useful for quantitating amount of child’s form vision and binocularity

Tools: single-symbol tests easier than linear tests; flickering light and Teller and Cardiff cards useful; use head-turning and eye movement to judge visual field in infant (or older child); speaker prefers bilateral visual fields because occluding tends to cause children to become uncooperative; test monocular visual fields after ocular examination to determine presence of optic atrophy or afferent pupillary defect; continue to observe conjugacy, rapid eye movement system, and extent and amplitude of movements; visual evoked potential (VEP) useful, but requires interpretation; electroretinography (ERG) useful in cases of anterior visual disorders not recognized anatomically (eg, fundus looks normal, but child has nystagmus and poor vision); optokinetic nystagmus (OKN) test useful in evaluating vision (binocularly), conjugacy, rapid eye movement system, and cerebral bilaterality and symmetry; drum test less useful (difficult to control speed); speaker prefers OKN strip because fixation easier to control; use vertical OKN for horizontal nystagmus; testing vestibulo-ocular reflex (VOR) reveals information about extent of eye movement and integrity of brainstem; movement can induce nystagmus; babies develop per-rotatory nystagmus while spinning; if baby has no vision, nystagmus continues when spinning stops

Types of visual impairment: absence of nystagmus generally indicates cortical visual loss and presence of nystagmus indicates anterior visual loss, early insults to brain cause anterograde transsynaptic degeneration or hypoplasia of optic nerve, which represents mixture of cortical visual loss and optic atrophy; when loss predominantly posterior, speaker defines as posterior visual loss; mild optic atrophy probably secondary; cortical visual loss with CNS disease causes CNS-type nystagmus

Pupils: difficult to examine in children because of hyperactive convergence reflex; speaker uses retinoscope or ophthalmoscope to examine quality and reactivity of red reflex; difficult to detect afferent pupillary defect

Discussion after examination: single examination does not provide final answer because visual system matures, and cerebrally-based visual deficits may improve; speaker avoids labeling child as “blind”; if examination unsuccessful, sedation or anesthesia sometimes necessary

Pediatric Uveitis:
Challenges in Diagnosis and Management

Nasrin Najm-Tehrani, MD, MB, BCh, MSc, Assistant Professor of Ophthalmology and Vision Sciences, Hospital for Sick Children, University of Toronto

Background: uveitis defined as inflammation in uvea (between sclera and retina); anterior uveitis often presents as chronic disease in children; usually juvenile idiopathic arthritis (JIA)-related uveitis or chronic idiopathic uveitis; study of 115 children showed »60% had idiopathic form; children who have complications at baseline often have flares as most significant inflammatory sign; patients with severe disease at presentation at higher risk for additional complications early in disease; study of patients with JIA-associated uveitis found high rate of cataracts, glaucoma, band keratopathy, maculopathy, and hypotony (all potential causes of blindness)

Uveitis in children with JIA: educate parents (many do not associate arthritis with eye problems); examine children £1 mo after diagnosis of probable JIA; examine children with fewer involved joints more frequently because they have higher risk for disease, especially if antibodies to nuclear antigens (ANA)-positive

Diagnosis: hand-held slit lamp not optimal for looking at anterior chamber (AC) activity (eg, possible to see keratic precipitates [KPs] but cannot count cells); amount of AC activity graded with slit lamp correlates with number of cells counted in fluid from AC by microscopy; use adult slit lamp; turn both width and length of slit beam to 1 mm; count and record number of cells at each patient visit; also grade amount of flare in eye with flare meter or subjective assessment

Silent disease: in absence of systemic features or signs of disease, child may not be examined until abnormal findings (eg, posterior synechiae) observed by optometrist; such children may do poorly because of later stage of disease and may show adverse features (eg, band keratopathy, posterior synechiae, cataracts, hypotony, macular edema, vision loss); examine eye and fundus and obtain intraocular pressure (IOP) if possible

Treatment: speaker uses topical steroids, eg, prednisolone acetate (eg, Pred Forte), as first-line and switches to rimexolone if concerned about IOP; nonsteroidal agents not as effective; dilation useful for short time when control of active uveitis needed; atropine stings less than cyclopentolate, but cyclopentolate preferable because it allows movement of pupil; dilating drops also provide confirmation of medication compliance; possible to use periocular injections of steroids (eg, sub-Tenon injections) under anesthesia with curved Steven’s cannula; speaker treats with 2 to 3 wk of intensive topical steroid first to evaluate possible steroid-induced rise in IOP (avoid injections if elevated IOP observed); avoid treating children with systemic steroids (other than short course) because of side effects

Immunomodulation: methotrexate (antimetabolite that inhibits DNA replication in actively dividing cells) most important agent; given orally or subcutaneously; dosage depends on surface area and weight of child; collaborate with rheumatologists when using systemic medication; dose also varies according to individual response and tolerance; subcutaneous administration increases bioavailability and produces fewer gastrointestinal side effects (eg, anorexia, nausea, vomiting); check liver function, hematologic status, and immunization status (eg, for chicken pox) before beginning therapy; teenagers must avoid alcohol and unprotected sex; methotrexate generally tolerated well by children and reduces amount of steroid therapy needed; if side effects not tolerated, supplement with folic acid on days methotrexate not given, reduce dose, switch to subcutaneous administration, and/or give antiemetic drugs

Biologic agents: few patients have received these for uveitis alone, but they work well for severe uveitis; prescribe in collaboration with rheumatologist because of potential for malignancy; require application through access program

Goals: prevent loss of vision by treating intraocular inflammation, managing complications, and treating amblyopia; keep in mind possibility of patient noncompliance and toxicity of drugs

Complications

Cataracts: can develop in immature visual system secondary to poorly controlled inflammation, use of steroids, and intraocular surgery; try to prevent or delay onset to prevent severe amblyopia; small cataracts may not require surgery; if surgery needed, control inflammation preoperatively, use oral steroids if necessary, begin therapy with methotrexate, and allow »6 wk for effect; implantation of intraocular lens (IOL) usually not recommended because poor outcomes common when placed in children; manage refraction after surgery with aphakic contact lenses and glasses; manage postoperative inflammation aggressively

Glaucoma: long-term use of steroids may elevate IOP and contribute to development of glaucoma; many children have IOP >30 mm Hg; elevation of IOP usually occurs 2 to 3 wk after initiation and can persist even after discontinuing steroids; if inflammation controlled, treat by reducing dose of steroids; if not, continue with topical steroids until steroid-sparing agents available; recognize respiratory symptoms associated with b-blockers used to treat glaucoma; brimonidine can cause drowsiness; systemic carbonic anhydrase inhibitors (eg, acetazolamide) can cause lethargy and limit growth; always perform careful gonioscopy (while child sleeps, if necessary); consider angle surgery before surgery for filtration drainage

Posterior segment inflammation and cystoid macular edema: treat with steroids

Questions and Answers

Complications associated with IOLs: impossible to entirely rid eye of inflammation; patients with IOLs may develop significant fibrosis with movement of implant and formation of secondary membrane with fibrin over IOL

When to shift from topical treatment to immunosuppression: educate parents about consequences of long-term topical steroid use; methotrexate can reduce amount of steroids needed; work closely with rheumatologist; decision to switch to methotrexate dependent on duration and frequency of therapy with steroids and level of response (ie, if no response after »3 mo of intensive therapy)