Although amblyopia is normally defined as a deficit invisual acuity despite full optical correction and theabsence of observable pathology, the association be-tween ocular pathology in early life and amblyopicvisual loss is well established. For example, the presenceof monocular congenital cataract (von Noorden andMaumenee, 1968; von Noorden, 1981), unilateral ptosis(Anderson and Baumgartner, 1980; Harrad et al., 1988)or a wide variety of unilateral ocular pathologies presentduring early childhood (Kushner, 1981, 1984) canprecipitate often severe (deep) cases of amblyopia. Thesestudies provide strong support for the view that aunilateral decrease in the signal emerging from one eyebecause of patching or pathology during a sensitiveperiod early in life will precipitate a functional ambly-opia (Hardesty, 1959; Awaya et al., 1973; von Noorden,1981). In cases where the pathology cannot be resolvedthe total visual deficit reflects the combination of theprimary (organic) visual loss together with a super-imposed functional amblyopia. Recently, it has beensuggested that the visual deficit in many cases of themuch milder and more common forms of amblyopiamay also represent the sum of functional and organiccomponents (Lempert, 2000, 2003, 2004). That is, theamblyopia typically associated with strabismus and/oranisometropia, and considered to exist in the absence oforganic causes, may, in fact, be superimposed upon theloss which is due to a subtle organic defect. Thishypothesis clearly contradicts the generally held viewthat almost all amblyopias are caused by either strabis-mus or anisometropia in the absence of ocular patho-logy. This controversial hypothesis has prompted us tore-examine the traditional view of the causal agents formost amblyopias, and the implications for remediationof this condition.The vast majority of the 2–2.5% (von Noorden, 1996)ofhumansdiagnosedwithamblyopiaeitherhave,orhavepreviously had, strabismus and/or anisometropia. Theabsenceofclinically-detectedpathology,coupledwiththefact that experimentally induced anisometropia andstrabismusinanimalscausebroadlysimilarvisualdeficits(e.g. Harwerth et al., 1983; Smith et al., 1985), has led tothe ubiquitous terms anisometropic and strabismicamblyopia – with the resultant implication of a directcausalrelationship.Thatis,currentclinicaldogmaassertsthat chronic abnormal sensory input because of strabis-mus or anisometropia precipitates a neural deficit that ismanifest clinically as reduced monocular visual acuity.Improvement in visual acuity after treatment consistingonly of manipulation of sensory input (e.g. spectaclesand/or occlusion therapy) can be viewed as a formalconfirmation of the functional amblyopia diagnosis.Although the association between amblyopia andanisometropia and/or strabismus is clear, there has longbeen uncertainty regarding the causal relationshipsbetween anisometropia and strabismus and amblyopiain humans. The causal relationship is self-evident inlongitudinal animal studies in which either strabismus oranisometropia have been shown to precipitate ambly-opia (e.g. Kiorpes and Boothe, 1980; Harwerth et al.,1983; Smith et al., 1985). Furthermore, studies ofhuman infantile esotropia show that esotropia precedesthe development of amblyopia (Birch and Stager, 1985).Unfortunately, such longitudinal studies are rare in thehuman amblyopia literature. In addition to the absenceof definitive longitudinal data from human amblyopes,there are many experimental results that suggest a quitedifferent causal relationship between amblyopia andstrabismus or anisometropia. For example, althoughanimal investigations show that sustained, unilateralblurring is required to precipitate amblyopia (Smithet al., 1985), longitudinal refractive studies of human
Read full abstract