Strabismic Amblyopia Strabismus associated with strong fixation preference results in constant unilateral suppression of cortical activity related

to the nonpreferred eye (deviared eye). Constanr suppression in the visually immature patient results in poor vision of the nonpreferred eye (deviared eye), and loss of binocular vision. This type of amblyopia is called strabismic amblyopia and occurs despire the fatr that both eyes have clearly focused retinal images. Column three of igure !"! shows that strabismic amblyopia results in a reduction in binocular cells that thc ma#ority of cortical cells are monocular and driven by rhe preferred eye (right eye ), and the deviated eye (left eye) has the smallest contical representation. Strabismic amblyopia is common, and occurs in approximately $%& of patients with congenital esotropia. Strabismic amblyopia. however, is very uncommon in patients with intermittent strabismus and fusion patients with intermittent exotropia or in patients with incomitant strabismus and fusion such as 'uane(s syndrome rarely develop strabismic amblyopia. Strabismic amblyopia can be moderate to severe and in some cases even result in eccentric fixation and visual acuity of )%*)%% or worse. +onocular ,attern 'istortion Amblyopia -nilateral or asymmetric retinal image blur can produce amblyopia and loss of binoculariry depending on the severity of the condition. +onocular retinal image blur causes cortical suppression. thus there is a combined mechanism of pattern distortion plus constant cortical suppression. Column four of igure !"! shows that unilateral rerinal image blur reduces monocular cell counts from the blurred eye (atropini/ed eye) and that binocular cortical cell counts are also reduced but to a lesser degree. The relatively mild monocular image blur associated with atropine allows for development of peripheral fusion and some srereopsis (see monofixation). 0ote that in column four of igure !"! a significant number of binocular cortical cells remain, despite the presence of amblyopia. Clinically, mild image blur such as that associated with mild anisometropia causes mild anisometropic amblyopia, and allows for the development of peripheral fusion and stereopsis (i.e., monofixarion syndrome). A monocular image that is severely blurred during infancy, however, can cause severe amblyopia with loss of binocular function and the development of secondary strabismus (esotropia, exotropia. or hypertropia). This dense amblyopia with loss of binocularity commonly occurs in patients with dense monocular congenital cataracts. The ophthalmic literature often refers to amblyopia associated with monocular image blur as pattern deprivation amblyopia. This usage is misleading because unilateral image blur results in pattern distortion, nor deprivation. 1oth pattern distortion and suppression contribute to the amblyopia. A better term is monocular pattern distortion amblyopia. Anisometropic amblyopla is caused by a difference in refractive errors that results in a unilateral asymmetric image blur. it is one of the most common types of amblyopia. +ost patients with anisometropic amblyopia have straight eyes and appear 2normal3, so the only way to identify these patients is through vision screening. Stereo acuity testing has had limited value in screening for anisometropic amblyopia because most patients have relatively good stereopsis (between 4% and 5%%% second arc). +yopic anisometropia usually does not

cause significant amblyopia unless the difference in refractive error is greater than $.%% diopters. 6ypermetropic anisometropia on the other hand is fre7uently associated with severe amblyopia. As little as 85.$% hypermetropic anisometropia can be associated wirh significant amblyopia, while moderate hypermetropic anisometropia (8 9.%%) can cause severe amblyopia with visual acuity of )%*)%%. +yopic anisometropic amblyopia is usually mild and amenable to treatment even in late childhood, whereas hypermetropic amblyopia is often difficult to treat past : or $ years of age. The reason for this difference is probably twofold, irst, myopia is usually ac7uired afrer the critical period, allowing good initial visual development. Second, with myopic anisometropia the more myopic eye is used for near and the less myopic eye is used for the distance. ;n contrast, patients with hypermetropic anisometropia always use the less hypermetropic eye (i.e., re7uires less accornmodative effort) and constantly suppress the rnore hypermetropic eye.

1ilateral ,attern 'istortion Amblyopia ;f visual maturation continues under tle influence of bilateral blured retinal images, conical organi/ation will be adversely affected, resulting in bilateral pattern disortion amblyopia. ,attem distortion in its pure form wiithout suppression occurs when there is bilateral symmetrical image blur and no strabismus. Clinicaliy the effects of pure image blur are seen in cases of bilateral high hypermetropia, bilateral symmetrical astigmatism, or with bilateral ocular opacities such as bilateral congenital cataracrs and bilateral ,eter<s anomaly. 1ilateral pattern distortion causes bilateral poor vision but it does not preclude the development of at least some binocular vision usually with gross stereopsis. Column five of igure !"! shows that cats reared with bilaterally blurred retinal images develop the normal number of binocular cortical cells even though they have bilateral pattern distortion amblyopia. ;n cases where the image blur during the neonatal period is so severe that essenrially no pattern stimulation is provided, extremely poor vision and sensory nystagmus develops. ;n cases of dense bilateral congenital opacities, bilateral amblyopia and nystagmus will occur unless the image is cleared.by ) to 9 months of age. This type of nystagmus is called sensory nystagmus.;t is associated with bilateral severe amblyopia, or other causes of congenital blindness such as macular or optic nerve patlology. Sensory nystagmus does not occur with cortical blindness because extra sriate visual pathways supply the fixation reflex. Ac7uired opacities after = months of age do not develop sensory nystagmus as the motor component of fixation has already been established. The presence of sensory nystagmus indicates severe amblyopia, usually )%*)%% visual acuity or worse, but some improvernent of vision and dampening of the nystagmus can occur even with late therapeutic iniervention. Ametropic amblyopia (bilateral hypermetropic amblyopia) usually ocorrs wirh hypermetropia usually occurs with hypermetropia of 8$.%% or more without significant anisometropiain this case visual acuity is decreased in each eye, the eyes are usually straight, and the patients usually have a gross stereopsis. >hen patients are first given their optical correction, visual acuity does not significantly improve, the lac? of improvement with prescription of spectacle correction often leads the examiner. The treatment of bilateral high hypermetropic amblyopia

is to prescribe full hypermetropic correction. ,atients with bilateral amblyopia do not fully acomodate, therefore it is important to give them their full hypermetropic correction, even in the absence of strabismus. ;n most cases, visual acuity will slowly improve if the glasses are worn full time, with final visual acuities usually in the range of )%*:% to )%*)$. 1ilateral meridional amblyopia is similar to bilateral hypermetropic amblyopia in that it is bilateral condition and is secondaryto pattern distortion. Significant meridional amblyopia occurs with astigmatism of 89.%% or more. ;n order to avoid meridional amblyopia, it is suggested that astigmatisms of 8).$% or more should be treated in preschool children, astigmatisms over 89.%% to 8:.%% should be treated even in infants. A+1@AB,;C C;S;B0