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Figure 2.104. A persistent and engorged tunica vasculosa lentis occurs in those infants who are very immature and develop severe active retinopathy of prematurity.

Cicatricial (Spontaneous Regression) Retinopathy of Prematurity

The anatomical macular outcome which correlates with final visual acuity is measured by two parameters: macular ectopia and vessel traction.

Figure 2.105. This is the grading system for macular ectopia. The normal macular position is between 2 and 3 disc diameters from the temporal margin of die optic disc. The numbering system is shown.

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Optic Disc With Vertical Tilt

Figure 2.106. In the grading system for vessel traction, the normal position of retinal vessels for die first two disc diameters is between the vertical meridian and the 30 sectors temporal to die vertical meridian. The numbering system is shown.

Erythema Newborns

Figure 2.107. This retinal montage shows nasal dragging of the macula with macular ectopia of 2 and vessel traction of 1. Vision is 20/400.

Figure 2.108. In this retinal montage note die temporal dragging of the macula with macular ectopia of 4 and vessel traction of 2. Vision is 20/400.

Figure 2.109. An angle closure glaucoma is a common complication of very severe ROP. Conjuctival erythema, corneal clouding, a nondilatable pupil, and a flat anterior chamber are present.

Macular Dragging Rop

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Figure 2.110. In complete retinal detachment there is a fibrovascular mass behind the lens which provided the original name for retinopathy of prematurity: "RLF" or retrolental (behind the lens) fibroplasia (fibrovascular mass).

Erythema Newborns

Differential Diagnosis for Retinopathy of Prematurity

A. Abnormal neuroectodermal migration or differentiation of the retina (from inner to outer layers) can be associated with abnormal migration or differentiation of the central nervous system as in the following examples.

Figure 2.111. Walker-Warburg syndrome is an autosomal recessive disorder with absence of the normal cascade of retinal differentiation reflected in abnormal development of all retinal layers. Remnant retina is "retrolental" (behind the lens) and lissencephaly is present in the central nervous system.

Images Kneist Syndrome

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Retinopathy Premature

Figure 2.112. The Dandy-Walker deformity with a posterior encephalocele is another autosomal recessive disorder which demonstrates the absence of the normal cascade of retinal differentiation in the retinal periphery.

Figure 2.113. Stickler syndrome is an autosomal dominant (12ql3) disorder which may be diagnosed in the newborn period by recognizing the association between the Robin's anomalad of cleft palate, small mandible, and backward displacement of the tongue. A progressive arthropathy can be identified radiographically. The Kniest syndrome may be an autosomal recessive disorder of the same gene. Abnormal thinning of die peripheral retina which precedes giant retinal tears is seen in these retinas.

Figure 2.114. Juvenile retinoschisis is an Xdinked recessive (Xp22) disorder which can present with peripheral and/or foveal involvement. Clinically this is seen as vitreous veils and strands with retinal detachment peripherally and/or a star-shaped or spoke-like configuration in the fovea. Splitting of the nerve fiber layer in the inner retina is present pathologically.

Figure 2.115. Incontinentia pigmenti is an X-linked dominant (Xq27-28) disorder (i.e., limited to females). There is abnormal melanin widiin the retinal pigment epithelium and abnormal vascularization of the peripheral retina. Skin, skeletal and central nervous systems are also affected in this disorder.

Retinal Melanin

B. Abnormal formation of inner retinal vessels can occur due to genetic abnormality and can be associated with abnormal vascularization of other organ systems as in the following examples.

Figure 2.116. Norrie syndrome is an X-linked recessive (Xp11.3) disorder which has been classically described as congenital blindness in all patients with a dysplastic retina histologically. Hearing loss and mental retardation have been associated progressive findings. Currently, this classic definition has been revised to include other phenotypes of the Norrie disease gene.

Norrie Disease

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Figure 2.117. Exudative vitreoretinopathy can be a phenotype of die Norrie disease gene (Xpll.3) in term infants with ocular involvement only. Clinically, there is a progressive peripheral retinal neovascularization resembling cicatricial Grade II to IV RLF with macular ectopia as shown in this montage. An autosomal dominant ( 1 lql3 ) exudative vitreoretinopathy may be clinically indistinguishable.

Asian Premature BabiesCns And Retina

Figure 2.118. Preterm infants can present with a phe-notype of the Norrie disease gene that involves all of the systems vascularized by the dorsal embryonic aortic arches (aorta, thymus, ear, brain, and eye). Clinically, there is an acute peripheral retinal neovascularization resembling active Stage III ROP as shown in this montage.

C. Abnormal formation of inner retinal vessels can occur in response to an intrauterine insult and can be associated with gross CNS changes as in the following examples.

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Normal Infant Retina

Figure 2.119. The retina of an Asian infant who presented with an anterior encephalocele. Anterior encephaloceles and this retinal picture are more common in Asians and Africans because of their thin ethmoidal structures.

Figure 2.120. This is the retina of an infant who suffered an intrauterine infarction of both middle cerebral arteries. Similar intrauterine stresses can be associated with retinal pictures virtually indistinguishable from ROP.

OPTIC NERVE

Optic Nerve Hypoplasia

Figure 2.121. Note the hypoplastic optic nerve on the left and a normal optic nerve in the fellow eye on the right. Optic nerve hypoplasia may occur unilaterally or bilaterally, both sporadically, as part of de Mosier syndrome with absence of corpus callosum, and as part of autosomal recessive optic nerve hypoplasia. It may be necessary to evaluate the endocrine status of the infant (growth hormone and thyroid hormone are most commonly decreased).

Figure 2.122. Aicardi's syndrome presents with characteristic chorioretinal "lacunar" lesions. The syndrome is an X-linked dominant (Xp22) disorder (limited to females). Optic nerve colobomas can occur. The CT scan shows agenesis of the corpus callosum and affected patients have severe mental retardation and infantile massive spasms.

Figure 2.121. Note the hypoplastic optic nerve on the left and a normal optic nerve in the fellow eye on the right. Optic nerve hypoplasia may occur unilaterally or bilaterally, both sporadically, as part of de Mosier syndrome with absence of corpus callosum, and as part of autosomal recessive optic nerve hypoplasia. It may be necessary to evaluate the endocrine status of the infant (growth hormone and thyroid hormone are most commonly decreased).

Optic Nerve Hypoplasia

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Figure 2.123. Optic nerve coloboma (without microphthalmia) may occur as an autosomal dominant or sporadically. It is appropriate to examine other family members, especially any with a history of "glaucoma," because of variable expressivity.

Optic Nerve Hypoplasia

Figure 2.124. Morning glory optic nerve anomaly is characterized by an enlarged and excavated disc. Note the symmetry of the fundus excavation with respect to the disc which suggests an anomalous funnel-shaped enlargement of the distal optic stalk at its junction with the primitive optic vesicle as the primary embry-ological defect. Vision is always poor and diere may be associated defects such as the transsphenoidal form of basal encephalocele.

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