Macular Diseases

Abstract

The macula (anatomically fovea), situated 3 mm lateral to the optic disc, is the most sensitive part of visual acuity. The foveola is a central 0.35 mm wide zone in the macula. The inner retinal layers in the margins of the pit are displaced laterally. Figure 3.1 displays the anatomical schematic diagram of the macula.

The macula (anatomically fovea), situated 3 mm lateral to the optic disc, is the most sensitive part of visual acuity. The foveola is a central 0.35 mm wide zone in the macula. The inner retinal layers in the margins of the pit are displaced laterally. Figure 3.1 displays the anatomical schematic diagram of the macula.

Fig. 3.1

Anatomical schematic diagram of the macula

The macular diseases mainly involve congenital anomaly (congenital macular coloboma), central serous chorioretinopathy, vitreomacular traction syndrome, vitreous hemorrhage beneath the inner limiting membrane, macular edema, macular hole, macular atrophy and proliferative diseases of the macula, such as retinal angiomatous proliferation (RAP), choroidal neovascularization (CNV), and polypoidal choroidal vasculopathy (PCV).

Stereoscopic photography plays an important role in macular diseases [1,2,3]. In the past, stereoscopic slide film photography of the retina is the standard with which other imaging modalities have been compared when identifying age-related macular degeneration (AMD). The Age-Related Eye Disease Study, a multicenter prospective cohort study of 4757 participants designed to access the clinical course, prognosis, and risk factor for age-related macular degeneration and cataract, uses stereoscopic color fundus photographs in a standardized fashion by certified photographers [4, 5]. In ETDRS, besides the grading diabetic retinopathy severity by stereoscopic retinal photography, the clinically significant macular edema (CSME), which is one of the key factors affecting the visual acuity, is also diagnosed correctly by stereoscopic digital fundus photography. The kappa (ƙ) values among contact lens biomicroscopy (CLBM), slit-lamp biomicroscopy with 90D/78D or by stereoscopic pairs are more than 0.6. So, in most cases, the stereoscopic photography can be used as a diagnosis tool, especially for screening and telemedicine [4,5,6].

Abnormalities in macular diseases are various [3, 7, 8]. Drusen are yellow-white deposits within Bruch’s membrane underlying the RPE and vary greatly in appearance, ranging from small round, flat spots in size and shape to large deposits even confluent with adjacent drusen. Geographic atrophy may show a sharply demarcated, usually circular zone of partial or complete depigmentation of RPE and exposure of underlying large choroidal blood vessels. The three kinds of choroidal neovascularization (type I, II, and III), which are correspondent with occult CNV, classic CNV, and retinal angiomatous proliferation (RAP), are more vividly and comprehensively shown on stereoscopic pairs than monoscopic and even OCT scans. The retina will be dome-shaped with intra-retinal exudates and cyst, accompanying by intra-retinal, sub-retinal and sub-RPE hemorrhages, sub-retinal and sub-RPE neovascularies, etc. The anastomosis between retina and choroid will be shown clearly on stereoscopic FFA pairs [7]. Macular holes are well-defined defects in the middle of the macula in various sizes. Sometimes, there is a thin membrane above the posterior retina called epi-retinal membrane (ERM).

Generally speaking, with the combination of stereoscopic photography and other high-tech tools such as OCT, more and more macular signs and characteristics will be explored.

Fig. 3.2

Central serous chorioretinopathy

I. The central reflex was disappeared

II. The apex of sensory retinal detachment

III. Fold of ILM

Fig. 3.3

Central serous chorioretinopathy

I. Leakage from venules inferior-nasal to the macula

II. The area of sensory retinal detachment

Fig. 3.4

Sensory retinal detachment

I. Apex of detachment

II. Fold of epiretinal tissue (ILM)

III. Intermediate retinal exudates

IV. Vessels located in the depressed area, which was lower than the area I

Fig. 3.5

Central serous chorioretinopathy

I. Retinal detachment in the posterior pole

II. One of the apexes of detachment

III. Bottom of detachment

IV. The other one of apexes of detachment

V. Retinal fold

Fig. 3.6

Pouch-shaped retinal pigment epithelium detachment

I. The area of RPE detachment, which was larger in the color photograph than in the fluorescein angiography

II. Apex of detachment

III. Sub-RPE fluid

IV. Blocked fluorescence by hemorrhage and intermediate hyperfluorescence

Fig. 3.7

Idiopathic macular hole

I. Full thickness macular hole

II. Shallow retinal detachment in the adjacent area

Fig. 3.8

Secondary macular hole

I. Full thickness macular hole

II. Proliferative membrane in the vitreous

III. Retinal detachment

IV. Ghost vessels

V. Segmented sheath in the retinal arteries

VI.   Artery-Vein crossing of the superficial retinal artery and deep retinal vein

VII. ILM fold

Fig. 3.9

Macular hole secondary to optic disc pit

I. Full thickness macular hole

II. Shallow retinal detachment in the adjacent area

III. Fold of posterior hyaloids and ILM, the retinal vessels were obscure

IV. Choroidal coloboma

V. Optic disc pit

Fig. 3.10

Retinal detachment secondary to macular hole

I. Full thickness macular hole, approximately 1/4 PD in diameter

II. Retinal detachment and fold

III. Proliferation under the retina like a streak

IV. Peripapillary atrophy

Fig. 3.11

Retinal detachment secondary to juxta foveal hole

I. Suspected para-macular hole, approximately 1/4 PD in diameter, which was confirmed by OCT

II. Detached macula

III. Vitreous band

IV. Peripapillary atrophy

Fig. 3.12

Dry age-related macular degeneration

I. Diffused hard drusen

II. Confused soft drusen

III. Macula uninvolved

Fig. 3.13

Juxtafoveal choroidal neovascularization

I. Juxtafoveal choroidal neovascularization

II. RPE detachment and exudates

III. The area of sensory retinal detachment

IV. Macular edema

Fig. 3.14

Choroidal neovascularization

I. Subfoveal CNV

II. Superficial retinal exudates

III. Exudates in the inner retina

IV. Deep retinal hemorrhage

V. Suspected area of CNV

Fig. 3.15

Choroidal neovascularization

I. Subfoveal choroidal neovascularization

II. Intra-retinal hemorrhage around the lesion

Fig. 3.16

Juxtafoveal CNV

I. Suspected area of CNV

II. Sensory retinal detachment

III. Sub-RPE hemorrhage

IV. Sub-retinal hemorrhage

Fig. 3.17

Sub-macular choroidal neovascularization

I. Sub-macular choroidal neovascularization

II. Small thread-like hemorrhage

III. Dotted exudates

IV. Localized narrowing of retinal vessels and drusen

Fig. 3.18

Polypoidal choroidal vasculopathy

I. Multiple suspected polypoidal lesions

II. Deep retinal hemorrhage

III. Superficial retinal exudates

IV. Sub-retinal hemorrhage

V. Sub-RPE hemorrhage

Fig. 3.19

Retinal angiomatous proliferation (RAP)

I. Intra-retinal neovascularization

II. Sub-retinal choroidal neovascularization

III. Deep retinal exudates

IV. White-dotted sub-retinal exudates

Fig. 3.20

Retinal angiomatous proliferation (RAP) on FFA

I. Intra-retinal neovascularization

II. Sub-retinal choroidal neovascularization

III. Elevated fovea and scattered exudates

Fig. 3.21

Retinal angiomatous proliferation (RAP) on FFA

I. Intra-retinal neovascularization on early FFA

II. Intra-retinal neovascularization on mid-stage FFA

III. Fluorescent leakage of neovascular of neovascular

Fig. 3.22

Polypoidal choroidal vasculopathy

I. Orange elevation

II. Vitreous hemorrhage

III. Sub-retinal hemorrhage

IV. Chronic sub-RPE hemorrhage

V. Intermediate retinal exudates

Fig. 3.23

RPE tear

I. Folded RPE in triangle shape

II. Exposed sclera

III. Intra-retinal exudates

Fig. 3.24

RPE tear

I. Folded RPE or RPE tear

II. Exposed sclera

III. Normal RPE area

IV. Geographic atrophy of macula

Fig. 3.25

Silicone oil tamponade of PCV

I. Chronic choroidal lesion

II. The retinal artery went over the retinal vein

III. The reflex of silicone oil

IV. Deep retinal hemorrhage

Fig. 3.26

Macular hemorrhage

I. Thickened posterior hyaloid and strong reflex

II. Yellow-white epiretinal hemorrhage

III. Sub-retinal hemorrhage

Fig. 3.27

Sub-macular choroidal neovascularization membrane

I. Apex of elevation and appeared white

II. The second layer of exudates

III. The third layer of exudates and appeared yellow-white

IV. Pigment proliferation and small sub-retinal membrane

Fig. 3.28

Juxtafoveal sub-retinal mixed choroidal neovascularization

I. Sub-retinal grey scar

II. Sub-retinal choroidal neovascularization

III. Sub-retinal pigment proliferation

IV. Sub-retinal hemorrhage

V. Epiretinal hemorrhage

VI. Scattered sub-retinal dotted exudates

Fig. 3.29

Sub-macular fibrous membrane

I. Apex of sub-retinal membrane

II. Suspending retinal vessels

III. Pigment proliferation

IV. Retinal artery sheath

V. The retinal artery went over the membrane

Fig. 3.30

Macular edema

I. Elevation of macula and loss of central reflex

II. The retinal artery went beneath the retinal vein

Fig. 3.31

Macular edema

I. Cystoid macular edema

II. Distorted veins on the optic disc

III. Vitreous opacities

Fig. 3.32

Macular edema after grid laser treatment

I. The macula was flat

II. Laser spot

III. The end of retinal vein was dilated

IV. Neovascularization of the optic disc

Fig. 3.33

Macular radial hard exudates

I. The end of retinal artery was dilated

II. Superficial retinal exudates

III. Deep retinal exudates

IV. Grey-whitish exudates

Fig. 3.34

Juxtafoveal telangiectasis

I. The area of macular edema

II. The deep retinal vessels were dilated

III. Intermediate retinal exudates

Fig. 3.35

Adult Coats’ disease

I. The lesion was elevated like three layers of cake

II. The blood supply of the retinal artery was insufficient than the other retinal branches

III. Suspected abnormal vessels

Fig. 3.36

Adult Coats’ disease of the macula

I. Superficial retinal exudates

II. Large amount of yellow-white exudates and crystal in the deep retina

III. Deep retinal hemorrhage

Fig. 3.37

Coats’ disease

I. Yellow-whitish sub-retinal exudates

II. Exudates of the retinal artery

III. Irregular diameter and exudates of retinal veins

IV. The end of the retinal vessels was dilated

Fig. 3.38

Macular hemorrhage in various layers

I. Pre-retinal hemorrhage looked alike a boat

II. Sub-retinal hemorrhage

III. Sub-RPE hemorrhage

IV. The retinal vessels were distorted and dilated

Fig. 3.39

Boat-like epiretinal hemorrhage

I. The serum

II. The platelets

III. The white blood cells

IV. The deoxygenated hemoglobin

V. The oxygenated red blood cells

Fig. 3.40

Epiretinal hemorrhage

I. The boat-like epiretinal hemorrhage like a dome

II. After dissection of posterior limiting membrane by Nd:YAG laser, the hemorrhage was disseminated and absorbed

III. Thickened posterior hyaloids and folds

Fig. 3.41

Proliferative vitreoretinopathy

I. Proliferative membrane and streaks

II. Pseudo hole

III. Atherosclerosis of superior temporal retinal artery

Fig. 3.42

Epi-retinal membrane

I. Epi-retinal membrane extended from the optic disc to the periphery

II. Distorted retinal veins

III. Irregular diameter of the retinal vein

Fig. 3.43

Retinal hemorrhage of different retinal layers

I. Sub-RPE hemorrhage, the lesion was highly elevated

II. Deep retinal hemorrhage

III. Sub-retinal hemorrhage

IV. The central reflex was lost

Fig. 3.44

Chronic retinal hemorrhage

I. Pre-retinal hemorrhage

II. Suspected location of retinal macroaneurysm

III. Intermediate retinal hemorrhage

IV. Artery-Vein nicking (Salus Sign)

Fig. 3.45

Sub-retinal parasitic infection

I. Suspected scolex of the parasite

II. Intra-retinal reaction of multiple retinal layers

III. Exudative retinal detachment

IV. Dotted yellow-whitish exudates

V. Ghost vessel of retinal vein

Fig. 3.46

Coloboma of macula

I. Exposed sclera

II. Choroidal vessels

III. Boundary of coloboma

IV. Impending retinal vessels

Fig. 3.47

Congenital coloboma of macula

I. The sclera was exposed in the area of coloboma

II. Suspending choroidal vessels

III. Mottled epithelial pigment proliferation

IV. The boundary of coloboma

Fig. 3.48

Stargardt disease

I. Bull-eye shaped lesion, irregular with pigmentation

II. Retinal and choroidal atrophy like a basin

III. Retinal vessels that passed through the lesion went attenuated

Fig. 3.49

Macular atrophy

I. Fovea

II. Irregular diameter of retinal veins

III. RPE atrophy

Fig. 3.50

Macular atrophy after branch retinal artery occlusion

I. Macular atrophy and thinning

II. Thinning of inferior temporal retinal artery

III. Pigment proliferation