Echographic Findings in Hemorrhagic Disciform Lesions

Marcela Valencia, MD, Ronald L. Green, MD, Pedro F. Lopez, MD

Purpose: To examine the potential role of ocular echography in the evaluation of hemorrhagic disciform disease.

Methods: Using standardized ocular echography, the authors examined 85 eyes of 85 patients with hemorrhagic disciform lesions.

Results: Hemorrhagic disciform lesions ·are located in the subretinal pigment epi­thelial space and appear echographically as solid chorioretinal elevations characterized by a bumpy, lobulated surface with indistinct peripheral margins, irregular internal struc­ture, and medium to high internal reflectivity. These lesions could be reliably differentiated from associated subretinal or suprachoroidal hemorrhage by ocular echography when the maximal height of the lesion was 1 mm or greater.

Conclusions: Hemorrhagic disciform disease is usually complex with distinct com­ponents that may not be discernible on biomicroscopic or angiographic examination, in part because of overlying subretinal hemorrhage. Ocular echography provides a readily available clinical tool to define the components of hemorrhagic disciform disease more precisely. Improved characterization of hemorrhagic disciform lesions may improve un­derstanding of the natural history and response to therapy of hemorrhagic age-related macular degeneration. Ophthalmology 1994;101:1379-1383

Recent advances in vitreoretinal surgery have provided new methods for the evacuation of massive presumed subretinal hemorrhage. 1-

10 Appropriate patient selection and optimal surgical approach, however, may depend, in part, on whether the hemorrhage is located in the sub­retinal, subretinal pigment epithelial, or suprachoroidal space. Unfortunately, such precise localization may be difficult to ascertain, not only during the preoperative clinical or angiographic examination, but even during the intraoperative evacuation of the presumed subretinal hemorrhage. We reviewed the echographic findings of85 patients with hemorrhagic disciform lesions to determine whether ocular echography might be a useful adjunct to

Originally received: October 25 , 1993. Revision accepted: March 3, 1994. From the Doheny Eye Institute, and the Department ofOphthalmology, University of Southern California School of Medicine, Los Angeles. Presented in part as a poster at the American Academy ofOphthalmology Annual Meeting, Chicago, November 1993. Each author states that she/he has no proprietary interest in the devel­opment or marketing of any products described in this article. Reprint requests to Pedro F. Lopez, MD, Doheny Eye Institute, 1450 San Pablo Street, Los Angeles, CA 90033.

define the location of presumed subretinal hemorrhage more exactly.

Materials and Methods

We performed a retrospective review of235 patients (235 eyes) with an echographic diagnosis of disciform lesion who were evaluated by the ocular echography service of the Doheny Eye Institute between 1981 and 1992. One hundred thirteen eyes with disciform lesions without as­sociated vitreous or intralesion hemorrhage by clinical or echographic examination were excluded. Thirty-seven eyes with diabetic retinopathy, glaucoma, retinal venous occlusive disease, macular edema, retinal tears, intraocular tumors, previous trauma, or previous surgery also were excluded. The remaining 85 eyes (85 patients) are the subject of this report. All patients were examined using the technique of Standardized Echography first described by Ossoinig. 11 - 13 Standardized A-scan echography was performed with either a 7200MA Kretztechnic or Bio­physic-Ophthascan-S unit (Alcon, Inc, Fort Worth, TX). Contact B-scan echography was performed with either a CooperVision Ultrascan or Biophysic-Ophthascan-S unit (Alcon, Inc).

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Figure 1. A, B-scan echogram. Transverse view demonstrates a mod­erately elevated, lobulated, hemorrhagic disciform lesion (arrow). B, A-scan echogram demonstrates a high reflective surface spike of the dis­ciform lesion (large arrow) and medium to high reflective spikes from the internal aspects of the lesion (small arrows).

Results

Of the 85 patients whose eyes were included in this study, 36 were men and 49 were women. The average age of these patients was 78 years (range, 49-96 years). There were 46 right eyes and 39 left eyes. The mean duration of follow-up was 135 days (range, 20-990 days).

On echographic examination, the hemorrhagic disci­form lesions appeared as solid chorioretinal elevations characterized by a bumpy, lobulated surface with indis­tinct peripheral margins, irregular internal structure, and medium to high internal reflectivity (Fig l ). The average maximal height of the hemorrhagic disciform lesions (measured from the inner scleral lamella) was 3.5 mm (range, 2.2-7.6 mm). Extent ofthe hemorrhagic disciform lesions varied from small localized lesions in the macular area to large diffuse lesions that expanded from the pos­terior pole to the inferotemporal periphery.

Hemorrhagic disciform lesions involved the macular area in all except one eye that had an eccentric disciform process in the peripheral fundus. The hemorrhagic dis­

ciform lesions were confined to the macular area in 34 eyes ( 40%), but frequently extended from the macular area to the periphery. The most common location ofsuch peripheral extension was the inferotemporal fundus in 39 eyes ( 46%), followed in descending frequency by the su­perotemporal fundus in 7 eyes {8%) and the superonasal fundus in 3 (4%).

An overlying retinal detachment characterized as a smoothly elevated, highly reflective membrane on B-scan with a 100% initial spike on A-scan echography was pres­ent in 41 ( 48%) ofthe 85 eyes with hemorrhagic disciform lesions (Fig 2). In all these eyes, the overlying retinal de­tachment could be distinguished clearly from the under­lying hemorrhagic disciform lesions, indicating that the internal boundary of the hemorrhagic lesions was at the level of the retinal pigment epithelium (Fig 3). The over­lying retinal detachments were hemorrhagic in 28 eyes (33%) and serous in 13 eyes (15%).

Hemorrhagic retinal detachment was differentiated from serous retinal detachment by the presence of sub-

Figure 2. A, B-scan echogram. Transverse view demonstrates a mod­erately elevated, hemorrhagic disciform lesion (small arrows) and a shallow overlying retinal detachment (large arrow). B, A-scan echogram dem­onstrates a highly reflective spike of the overlying retinal detachment (small solid arrow), a highly reflective surface spike from the surface of the hemorrhagic disciform lesion (large solid arrow), and medium to high reflective internal spikes (open arrow) of the lesion.

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Figure 3. Transverse B-scans of a hemorrhagic disciform lesion. A, shal­low retinal detachment (arrow) overlying lobulated, hemorrhagic disciform lesion. B, shallow choroidal detachment (black arrow) underlying pe­riphery oflobulated, hemorrhagic disciform lesion (white arrows). Notice the vitreous hemorrhage (open arrow) overlying the disciform lesion. C, an echodense plaque (black arrow) representing calcification of Bruch's membrane is present at the base of the hemorrhagic disciform lesion (white arrow) and results in moderate shadowing of orbital tissue posterior to the lesion.

retinal opacities, which, in some eyes, had mild after­movement on dynamic echography. The location of the hemorrhagic retinal detachments overlying the disciform lesions was the inferotemporal fundus in 13 eyes ( 46%), the macular region in 10 (36%), the superonasal fundus in 2 {7%), the superotemporal fundus in 2 {7%), and the inferonasal fundus in 1 ( 4%). The serous retinal detach­ments overlying the hemorrhagic disciform lesions were located in the macular region in eight eyes ( 61%), in the inferotemporal fundus in four (31%), and in the supero­temporal fundus in one (8%).

In two eyes, linear calcification at the base of the hem­orrhagic disciform lesions (presumably at the level of the elastic layer of Bruch's membrane) could be identified (Fig 3). This finding suggested that the external boundary of the hemorrhagic disciform lesions was Bruch's mem­brane. In one of these two eyes, a shallow hemorrhagic choroidal detachment (Fig 3) could be seen underlying the peripheral aspect ofthe hemorrhagic disciform lesion, further confirming that the disciform lesion was confined to the space between the retinal pigment epithelium and Bruch's membrane. The findings in this patient also in­dicate that suprachoroidal hemorrhage may, in some eyes, underlie hemorrhagic disciform lesions and be responsible for a component of the chorioretinal elevation in these lesions.

Discussion

The prognosis ofpresumed subretinal hemorrhage in age­related macular degeneration is very poor, in contrast to that of subretinal hemorrhages in other conditions, such as trauma, the presumed ocular histoplasmosis syndrome, and ruptured retinal arterial macroaneurysms. 14

-18 Are­

cent study by Bennett and co-workers18 of the natural history of presumed subretinal hemorrhage shows that three prognostic factors correlated with poor visual out­come: the presence of thick subretinal hemorrhage that caused visible retinal elevation, the presence ofsubretinal hemorrhage greater than 4 disc diameters in greatest di­mension, and an underlying diagnosis ofage-related mac­ular degeneration. Multivariate analysis of these prog­nostic factors disclosed that the presence of age-related macular degeneration had the greatest negative impact on final visual outcome. The mean final visual acuity in patients with presumed subretinal hemorrhage and age­related macular degeneration was 20/1700, compared with a mean final visual acuity of 20/200 in patients with sub­retinal hemorrhage caused by conditions other than age­related macular degeneration. In that study, however, it was not possible by the clinical examination methods used to reliably differentiate patients with subretinal hemor­rhage alone from those with combined subretinal and subretinal pigment epithelial hemorrhage or hemorrhagic disciform lesions. 18

This distinction may be important because previous observations by Gass14 suggest that the visual prognosis is better for hemorrhage in the subretinal space alone than for hemorrhage beneath both the subretinal and subretinal

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pigment epithelial space. Gass14·15 observed that when there is bleeding from choroidal neovascularization in pa­tients with age-related macular degeneration, the hem­orrhage spreads beneath the retinal pigment epithelium and only secondarily breaks through into the subretinal space. In other conditions, however, such as trauma, the presumed ocular histoplasmosis syndrome, and ruptured retinal arterial macroaneurysms, the hemorrhage that is visible beneath the retina is present almost exclusively in the subretinal space. 16·17

Recent attempts to improve the poor outcome of pre­sumed subretinal hemorrhage in patients with age-related macular degeneration have focused on the use of vitreo­retinal surgical techniques to evacuate the hemor­rhage.1-4·6·8·10 These studies have had variable outcomes, with some patients achieving improved vision, whereas others remained unchanged, or actually worsened. In part, the variability in outcome after surgical intervention may reflect differences in the preoperative characteristics of the hemorrhagic lesions. Biomicroscopically, hemorrhagic disciform macular degeneration can range from a small area of subretinal hemorrhage at the margin of otherwise nonhemorrhagic subretinal neovascularization, to a larger annular "moat-like" subretinal hemorrhage surrounding an elevated hemorrhagic disciform lesion, to a massive elevated subretinal hemorrhage, with or without an un­derlying hemorrhagic disciform lesion, that extends from the posterior pole to the inferotemporal periphery. In many of these eyes, identification of the presence, thick­ness, and extent of associated hemorrhagic disciform le­sions beneath even a thin layer of subretinal hemorrhage may be difficult, if not impossible, by biomicroscopic or angiographic means. 18 Even during the surgical evacuation of presumed subretinal hemorrhages, identification of which hemorrhagic components are present above or be­low the retinal pigment epithelium can be difficult. Our findings suggest that ocular echography may be useful in evaluating hemorrhagic lesions that are visible beneath the retina to identify and localize the various components of the lesion.

An additional benefit ofocular echography in the eval­uation of hemorrhagic disciform lesions is the differen­tiation of these lesions from choroidal tumors, which may have a clinically similar appearance, particularly in the presence of a vitreous hemorrhage. 12·13 This technique is useful particularly when the lesion is 1 mm or greater in maximal height. 12·13 The most common choroidal tumors clinically encountered are choroidal melanomas, choroi­dal hemangiomas, and choroidal metastases. The cho­roidal melanoma typically has a dome-shaped or mush­room-shaped configuration, has low-to-medium reflec­tivity, and demonstrates internal vascularity. The choroidal hemangioma usually is dome-shaped, has a high internal reflectivity, and has a regular internal structure. Choroidal metastases have a very irregular surface contour and typically have a medium to high internal reflectivity. Choroidal metastases therefore do share similar echo­graphic characteristics to hemorrhagic disciform lesions, but clinically it is usually not difficult to differentiate these two types oflesions. Generally, metastatic tumors are only

mildly elevated, and it is rare for choroidal metastases to be associated with vitreous hemorrhage, extensive sub­retinal hemorrhage, or subretinallipid exudate.

The principal lesion in hemorrhagic age-related mac­ular degeneration is the hemorrhagic disciform le­sion.14•15·19-23 These lesions are characterized echograph­ically by a solid chorioretinal elevation with a bumpy, lobulated surface, indistinct peripheral margins, irregular internal structure, and medium to high internal reflectiv­ity.12·13 Echographically, we located these lesions between the subretinal space and linear areas of calcification, pre­sumably the calcified elastic lamina of Bruch's mem­brane.14·15·19-23 These echographic findings confirm the results of previous histopathologic studies, 14·15·19-23 which located the hemorrhagic disciform lesions between the split layers of the retinal pigment epithelial basement membrane and the inner collagenous layer of Bruch's membrane. The presence ofsubretinal pigment epithelial hemorrhage (clotted or unclotted) and fibrovascular pro­liferation appeared to explain the irregular internal struc­ture and medium to high internal reflectivity that are characteristic of hemorrhagic disciform lesions on ocular echography. Hemorrhagic retinal pigment epithelial de­tachments and hemorrhagic disciform lesions comprise a clinical and histopathologic continuum. Limited subret­inal pigment epithelial hemorrhage overlying or arising from the edge of a choroidal neovascular membrane may result in the smooth dome-shaped echographic appearance of a hemorrhagic retinal pigment epithelial detachment, whereas more extensive subretinal pigment epithelial hemorrhage may result in the more rectangular or oblong echographic appearance characteristic of hemorrhagic disciform lesions.

In patients with hemorrhagic age-related macular de­generation, intraoperative evacuation of presumed sub­retinal hemorrhage often shows the presence of fluid sub­retinal blood adjacent to or surrounding regions of dense solid clot.1-4·6·8·10 The fluid blood is usually readily evac­uated with or without the administration of exogenous tissue plasminogen activator. 1-4·6·8•10 The dense residual clot, which occasionally contains surgically inseparable fibrovascular tissue (choroidal neovascularization),2·10·23 often requires mechanical lysis or extraction. In these eyes, we suspect that the fluid subretinal blood is truly located in the subretinal space and represents breakthrough hem­orrhage, whereas at least a portion of the solid clot visible beneath the retina is located in the subretinal pigment epithelial space and represents an associated hemorrhagic disciform lesion 19-23 that often may be detectable pre­operatively by ocular echography. In our experience, fluid blood visible beneath the retina is found usually in the subretinal space, whereas clotted blood may be found in either the subretinal or subretinal pigment epithelial space. In part, this may reflect differences in either the concen­tration or activation of endogenous fibrinolytic agents (plasminogen activators) between the subretinal and sub­retinal pigment epithelial space.24- 26 Alternatively, loss of fibrinogen after clotting or passage through the subretinal pigment epithelial space may decrease the tendency of breakthrough subretinal hemorrhage to coagulate. The

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finding that at least a portion, if not all, of the solid clot visible beneath the retina in patients with hemorrhagic age-related macular degeneration appears to represent subretinal pigment epithelial (rather than subretinal) hemorrhage further suggests that delivery of exogenously administered tissue plasminogen activator to the subret­inal pigment epithelial space, as well as to the subretinal space, may facilitate surgical evacuation of the bleeding associated with hemorrhagic disciform lesions.

Our findings indicate that ocular echography can be used to ditTerentiate true subretinal hemorrhage from subretinal pigment epithelial (hemorrhagic disciform le­sions) and suprachoroidal hemorrhage, as well as to es­tablish the thickness and extent ofeach ofthe components of the lesion in hemorrhagic disciform disease where the maximal height of the lesion is I mm or greater. Ocular echography thus provides information that may not be available on biomicroscopic or angiographic examination, particularly in cases with overlying subretinal hemorrhage. The more precise characterization of hemorrhagic disci­form disease that is possible with this clinical examination technique also may be valuable to future natural history studies and therapeutic trials. In addition, ocular echog­raphy may be useful in differentiating hemorrhagic dis­ciform lesions from hemorrhagic choroidal tumors, which would thereby minimize inappropriate surgical interven­tion in patients with hemorrhagic subretinal lesions.

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