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Massive Spontaneous Subretinal Hemorrhage
MASSIVE SPONTANEOUS SUBRETINAL HEMORRHAGE M I C H A E L A. B L O O M E , M.D., Houston,
Massive spontaneous subretinal hem orrhage describes the sudden appearance of a massive retinal or choroidal hemor rhage, or both. The initial location of this hemorrhage has been described as ei ther choroidal (massive, spontaneous cho roidal hemorrhage) 1 - 5 or beneath the pig ment epithelium (an extensive form of hemorrhagic detachment of the pigment epithelium) 6 - 8 ; but, the blood may quick ly extend into the neurosensory retina and vitreous. Regardless of the initial location of the hemorrhage, the importance of rec ognizing this entity is demonstrated by the numerous eyes that have been enu cleated with a diagnosis of malignant melanoma. 9 * 10 This hemorrhage appears as an elevated mass under the retina. It is usually greater than one quadrant in size and may in volve the entire retina. The mass charac teristically has smooth borders and varies from dark brown to bluish black in color. It frequently enlarges rapidly and the subsequent appearance of blood in and under the neurosensory retina (especially at the borders of the mass) is important in differentiating this lesion from a melano ma. Massive vitreous hemorrhage, either initially or later, is common and may make the diagnosis difficult. Because of its large size, the mass may displace the lens-iris diaphragm anteriorly, resulting in intractable angle-closure glaucoma. 5 In time the lesion develops into an elevated area of fibrosis surrounded by atrophy and proliferation of the pigment epitheli um.
AND R I C H A R D S. R U I Z ,
M.D.
Texas
massive, spontaneous subretinal hemor rhage seen during the last 15 years. CASE REPORTS Case 1—A 58-year-old woman first seen in Sep tember 1965 had a history of blurred vision in the left eye of three weeks' duration. At this time, visual acuity was R.E.: 6/6 (20/20), and L.E.: finger count ing. A vitreous hemorrhage was present in the left eye. Although the posterior pole could not be visua lized because of the vitreous blood, a gray, elevated mass could be seen in the superior-nasal quadrant (Fig. 1). Examination of the right eye was normal. A radioactive phosphorus uptake ( 32 P) test comparing the superior-nasal quadrant of the left eye with the other three quadrants was negative. The mass did not transilluminate. The patient was observed for 18 months with no treatment. By October 1966, the vitreous blood had completely cleared and the visual acuity was 6/6 (20/20). A large, elevated fibrotic scar surrounded by pigment proliferation was present in the superiornasal quadrant (Fig. 2). The patient has subsequent ly been observed yearly with no change in the ocular examination. Case 2—A 48-year-old woman first seen in De cember 1966 had a history of decreased vision in the right eye for one week. Visual acuity was R.E.: finger counting; L.E.: 6/4.5 (20/15). On the right, there was a large, dark, highly elevated mass under
We describe herein eight patients with From the Department of Ophthalmology, Univer sity of Texas Medical School, Houston, Texas. Reprint requests to Michael A. Bloome, M.D., Houston Eye Associates, 1100 Hermann Profession al Building, Houston, TX 77030. 630
Fig. 1 (Bloome and Ruiz). Case 1. Left eye, a dark elevated mass superior-nasally and extensive vitre ous hemorrhage were present.
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Fig. 2 (Bloome and Ruiz). Case 1. Left eye, one year later. A large, elevated scar surrounded by atrophy and proliferation of the pigment epithelium had replaced the elevated mass. Visual acuity was 6/6 (20/20). the retina which covered the entire lower half of the fundus. A small mass surrounded by a thin layer of blood under the neuroepithelium was present in the macula (Fig. 3). The vitreous was clear, and the uninvolved left eye was normal.
Fig. 3 (Bloome and Ruiz). Case 2. Right eye, a dark, elevated subretinal mass involved the lower retina. A smaller lesion surrounded by a thin layer of blood under the neuroepithelium (arrow) was pre sent in the macula.
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The patient was hospitalized, and a 3 2 P test using the superior quadrants of the involved eye as con trols was negative. One day later, a marked vitreous hemorrhage obscured further visualization of the fundus. During the subsequent year the hemorrhage organized into a dense membrane, and in September 1967, the right eye was enucleated because of the possibility of an occult neoplasm. Microscopic ex amination showed diffuse chorioretinal scarring consistent with a disciform type of degeneration and no evidence of a neoplasm or subretinal neovascularization. Case 3—A 61-year-old man had a sudden onset of visual loss in the right eye in September 1967. He had previously had an ocular examination in 1963, at which time visual acuity in the right eye was 6/30 (20/100) and disciform degeneration of the macula was noted. The vision in the left eye, at that time, was 6/6 (20/20), but macular drusen were present. He had been taking numerous medications for hypertension, and in 1964 had been given anticoag ulants for a myocardial infarction. Visual acuity was light perception in the right eye and 6/7.5 (20/25) in the left eye. The right eye had a shallow anterior chamber and a pressure of 29 mm Hg, whereas the left eye was normotensive with a deep chamber. A solid, dark, elevated mass involved the entire right fundus. A large amount of blood was also present under the neuroepithelium and in the vitreous (Fig. 4). In the left eye, the pre viously noted macular drusen were present as well as three small subretinal hemorrhages nasal to the disk. The patient was immediately hospitalized, and the anticoagulants were discontinued, with a rapid normalization of his clotting time. Despite this, the
Fig. 4 (Bloome and Ruiz). Case 3. Right eye, a solid, dark elevated mass involved the entire retina. A large amount of blood was also present in the vitreous and under the neuroepithelium.
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mass continued to increase in size during the next week, resulting in further forward displacement of the lens-iris diaphragm and an increase in the intra ocular pressure. A 32 P test was attempted and inter preted as normal. Ten days after admission, the eye was enucleated because of intractable glaucoma and the absence of light perception. Microscopic examination revealed a total hemorrhagic detachment of the retina as well as marked hemorrhage under the pigment epithelium and in the choroid and suprachoroidal space (Fig. 5). No subretinal neovascularization could be seen, al though the massive amount of hemorrhage made the microscopic examination difficult. The patient had an uneventful recovery and main tained good vision in the left eye until April 1970, at which time a retinal tear accompanied by vitreous hemorrhage resulted in a sudden decrease in vision. The patient died of a myocardial infarction in No vember 1972. Case A—A 47-year-old woman first seen in Febru ary 1968 had a history of blurred vision and floaters in the left eye for two days. She had been in excellent health, although there was a family history of diabetes mellitus.
Fig. 5 (Bloome and Ruiz). Case 3. Histologic section of enucleated eye. Marked hemorrhage was present in both the choroid and suprachoroidal space. The retina and pigment epithelium were also detached by the hemorrhage and are not present in this field (hematoxylin-eosin, x 45).
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Visual acuity was hand motion temporally in the left eye and 6/7.5 (20/25) in the right eye. A marked vitreous hemorrhage was present in the left eye; but, with an indirect ophthalmoscope, a solid dark mass could be seen temporally and inferiorly. A moderate amount of blood under the neuroepithelium sur rounded the mass (Fig. 6). Examination of the right eye was normal. The patient was hospitalized, and a 32 P test using the superior-nasal quadrant as a control was nega tive. She was subsequently observed with no treat ment. The vitreous hemorrhage slowly resolved and, in June 1971, the visual acuity of the left eye was hand motions. A diffuse posterior subcapsular cata ract was noted on slit-lamp examination. Two large, elevated scars were present in the inferotemporal and nasal quadrants and were surrounded by atro phy and proliferation of the pigment epithelium (Fig. 7). The right eye has remained normal. Case 5—A 39-year-old woman first seen in Febru ary 1970 had a history of decreased vision in the left eye for several weeks. Visual acuity in this eye was 6/12 (20/40). A small subretinal hematoma was pres ent superior to the macula, and two small atrophic areas were situated between the macula and disk. Visual acuity in the uninvolved eye was 6/6 (20/20), and the ocular examination was normal. The patient was treated with systemic corticosteroids for one month, during which time her visual acuity im proved to 6/6 (20/20) and was accompanied by a slight decrease in the size of the lesion. In April 1970, the visual acuity suddenly decreased to 6/60 (20/200) with an enlargement of the lesion and extension into the macula (Fig. 8). During the next
Fig. 6 (Bloome and Hui/.). Case 4. Lett eye, a dark, elevated mass occupied the entire temporal and inferior retina. Marked vitreous hemorrhage was also present.
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month, a massive vitreous hemorrhage reduced the visual acuity to light perception and totally ob scured fundus visualization. The patient was lost to follow-up for five years,
during which time a similar episode occurred in the right eye. In April 1975, she was again examined. The visual acuity was light perception in the right eye and hand motions in the left eye. A mature cataract was present in the right eye, and the fundus could not be seen. Although a diffuse posterior subcapsular cataract was present in the left eye, the fundus could be seen with an indirect ophthalmo scope. A large, fibrotic scar was present in the temporal retina, and a small scar was seen in the superior-nasal quadrant. Atrophy and pigment pro liferation surrounded both lesions (Fig. 9). Case 6—A 67-year-old man first seen in May 1971, had a history of decreased vision in the right eye for three days. He had also noticed a gradual decrease in vision in the left eye for several years. He had been taking antihypertensive medications and had been given anticoagulants three months previously for a myocardial infarction. Visual acuity was hand motions in the right eye and 6/18 (20/60) in the left eye. On the right, a solid, dark, elevated mass involved the entire fundus ex cept for a small central area. Although no vitreous hemorrhage was present, blood under the neuroepithelium surrounded the mass (Fig. 10). In the left eye, an old disciform scar was present in the tempor al paramacular area. The patient was subsequently observed by anoth er ophthalmologist until May 1975, when we reexamined him. Visual acuity was light perception in the right eye and 6/18 (20/60) in the left eye. A mature cataract in the right eye prevented fundus visualization. The left eye was unchanged. Despite a
Fig. 8 (Bloome and Ruiz). Case 5. Left eye, the subretinal hematoma had extended into the macula reducing the vision to 6/60 (20/200). Two small atrophic scars were present between the disk and macula.
Fig. 9 (Bloome and Ruiz). Case 5. Left eye, five years later. A large, elevated scar surrounded by pigment proliferation was present in the temporal retina. Numerous areas of atrophy and pigment proliferation were scattered throughout the retina.
I |
Fig. 7 (Bloome and Ruiz). Case 4. Left eye, three years later. Two elevated scars surrounded by atro phy and proliferation of the pigment epithelium had replaced the subretinal hemorrhage.
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Fig. 10 (Bloome and Ruiz). Case 6. Right eye, a dark, elevated subretinal hematoma involved the entire retina. guarded prognosis, the patient underwent an uneventful cataract extraction in June 1975. The best corrected visual acuity in the right eye follow ing surgery was hand motions. Multiple areas of elevated fibrous tissue surrounded by pigment pro liferation and atrophy were present in the retina (Fig. 11). Case 7—A 43-year-old woman first seen in Sep-
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tember 1972 had a history of decreased vision in the left eye for four days. Visual acuity was 6/6 (20/20) in the right eye and finger counting in the left eye. A large, dark elevated mass was present in the posteri or pole of the left eye. Surrounding this lesion was an area of preretinal hemorrhage as well as a small hemorrhagic detachment of the pigment epithelium temporal to the lesion. The right eye was normal. Within a week after the initial examination, a massive vitreous hemorrhage obscured further visu alization of the retina. The patient was observed for the next six months, during which time the vitreous blood resolved. In March 1973, visual acuity in this eye was 6/60 (20/200) and an elevated scar was seen superior to the macula. The patient was lost to follow-up. Case 8—A 77-year-old man first seen in December 1974 had a history of decreased vision in the right eye for three weeks. He had been taking quinidine for atrial fibrillation as well as various antihypertensive medications. Visual acuity was finger counting in the right eye and 6/60 (20/20) in the left eye. On the right, a large, elevated dark mass was present in the macular area. The lesion was approximately five disk diameters in size and was surrounded by a ring of blood under the neuroepithelium. The left eye was normal except for a few macular drusen. A diagnosis of hemorrhagic detachment of the pig ment epithelium secondary to disciform degenera tion was made and the patient was observed. In February 1975, the visual acuity had improved to 6/60 (20/200), and a typical disciform scar was seen in the macula. Fluorescein angiography revealed a small area of subretinal neovascularization temporal to the scar. In July 1975, the vision in the involved right eye suddenly decreased to hand motions. An other larger, elevated mass now occupied the superi or and inferior temporal quadrants, and its nasal border was adjacent to the previous scar. A moderate amount of vitreous blood was present. During the next six months, the vitreous blood cleared and the mass was replaced by a large, fibrotic scar surround ed by atrophy and proliferation of the pigment epithelium. The final visual acuity remained hand motion. DISCUSSION
Fig. 11 (Bloome and Ruiz). Case 6. Right eye, five years later. The eye had recently undergone an uneventful cataract extraction. An elevated, crescentic scar surrounded by atrophy and pigment prolifer ation had replaced the subretinal hemorrhage.
Although the number of patients in this series is relatively small, it is, to our knowledge, the largest series of massive spontaneous subretinal hemorrhage ever reported (Table). The eight patients ranged in age from 39 to 77 years with only two patients under the age of 45 years. Four of our patients had a previous history of hyper tension and two were taking anticoagu lants at the time of their hemorrhage. Two patients had disciform macular degenera-
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TABLE SUMMARY OF CASE REPORTS*
Patient Age, Sex Medical No. (yrs) History
Initial Visual Acuity
Mass Size Vitreous (Quadrants) Hemorrhage
32p
Test
1
58,F
—
C.F.
1
+
—
2 3 4 5
48,F 61,M 47,F 39,F
H H,A
2 4 3 1
+ + + +
— — —
6 7
67,M 43,F
H,A
C.F. L.P. H.M. 6/12 (20/40) H.M. C.F.
4 1
-
+
N.D. N.D.
8
77,M
H
H.M.
2
+
N.D.
— — —
N.D.
Final Visual Acuity
Remarks
6/6 (20/20) E E S.D.D., Involved eye H.M. H.M. H.M. S.D.D., Other eye 6/60 (20/200) H.M. S.D.D., Involved eye
*H signifies hypertension; A, anticoagulants; N.D., not done; E, enucleated: S.D.D., senile disciform degeneration; C.F., ringer counting; H.M., hand motions; L.P., light perception.
tion in the involved eye before the hemor rhage and one other patient had a similar diagnosis in the uninvolved eye. The ini tial visual acuity varied from 6/12 (20/40) in one case to finger counting or less in seven cases and was dependent on the site and extent of the hemorrhage as well as the presence of vitreous blood. The final visual acuity was equally poor with one exception (6/6 [20/20]) and depended on the location of the scar tissue. The subretinal hemorrhages were large (two quad rants or more in six cases) and involved the entire fundus in two cases. Vitreous hemorrhage was present in seven cases and took from four months to three years to clear. In four cases, a 3 2 P uptake test was done, and all were negative. One patient developed angle-closure glauco ma caused by rapid enlargement of the mass with anterior displacement of the lens-iris diaphragm. The blood in massive, spontaneous subretinal hemorrhage may originate ei ther in the choroid or beneath the pig ment epithelium. Older reports frequent ly described the hemorrhage as choroidal but the histologic descriptions were often vague and incomplete. 1 - 3 Similar cases
have also been described as originating under the pigment epithelium but most of these cases were not documented histologically. 7,8 Regardless of the initial lo cation of the hemorrhage, the clinical picture is frequently similar. Massive hemorrhage in the choroid or under the pigment epithelium, or both, results in a large, dark mass, and further extravasa tion of the blood anteriorly may result in blood under the retinal neuroepithelium (especially at the borders of the mass) and in the vitreous. The clinical picture of massive, spon taneous subretinal hemorrhage can be caused by several entities. In cases re sulting from hemorrhage under the pig ment epithelium, the hemorrhage usually originates from a subretinal neovascular membrane which begins in the choriocapillaris and extends under the pig ment epithelium after passing through or around Bruch's membrane. 1 1 This sub retinal neovascularization has been described in numerous disorders, includ ing macular and peripheral disciform de generation, ocular histoplasmosis, macu lar drusen, and angioid streaks, and the like. 1 1 - 1 4 Most hemorrhages under the
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pigment epithelium are typically much smaller and less extensive than those in our cases, but massive hemorrhages can occur. Cases 5 and 8 initially had typical hematomas under the pigment epithelium before developing massive hemorrhages. These cases, as well as a few described by Silva and Brockhurst, 7 probably repre sent an extensive form of hemorrhagic detachment of the pigment epithelium. Some cases of massive, spontaneous subretinal hemorrhage (especially those with large masses involving the entire retina) originate in the choroid. Sien 4 de scribed a case of spontaneous choroidal hemorrhage in which a ruptured posterior ciliary artery was demonstrated histologically. A similar mechanism has been demonstrated in expulsive choroidal hem orrhage during or following cataract ex traction, 1 5 - 1 6 and also in a rare entity known as spontaneous expulsive cho roidal hemorrhage, in which an expulsive choroidal hemorrhage follows corneal perforation in eyes with long-standing glaucoma. 17 ' 18 Although these last two disorders differ from our cases in which no glaucoma or ocular hypotony was pre sent, it is possible that a choroidal vessel weakened by arteriosclerosis may bleed spontaneously or during anticoagulant therapy. Choroidal hemorrhage may also originate from a subretinal neovascular membrane in the choriocapillaris before it penetrates Bruch's membrane. Two of our patients with a previous history of disciform degeneration of the macula were taking anticoagulants before their hemorrhage. Wolter and McWilliams 19 and Feeman and associates 20 have reported cases of massive subretinal hem orrhage in patients with disciform macular degeneration who were given antico agulants. Pouliquen and Rousselie 21 also reported a similar case in a patient with no previous ocular disorders. The mas sive hemorrhages seen in our two patients may have resulted from the use of antico
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agulants. All ophthalmologists should be aware of this complication in patients with disciform degeneration of the macula. Regardless of the anatomic origin or cause of the hemorrhage, the clinical pic ture must be recognized for proper man agement. A thorough history for the pres ence of hypertension, arteriosclerosis, and drugs such as anticoagulants is man datory. A history of previous visual loss from macular disease or the finding of macular pathology in the other eye may also suggest the correct diagnosis. Frequent fundus examinations with in direct ophthalmoscopy and scleral de pression are the best means of confirming the diagnosis. The presence of a rapidly expanding mass is much more suggestive of subretinal hemorrhage than a tumor. The initial or subsequent appearance of blood under the retinal neuroepithelium and in the vitreous also points to a sub retinal hemorrhage. The eventual ap pearance of an elevated area of fibrosis surrounded by atrophy and pigment pro liferation establishes the diagnosis. Unfortunately, the frequent presence of massive vitreous hemorrhage may ob scure the fundus initially or prevent ade quate follow-up examinations. If a patient has a vitreous hemorrhage (or if it occurs at a later date), bedrest with bilateral patches and head positioning may be helpful to facilitate viewing the lesion. Ultrasonography is essential when the retina cannot be visualized and is also helpful in following the mass when a late hemorrhage occurs. 22 A 32 P test is indicat ed if the lesion is suggestive of a melano ma and can be adequately localized with diathermy on bare sclera. A negative 32 P test makes the diagnosis of melanoma unlikely and warrants following the le sion closely. Ultrasonography is also use ful in confirming the lesion's location before the 32 P test. Once the diagnosis is confirmed, treat ment should be conservative in hopes of
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obtaining useful vision. If angle-closure glaucoma is present, drainage of the cho roidal hemorrhage through a posterior sclerotomy and puncture of the choroid, as well as reformation of the anterior chamber, may be necessary. 5 A vitrectomy late in the course of the disease to remove old, organized blood may result in some visual improvement. A vitrectomy early in the disease may allow the fundus to be visualized but is a dangerous undertaking if an elevated mass is pre sent. SUMMARY
We studied eight cases of massive, spontaneous subretinal hemorrhage which was characterized by the sudden appearance of one or more large, dark, elevated hematomas under the retina. The initial location of the hemorrhage may have been in the choroid or under the pigment epithelium, or both, but further extravasation of the blood anteriorly fre quently resulted in hemorrhage under the neurosensory retina and in the vitreous. Although the lesion can initially be mistaken for a melanoma, the diagnosis is confirmed by the formation of a typical, elevated scar in the involved area. Fre quent fundus examinations are essential, and ultrasound, radioactive phosphorus uptake testing, and bilateral patching are helpful in establishing the diagnosis. The hemorrhage is usually the result of a ruptured vessel from a subretinal neovascular network or arteriosclerotic degener ation and may be precipitated by the use of anticoagulants. REFERENCES 1. Wurdemann, H. V., and Verhoeff, H.: Sponta neous separation of the choroid simulating choroi dal sarcoma. Am. J. Ophthalmol. 10:479, 1927. 2. Seale, E. A.: Rapid interstitial degeneration of the cornea following choroidal hemorrhage. Br. J. Ophthalmol. 15:514, 1931.
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3. Crigler, L. W.: Subchoroidal hemorrhage diag nosed as sarcoma of the choroid. Arch. Ophthalmol. 8:690, 1932. 4. Sien, A. Y.: A case of choroidal apoplexy diag nosed as a sarcoma of the choroid. Ophthalmologica 11:1, 1948. 5. Chan, P. H., and Havner, W, H.: Spontaneous massive choroidal hemorrhage. Am. J. Ophthalmol. 56:568, 1963. 6. Reese, A. B., and Jones, I. S.: Hematomas under the pigment epithelium. Am. J. Ophthalmol. 53:897, 1962. 7. Silva, V. B., and Brockhurst, R. J.: Hemorrhagic detachment of the peripheral pigment epithelium. Arch. Ophthalmol. 94:1295, 1976. 8. Uyama, M., Miyamoto, K., and Ogawa, I.: Subretinal hemorrhage. Folia Ophthalmol. Jap. 20: 692, 1969. 9. Ferry, A. P.: Lesions mistaken for malignant melanoma of the posterior uvea. Arch. Ophthalmol. 72:463, 1964. 10. Berkow, J. W., and Font, R. L.: Disciform macular degeneration with subpigment epithelial hematoma. Arch. Ophthalmol. 82:51, 1969. 11. Gass, J. D. M.: Choroidal neovascular mem branes. Their visualization and treatment. Trans. Am. Acad. Ophthalmol. Otolaryngol. 77:310, 1973. 12. Galinos, S. O., Asdourian, G. K., Wolf, M. B., Goldberg, M. F., and Busse, B. J.: Choroido-vitreal neovascularization after argon laser photocoagulation. Arch. Ophthalmol. 93:524, 1975. 13. Frank, R. N.: Argon laser photocoagulation and subretinal neovascularization. Ophthalmic Surg. 52:56, 1974. 14. Sarks, S. H.: New vessel formation beneath the retinal pigment epithelium in senile eves. Br. J. Ophthalmol. 57:951, 1973. 15. Manschot, W. A.: Glaucoma-vascular necrosis-expulsive hemorrhage. Acta Ophthalmol. 23:309, 1945. 16. The pathology of expulsive hemor rhage. Am. J. Ophthalmol. 40:15, 1955. 17. Williams, D. K., and Rentiers, P. K.: Sponta neous expulsive choroidal hemorrhage. Arch. Oph thalmol. 83:191, 1970. 18. Winslow, R. L., Stevenson, W., and Yanoff, M.: Spontaneous expulsive choroidal hemorrhage. Arch. Ophthalmol. 92:33, 1974. 19. Wolter, J. R., and McWilliams, J. R.: Rupture of disciform macular degeneration. Am. J. Ophthal mol. 59:1044, 1965. 20. Feeman, S. S., Bartlett, R. E., Roth, A. M., and Foos, R. Y.: Intraocular hemorrhage and blind ness associated with systemic anticoagulation. J. A. M. A. 220:1354, 1972. 21. Pouliquen, Y., and Rousselie, F.: Spontane ous choroidal hematoma during anticoagulant treat ment. Bull. Soc. Ophthalmol. 66:5, 1966. 22. Hodes, B. L., and Choromokos, E.: Standard ized a-scan echographic diagnosis of choroidal ma lignant melanomas. Arch. Ophthalmol. 95:593, 1977.
Massive spontaneous subretinal hem orrhage describes the sudden appearance of a massive retinal or choroidal hemor rhage, or both. The initial location of this hemorrhage has been described as ei ther choroidal (massive, spontaneous cho roidal hemorrhage) 1 - 5 or beneath the pig ment epithelium (an extensive form of hemorrhagic detachment of the pigment epithelium) 6 - 8 ; but, the blood may quick ly extend into the neurosensory retina and vitreous. Regardless of the initial location of the hemorrhage, the importance of rec ognizing this entity is demonstrated by the numerous eyes that have been enu cleated with a diagnosis of malignant melanoma. 9 * 10 This hemorrhage appears as an elevated mass under the retina. It is usually greater than one quadrant in size and may in volve the entire retina. The mass charac teristically has smooth borders and varies from dark brown to bluish black in color. It frequently enlarges rapidly and the subsequent appearance of blood in and under the neurosensory retina (especially at the borders of the mass) is important in differentiating this lesion from a melano ma. Massive vitreous hemorrhage, either initially or later, is common and may make the diagnosis difficult. Because of its large size, the mass may displace the lens-iris diaphragm anteriorly, resulting in intractable angle-closure glaucoma. 5 In time the lesion develops into an elevated area of fibrosis surrounded by atrophy and proliferation of the pigment epitheli um.
AND R I C H A R D S. R U I Z ,
M.D.
Texas
massive, spontaneous subretinal hemor rhage seen during the last 15 years. CASE REPORTS Case 1—A 58-year-old woman first seen in Sep tember 1965 had a history of blurred vision in the left eye of three weeks' duration. At this time, visual acuity was R.E.: 6/6 (20/20), and L.E.: finger count ing. A vitreous hemorrhage was present in the left eye. Although the posterior pole could not be visua lized because of the vitreous blood, a gray, elevated mass could be seen in the superior-nasal quadrant (Fig. 1). Examination of the right eye was normal. A radioactive phosphorus uptake ( 32 P) test comparing the superior-nasal quadrant of the left eye with the other three quadrants was negative. The mass did not transilluminate. The patient was observed for 18 months with no treatment. By October 1966, the vitreous blood had completely cleared and the visual acuity was 6/6 (20/20). A large, elevated fibrotic scar surrounded by pigment proliferation was present in the superiornasal quadrant (Fig. 2). The patient has subsequent ly been observed yearly with no change in the ocular examination. Case 2—A 48-year-old woman first seen in De cember 1966 had a history of decreased vision in the right eye for one week. Visual acuity was R.E.: finger counting; L.E.: 6/4.5 (20/15). On the right, there was a large, dark, highly elevated mass under
We describe herein eight patients with From the Department of Ophthalmology, Univer sity of Texas Medical School, Houston, Texas. Reprint requests to Michael A. Bloome, M.D., Houston Eye Associates, 1100 Hermann Profession al Building, Houston, TX 77030. 630
Fig. 1 (Bloome and Ruiz). Case 1. Left eye, a dark elevated mass superior-nasally and extensive vitre ous hemorrhage were present.
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Fig. 2 (Bloome and Ruiz). Case 1. Left eye, one year later. A large, elevated scar surrounded by atrophy and proliferation of the pigment epithelium had replaced the elevated mass. Visual acuity was 6/6 (20/20). the retina which covered the entire lower half of the fundus. A small mass surrounded by a thin layer of blood under the neuroepithelium was present in the macula (Fig. 3). The vitreous was clear, and the uninvolved left eye was normal.
Fig. 3 (Bloome and Ruiz). Case 2. Right eye, a dark, elevated subretinal mass involved the lower retina. A smaller lesion surrounded by a thin layer of blood under the neuroepithelium (arrow) was pre sent in the macula.
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The patient was hospitalized, and a 3 2 P test using the superior quadrants of the involved eye as con trols was negative. One day later, a marked vitreous hemorrhage obscured further visualization of the fundus. During the subsequent year the hemorrhage organized into a dense membrane, and in September 1967, the right eye was enucleated because of the possibility of an occult neoplasm. Microscopic ex amination showed diffuse chorioretinal scarring consistent with a disciform type of degeneration and no evidence of a neoplasm or subretinal neovascularization. Case 3—A 61-year-old man had a sudden onset of visual loss in the right eye in September 1967. He had previously had an ocular examination in 1963, at which time visual acuity in the right eye was 6/30 (20/100) and disciform degeneration of the macula was noted. The vision in the left eye, at that time, was 6/6 (20/20), but macular drusen were present. He had been taking numerous medications for hypertension, and in 1964 had been given anticoag ulants for a myocardial infarction. Visual acuity was light perception in the right eye and 6/7.5 (20/25) in the left eye. The right eye had a shallow anterior chamber and a pressure of 29 mm Hg, whereas the left eye was normotensive with a deep chamber. A solid, dark, elevated mass involved the entire right fundus. A large amount of blood was also present under the neuroepithelium and in the vitreous (Fig. 4). In the left eye, the pre viously noted macular drusen were present as well as three small subretinal hemorrhages nasal to the disk. The patient was immediately hospitalized, and the anticoagulants were discontinued, with a rapid normalization of his clotting time. Despite this, the
Fig. 4 (Bloome and Ruiz). Case 3. Right eye, a solid, dark elevated mass involved the entire retina. A large amount of blood was also present in the vitreous and under the neuroepithelium.
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mass continued to increase in size during the next week, resulting in further forward displacement of the lens-iris diaphragm and an increase in the intra ocular pressure. A 32 P test was attempted and inter preted as normal. Ten days after admission, the eye was enucleated because of intractable glaucoma and the absence of light perception. Microscopic examination revealed a total hemorrhagic detachment of the retina as well as marked hemorrhage under the pigment epithelium and in the choroid and suprachoroidal space (Fig. 5). No subretinal neovascularization could be seen, al though the massive amount of hemorrhage made the microscopic examination difficult. The patient had an uneventful recovery and main tained good vision in the left eye until April 1970, at which time a retinal tear accompanied by vitreous hemorrhage resulted in a sudden decrease in vision. The patient died of a myocardial infarction in No vember 1972. Case A—A 47-year-old woman first seen in Febru ary 1968 had a history of blurred vision and floaters in the left eye for two days. She had been in excellent health, although there was a family history of diabetes mellitus.
Fig. 5 (Bloome and Ruiz). Case 3. Histologic section of enucleated eye. Marked hemorrhage was present in both the choroid and suprachoroidal space. The retina and pigment epithelium were also detached by the hemorrhage and are not present in this field (hematoxylin-eosin, x 45).
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Visual acuity was hand motion temporally in the left eye and 6/7.5 (20/25) in the right eye. A marked vitreous hemorrhage was present in the left eye; but, with an indirect ophthalmoscope, a solid dark mass could be seen temporally and inferiorly. A moderate amount of blood under the neuroepithelium sur rounded the mass (Fig. 6). Examination of the right eye was normal. The patient was hospitalized, and a 32 P test using the superior-nasal quadrant as a control was nega tive. She was subsequently observed with no treat ment. The vitreous hemorrhage slowly resolved and, in June 1971, the visual acuity of the left eye was hand motions. A diffuse posterior subcapsular cata ract was noted on slit-lamp examination. Two large, elevated scars were present in the inferotemporal and nasal quadrants and were surrounded by atro phy and proliferation of the pigment epithelium (Fig. 7). The right eye has remained normal. Case 5—A 39-year-old woman first seen in Febru ary 1970 had a history of decreased vision in the left eye for several weeks. Visual acuity in this eye was 6/12 (20/40). A small subretinal hematoma was pres ent superior to the macula, and two small atrophic areas were situated between the macula and disk. Visual acuity in the uninvolved eye was 6/6 (20/20), and the ocular examination was normal. The patient was treated with systemic corticosteroids for one month, during which time her visual acuity im proved to 6/6 (20/20) and was accompanied by a slight decrease in the size of the lesion. In April 1970, the visual acuity suddenly decreased to 6/60 (20/200) with an enlargement of the lesion and extension into the macula (Fig. 8). During the next
Fig. 6 (Bloome and Hui/.). Case 4. Lett eye, a dark, elevated mass occupied the entire temporal and inferior retina. Marked vitreous hemorrhage was also present.
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month, a massive vitreous hemorrhage reduced the visual acuity to light perception and totally ob scured fundus visualization. The patient was lost to follow-up for five years,
during which time a similar episode occurred in the right eye. In April 1975, she was again examined. The visual acuity was light perception in the right eye and hand motions in the left eye. A mature cataract was present in the right eye, and the fundus could not be seen. Although a diffuse posterior subcapsular cataract was present in the left eye, the fundus could be seen with an indirect ophthalmo scope. A large, fibrotic scar was present in the temporal retina, and a small scar was seen in the superior-nasal quadrant. Atrophy and pigment pro liferation surrounded both lesions (Fig. 9). Case 6—A 67-year-old man first seen in May 1971, had a history of decreased vision in the right eye for three days. He had also noticed a gradual decrease in vision in the left eye for several years. He had been taking antihypertensive medications and had been given anticoagulants three months previously for a myocardial infarction. Visual acuity was hand motions in the right eye and 6/18 (20/60) in the left eye. On the right, a solid, dark, elevated mass involved the entire fundus ex cept for a small central area. Although no vitreous hemorrhage was present, blood under the neuroepithelium surrounded the mass (Fig. 10). In the left eye, an old disciform scar was present in the tempor al paramacular area. The patient was subsequently observed by anoth er ophthalmologist until May 1975, when we reexamined him. Visual acuity was light perception in the right eye and 6/18 (20/60) in the left eye. A mature cataract in the right eye prevented fundus visualization. The left eye was unchanged. Despite a
Fig. 8 (Bloome and Ruiz). Case 5. Left eye, the subretinal hematoma had extended into the macula reducing the vision to 6/60 (20/200). Two small atrophic scars were present between the disk and macula.
Fig. 9 (Bloome and Ruiz). Case 5. Left eye, five years later. A large, elevated scar surrounded by pigment proliferation was present in the temporal retina. Numerous areas of atrophy and pigment proliferation were scattered throughout the retina.
I |
Fig. 7 (Bloome and Ruiz). Case 4. Left eye, three years later. Two elevated scars surrounded by atro phy and proliferation of the pigment epithelium had replaced the subretinal hemorrhage.
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Fig. 10 (Bloome and Ruiz). Case 6. Right eye, a dark, elevated subretinal hematoma involved the entire retina. guarded prognosis, the patient underwent an uneventful cataract extraction in June 1975. The best corrected visual acuity in the right eye follow ing surgery was hand motions. Multiple areas of elevated fibrous tissue surrounded by pigment pro liferation and atrophy were present in the retina (Fig. 11). Case 7—A 43-year-old woman first seen in Sep-
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tember 1972 had a history of decreased vision in the left eye for four days. Visual acuity was 6/6 (20/20) in the right eye and finger counting in the left eye. A large, dark elevated mass was present in the posteri or pole of the left eye. Surrounding this lesion was an area of preretinal hemorrhage as well as a small hemorrhagic detachment of the pigment epithelium temporal to the lesion. The right eye was normal. Within a week after the initial examination, a massive vitreous hemorrhage obscured further visu alization of the retina. The patient was observed for the next six months, during which time the vitreous blood resolved. In March 1973, visual acuity in this eye was 6/60 (20/200) and an elevated scar was seen superior to the macula. The patient was lost to follow-up. Case 8—A 77-year-old man first seen in December 1974 had a history of decreased vision in the right eye for three weeks. He had been taking quinidine for atrial fibrillation as well as various antihypertensive medications. Visual acuity was finger counting in the right eye and 6/60 (20/20) in the left eye. On the right, a large, elevated dark mass was present in the macular area. The lesion was approximately five disk diameters in size and was surrounded by a ring of blood under the neuroepithelium. The left eye was normal except for a few macular drusen. A diagnosis of hemorrhagic detachment of the pig ment epithelium secondary to disciform degenera tion was made and the patient was observed. In February 1975, the visual acuity had improved to 6/60 (20/200), and a typical disciform scar was seen in the macula. Fluorescein angiography revealed a small area of subretinal neovascularization temporal to the scar. In July 1975, the vision in the involved right eye suddenly decreased to hand motions. An other larger, elevated mass now occupied the superi or and inferior temporal quadrants, and its nasal border was adjacent to the previous scar. A moderate amount of vitreous blood was present. During the next six months, the vitreous blood cleared and the mass was replaced by a large, fibrotic scar surround ed by atrophy and proliferation of the pigment epithelium. The final visual acuity remained hand motion. DISCUSSION
Fig. 11 (Bloome and Ruiz). Case 6. Right eye, five years later. The eye had recently undergone an uneventful cataract extraction. An elevated, crescentic scar surrounded by atrophy and pigment prolifer ation had replaced the subretinal hemorrhage.
Although the number of patients in this series is relatively small, it is, to our knowledge, the largest series of massive spontaneous subretinal hemorrhage ever reported (Table). The eight patients ranged in age from 39 to 77 years with only two patients under the age of 45 years. Four of our patients had a previous history of hyper tension and two were taking anticoagu lants at the time of their hemorrhage. Two patients had disciform macular degenera-
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TABLE SUMMARY OF CASE REPORTS*
Patient Age, Sex Medical No. (yrs) History
Initial Visual Acuity
Mass Size Vitreous (Quadrants) Hemorrhage
32p
Test
1
58,F
—
C.F.
1
+
—
2 3 4 5
48,F 61,M 47,F 39,F
H H,A
2 4 3 1
+ + + +
— — —
6 7
67,M 43,F
H,A
C.F. L.P. H.M. 6/12 (20/40) H.M. C.F.
4 1
-
+
N.D. N.D.
8
77,M
H
H.M.
2
+
N.D.
— — —
N.D.
Final Visual Acuity
Remarks
6/6 (20/20) E E S.D.D., Involved eye H.M. H.M. H.M. S.D.D., Other eye 6/60 (20/200) H.M. S.D.D., Involved eye
*H signifies hypertension; A, anticoagulants; N.D., not done; E, enucleated: S.D.D., senile disciform degeneration; C.F., ringer counting; H.M., hand motions; L.P., light perception.
tion in the involved eye before the hemor rhage and one other patient had a similar diagnosis in the uninvolved eye. The ini tial visual acuity varied from 6/12 (20/40) in one case to finger counting or less in seven cases and was dependent on the site and extent of the hemorrhage as well as the presence of vitreous blood. The final visual acuity was equally poor with one exception (6/6 [20/20]) and depended on the location of the scar tissue. The subretinal hemorrhages were large (two quad rants or more in six cases) and involved the entire fundus in two cases. Vitreous hemorrhage was present in seven cases and took from four months to three years to clear. In four cases, a 3 2 P uptake test was done, and all were negative. One patient developed angle-closure glauco ma caused by rapid enlargement of the mass with anterior displacement of the lens-iris diaphragm. The blood in massive, spontaneous subretinal hemorrhage may originate ei ther in the choroid or beneath the pig ment epithelium. Older reports frequent ly described the hemorrhage as choroidal but the histologic descriptions were often vague and incomplete. 1 - 3 Similar cases
have also been described as originating under the pigment epithelium but most of these cases were not documented histologically. 7,8 Regardless of the initial lo cation of the hemorrhage, the clinical picture is frequently similar. Massive hemorrhage in the choroid or under the pigment epithelium, or both, results in a large, dark mass, and further extravasa tion of the blood anteriorly may result in blood under the retinal neuroepithelium (especially at the borders of the mass) and in the vitreous. The clinical picture of massive, spon taneous subretinal hemorrhage can be caused by several entities. In cases re sulting from hemorrhage under the pig ment epithelium, the hemorrhage usually originates from a subretinal neovascular membrane which begins in the choriocapillaris and extends under the pig ment epithelium after passing through or around Bruch's membrane. 1 1 This sub retinal neovascularization has been described in numerous disorders, includ ing macular and peripheral disciform de generation, ocular histoplasmosis, macu lar drusen, and angioid streaks, and the like. 1 1 - 1 4 Most hemorrhages under the
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pigment epithelium are typically much smaller and less extensive than those in our cases, but massive hemorrhages can occur. Cases 5 and 8 initially had typical hematomas under the pigment epithelium before developing massive hemorrhages. These cases, as well as a few described by Silva and Brockhurst, 7 probably repre sent an extensive form of hemorrhagic detachment of the pigment epithelium. Some cases of massive, spontaneous subretinal hemorrhage (especially those with large masses involving the entire retina) originate in the choroid. Sien 4 de scribed a case of spontaneous choroidal hemorrhage in which a ruptured posterior ciliary artery was demonstrated histologically. A similar mechanism has been demonstrated in expulsive choroidal hem orrhage during or following cataract ex traction, 1 5 - 1 6 and also in a rare entity known as spontaneous expulsive cho roidal hemorrhage, in which an expulsive choroidal hemorrhage follows corneal perforation in eyes with long-standing glaucoma. 17 ' 18 Although these last two disorders differ from our cases in which no glaucoma or ocular hypotony was pre sent, it is possible that a choroidal vessel weakened by arteriosclerosis may bleed spontaneously or during anticoagulant therapy. Choroidal hemorrhage may also originate from a subretinal neovascular membrane in the choriocapillaris before it penetrates Bruch's membrane. Two of our patients with a previous history of disciform degeneration of the macula were taking anticoagulants before their hemorrhage. Wolter and McWilliams 19 and Feeman and associates 20 have reported cases of massive subretinal hem orrhage in patients with disciform macular degeneration who were given antico agulants. Pouliquen and Rousselie 21 also reported a similar case in a patient with no previous ocular disorders. The mas sive hemorrhages seen in our two patients may have resulted from the use of antico
NOVEMBER, 1978
agulants. All ophthalmologists should be aware of this complication in patients with disciform degeneration of the macula. Regardless of the anatomic origin or cause of the hemorrhage, the clinical pic ture must be recognized for proper man agement. A thorough history for the pres ence of hypertension, arteriosclerosis, and drugs such as anticoagulants is man datory. A history of previous visual loss from macular disease or the finding of macular pathology in the other eye may also suggest the correct diagnosis. Frequent fundus examinations with in direct ophthalmoscopy and scleral de pression are the best means of confirming the diagnosis. The presence of a rapidly expanding mass is much more suggestive of subretinal hemorrhage than a tumor. The initial or subsequent appearance of blood under the retinal neuroepithelium and in the vitreous also points to a sub retinal hemorrhage. The eventual ap pearance of an elevated area of fibrosis surrounded by atrophy and pigment pro liferation establishes the diagnosis. Unfortunately, the frequent presence of massive vitreous hemorrhage may ob scure the fundus initially or prevent ade quate follow-up examinations. If a patient has a vitreous hemorrhage (or if it occurs at a later date), bedrest with bilateral patches and head positioning may be helpful to facilitate viewing the lesion. Ultrasonography is essential when the retina cannot be visualized and is also helpful in following the mass when a late hemorrhage occurs. 22 A 32 P test is indicat ed if the lesion is suggestive of a melano ma and can be adequately localized with diathermy on bare sclera. A negative 32 P test makes the diagnosis of melanoma unlikely and warrants following the le sion closely. Ultrasonography is also use ful in confirming the lesion's location before the 32 P test. Once the diagnosis is confirmed, treat ment should be conservative in hopes of
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obtaining useful vision. If angle-closure glaucoma is present, drainage of the cho roidal hemorrhage through a posterior sclerotomy and puncture of the choroid, as well as reformation of the anterior chamber, may be necessary. 5 A vitrectomy late in the course of the disease to remove old, organized blood may result in some visual improvement. A vitrectomy early in the disease may allow the fundus to be visualized but is a dangerous undertaking if an elevated mass is pre sent. SUMMARY
We studied eight cases of massive, spontaneous subretinal hemorrhage which was characterized by the sudden appearance of one or more large, dark, elevated hematomas under the retina. The initial location of the hemorrhage may have been in the choroid or under the pigment epithelium, or both, but further extravasation of the blood anteriorly fre quently resulted in hemorrhage under the neurosensory retina and in the vitreous. Although the lesion can initially be mistaken for a melanoma, the diagnosis is confirmed by the formation of a typical, elevated scar in the involved area. Fre quent fundus examinations are essential, and ultrasound, radioactive phosphorus uptake testing, and bilateral patching are helpful in establishing the diagnosis. The hemorrhage is usually the result of a ruptured vessel from a subretinal neovascular network or arteriosclerotic degener ation and may be precipitated by the use of anticoagulants. REFERENCES 1. Wurdemann, H. V., and Verhoeff, H.: Sponta neous separation of the choroid simulating choroi dal sarcoma. Am. J. Ophthalmol. 10:479, 1927. 2. Seale, E. A.: Rapid interstitial degeneration of the cornea following choroidal hemorrhage. Br. J. Ophthalmol. 15:514, 1931.
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3. Crigler, L. W.: Subchoroidal hemorrhage diag nosed as sarcoma of the choroid. Arch. Ophthalmol. 8:690, 1932. 4. Sien, A. Y.: A case of choroidal apoplexy diag nosed as a sarcoma of the choroid. Ophthalmologica 11:1, 1948. 5. Chan, P. H., and Havner, W, H.: Spontaneous massive choroidal hemorrhage. Am. J. Ophthalmol. 56:568, 1963. 6. Reese, A. B., and Jones, I. S.: Hematomas under the pigment epithelium. Am. J. Ophthalmol. 53:897, 1962. 7. Silva, V. B., and Brockhurst, R. J.: Hemorrhagic detachment of the peripheral pigment epithelium. Arch. Ophthalmol. 94:1295, 1976. 8. Uyama, M., Miyamoto, K., and Ogawa, I.: Subretinal hemorrhage. Folia Ophthalmol. Jap. 20: 692, 1969. 9. Ferry, A. P.: Lesions mistaken for malignant melanoma of the posterior uvea. Arch. Ophthalmol. 72:463, 1964. 10. Berkow, J. W., and Font, R. L.: Disciform macular degeneration with subpigment epithelial hematoma. Arch. Ophthalmol. 82:51, 1969. 11. Gass, J. D. M.: Choroidal neovascular mem branes. Their visualization and treatment. Trans. Am. Acad. Ophthalmol. Otolaryngol. 77:310, 1973. 12. Galinos, S. O., Asdourian, G. K., Wolf, M. B., Goldberg, M. F., and Busse, B. J.: Choroido-vitreal neovascularization after argon laser photocoagulation. Arch. Ophthalmol. 93:524, 1975. 13. Frank, R. N.: Argon laser photocoagulation and subretinal neovascularization. Ophthalmic Surg. 52:56, 1974. 14. Sarks, S. H.: New vessel formation beneath the retinal pigment epithelium in senile eves. Br. J. Ophthalmol. 57:951, 1973. 15. Manschot, W. A.: Glaucoma-vascular necrosis-expulsive hemorrhage. Acta Ophthalmol. 23:309, 1945. 16. The pathology of expulsive hemor rhage. Am. J. Ophthalmol. 40:15, 1955. 17. Williams, D. K., and Rentiers, P. K.: Sponta neous expulsive choroidal hemorrhage. Arch. Oph thalmol. 83:191, 1970. 18. Winslow, R. L., Stevenson, W., and Yanoff, M.: Spontaneous expulsive choroidal hemorrhage. Arch. Ophthalmol. 92:33, 1974. 19. Wolter, J. R., and McWilliams, J. R.: Rupture of disciform macular degeneration. Am. J. Ophthal mol. 59:1044, 1965. 20. Feeman, S. S., Bartlett, R. E., Roth, A. M., and Foos, R. Y.: Intraocular hemorrhage and blind ness associated with systemic anticoagulation. J. A. M. A. 220:1354, 1972. 21. Pouliquen, Y., and Rousselie, F.: Spontane ous choroidal hematoma during anticoagulant treat ment. Bull. Soc. Ophthalmol. 66:5, 1966. 22. Hodes, B. L., and Choromokos, E.: Standard ized a-scan echographic diagnosis of choroidal ma lignant melanomas. Arch. Ophthalmol. 95:593, 1977.