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Limited Choroidal Hemorrhage Mistaken for a Choroidal Melanoma
Limited Choroidal Hemorrhage Mistaken for a Choroidal Melanoma CRAIG M. MORGAN, MD, *t EVANGELOS S. GRAGOUDAS, MD*
Abstract: Considerable progress has been made during the last few years in evaluating patients with suspected choroidal melanomas but difficulties continue to persist. In this report, the authors describe three cases with an unusual localized posterior choroidal hemorrhage, which were thought to be choroidal melanomas and referred for proton beam irradiation. These limited hemorrhagic choroidal detachments presented as a dark brown mass of considerable elevation, but were discrete, well localized, and located posterior to the equator. Fluorescein angiography and ultrasonography may be of some value in differentiating these lesions from choroidal melanomas. Serial observations over time will establish the correct diagnosis. [Key words: choroidal detachment, choroidal effusion, choroidal hemorrhage, choroidal melanoma, expulsive choroidal hemorrhage, fluorescein angiography, limited choroidal hemorrhage, rhegmatogenous retinal detachment, ultrasonography.] Ophthalmology 94:41-46, 1987
Although several improvements have been made during the last few years in our ability to evaluate patients with suspected choroidal melanomas,I,2 difficulties continue to persist. Failure to differentiate a benign simulating lesion from a melanoma can lead to unnecessary treatment or enucleation. We report three cases with an unusual 10calized posterior choroidal hemorrhage, which were thought to be choroidal melanomas and were referred for proton beam irradiation.
CASE REPORTS Case 1. An 86-year-old man was referred for management of his choroidal melanoma on June 8, 1983. His past ocular history was significant for myopia and chronic open-angle glaucoma of both eyes treated with 2% pilocarpine. He had had muscle surgery as a child for right esotropia with a resultant 70-prism-diopter (PD) consecutive right exotropia and mild ambylopia in his right eye. He underwent an uneventful intracapsular cataract extrac-
From the Retina Service of the Massachusetts Eye and Ear Infirmary, and the Department of Ophthalmology: Harvard Medical School, Boston, and the Vitreo-Retina Service,t the W. K. Kellogg Eye Center, the University of Michigan Medical Center, Ann Arbor. Reprint requests to Evangelos S. Gragoudas, MD, Retina Service, Mas· sachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114.
tion of his left eye in 1978, which with aphakic correction had a visual acuity of 20/40. His past medical history was positive for a prostatectomy in 1970. The vision in his right eye deteriorated secondary to a cataract, and on May 31, 1983, an extracapsular cataract extraction under local anesthesia was performed. This was complicated by vitreous loss requiring an anterior vitrectomy. Preoperative examination of the posterior segment was reportedly normal except for pathologic cupping of the optic nerve. When the patient was seen on the first postoperative day, a "superior retinal detachment" was noted with an intraocular pressure of 18 mmHg. He was referred to a retina specialist who noted a "tumor underneath the retinal detachment." Results of a complete systemic evaluation by an internist, including a liver and brain scan, was normal. The patient was then referred for proton beam irradiation. On our initial examination, visual acuity of the right eye was counting fingers at 5 feet. Current ophthalmic medications included Cyclogyl (cyclopentolate) and a neomycin/polymyxin B/ 0.1 % dexamethasone combination. Results of an examination of the anterior segment showed a clear cornea with a well-opposed cataract wound, minimal anterior chamber inflammation, and a ruptured posterior capsule. Intraocular pressure was 26 mmHg. Results of a fundus examination showed a brown choroidallesion in the superior fundus extending from the superior temporal arcade to the equator (Fig 1, top left). Results of an examination ofthe left eye showed only retinal pigment epithelial changes in the macula. Immersion A- and B-mode ultrasonography results showed an aphakic globe with an axial length of 26.9 mm. A solid lesion was seen superiorly with a maximum height of7.5 mm (Fig 1, bottom left). Standardized A-scan ul-
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Fig 1. Limited choroidal hemorrhage after cataract extraction. Top left. wide-angle photograph. Bottom left. immersion B-mode ultrasonography of the same lesion. It is a solid-appearing lesion without choroidal excavation and maximum height of 7.5 mm. Bottom right. immersion B-mode ultrasonography 2.5 weeks later. The lesion is now only 2.5 mm in height.
trasonography results demonstrated a moderately high amplitude spike followed by attenuated spikes. No choroidal excavation was seen. On fluorescein angiography, minimal hyperfluorescence of the lesion was seen without leakage on the late pictures. Our diagnosis was a limited choroidal hemorrhage, which 0ccurred after cataract extraction, although the possibility of a choroidal malignant melanoma could not be excluded. Observation of the lesion was recommended. The patient was re-examined 21/2 weeks later. Visual acuity and intraocular pressure were unchanged. The lesion had the same color, but had decreased in size. Results from ultrasonography showed that the lesion measured only 2.5 mm in height (Fig I, bottom right). The internal texture was fairly homogenous with low reflectivity compatible with a fluid-filled space. Fluorescein angiography showed choroidal folds, and the diagnosis of a choroidal hemorrhage was established. Two weeks later, the patient's visual acuity improved to 20/70 and the intraocular pressure decreased to 16 mmHg. The choroidal hemorrhage had continued to decrease in size. The patient was last seen four months after his cataract extraction. His visual acuity was 20/40. There was now only a minimal amount of choroidal hemorrhage above the superior arcade.
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Case 2. A 68-year-old woman presented to her ophthalmologist with a one-week history of a "curtain" over the superior half of the visual field in her left eye associated with floaters and occasional photopsias. Her past medical history showed congestive heart failure and mitral valve disease, which required a commissurectomy in 1959. Current medications included Coumadin (warfarin sodium crystalline) 5 mg every other day alternating with 2.5 mg and digoxin 0 .25 mg daily. She was also on Dilantin (Phenytoin) 100 mg daily because of head trauma sustained in a motor vehicle accident 20 years previously. A retinal lesion was found in her left eye, and the patient was referred to a retina specialist who found a "pigmented elevated lesion associated with a secondary retinal detachment." He made the diagnosis of a choroidal malignant melanoma and referred the patient to us for confirmation of the diagnosis and appropriate treatment. On our initial examination, her visual acuity was 20/30 in the right eye and 20/60 in the left. Intraocular pressure was 13 mmHg in the right eye and II mmHg in the left. In the left eye, a brown-reddish subretinal elevated lesion was found in the midinferonasal quadrant (Fig 2, top left and right). An associated retinal detachment was present involving the inferior half of the retina without shifting of fluid. Immersion and contact ultra-
MORGAN AND GRAGOUDAS •
sonography results showed that the lesion had a maximum height of 2.6 mm. Results from standardized A-scan ultrasonography showed a high spike followed by rapid sound attenuation. The diagnosis was uncertain, but a choroidal melanoma could not be excluded. A period of observation was suggested. Two and a half weeks later, the visual acuity was unchanged, but the intraocular pressure was 4 mmHg. The choroidal lesion had changed color and was much smaller. A diagnosis of a retinal detachment associated with a resolving small hemorrhagic choroidal detachment was made. She was followed by the referring retina specialist until her death three months later. During that time, the lesion had continued to decrease in size. Case 3. A 72-year-old woman was referred to us on October 27, 1981, with a diagnosis ofa choroidal malignant melanoma in the right eye. Past ocular history showed bilateral intracapsular cataract extractions in August 1973 and a scleral buckling procedure in the right eye on December 17, 1979, for a rhegmatogenous retinal detachment involving the temporal retina. A silicone implant was applied from the 8- to 1:30-0'clock position under an encircling band. She also had chronic open-angle glaucoma treated with 0.5% timolol in both eyes. Her medical history included adult onset diabetes mellitus and hypertension treated with Orinase (tolbutamide) and Lasix (furosemide), respectively. One month before our examination, a persistent "headache" developed in the region of her right eye. She was seen by her ophthalmologist who found a "large melanoma tumor inferonasally" in the right eye. A metastatic workup including a brain scan was performed. On examination, her visual acuity was 20170 in the right eye and 20/40 in the left eye. Intraocular pressure was 24 mmHg in both eyes. Results from an anterior segment examination showed well-healed cataract wounds. In the inferonasal quadrant of the right eye, a dark choroidal lesion was seen extending from posterior to the circumferential buckle to approximately 2 disc diameters from the optic disc (Fig 3, second row left and right). The surface of the lesion had a smooth appearance and no retinal pigment epithelial changes were present. Results from immersion ultrasonography showed an aphakic globe with an axial length of29.4 mm. A dome-shaped homogenous mass lesion was seen just posterior to the scleral buckle with a maximum height of 5.1 mm. Standardized A-scan echography results demonstrated rapid acoustic attenuation of the internal echoes. On fluorescein angiography (Fig 3, third row left and right), there was hyperfluorescence of the lesion during the transit with no evidence of vascularity or leakage on the late films. A diagnosis of a limited choroidal hemorrhage simulating a choroidal malignant melanoma was made, and observation was suggested. The patient was re-examined six weeks later, and the dark lesion had nearly resolved (Fig 3, bottom left and right).
DISCUSSION The suprachoroidal space is a potential space which is external to the main layers of the choroid. This space is approximately 30 ~m in thickness, and consists of a fine network of collagen laminas arranged tangentially and attached to the sclera. 3 Normally, approximately 10 ~l of fluid are present in the suprachoroidal space; this acts as a lubricant and aids in the movement of the choroid during accommodation. 4 If the rate of fluid accumulation in the suprachoroidal space is greater than the exit of fluid, a choroidal detachment develops. This is most often seen after intraocular surgery where hypotony and inflam-
LIMITED CHOROIDAL HEMORRHAGE
mation are created, but may also occur spontaneously,S after trauma, 3 or in association with uveitis. 6 Occasionally, formation of a choroidal detachment may be delayed considerably after intraocular surgery. Systemic disorders such as hypertension or arteriosclerotic disease may decrease the ability of the choroidal vessels to retain fluid and promote further transudation of fluid into the suprachoroidal space. 4 Hemorrhage in the suprachoroidal space is less common, but can result from similar abnormal conditions such as hypotony, inflammation, trauma, and vascular diseases, which compromise the vasculature boarding the suprachoroidal space. 7 The elderly have an increased risk of having hemorrhagic choroidal detachments. 3 Nonexpulsive suprachoroidal hemorrhages can occur during an intraocular operation, postoperatively or spontaneously.7,8 This is usually associated with rupture of one of the long or short posterior ciliary arteries into the suprachoroidal space. The exact mechanism of delayed postoperative choroidal hemorrhage is not fully understood, but is often related to prolonged hypotony. In some instances, a small choroidal hemorrhage may occur initially which then stops spontaneously, and then at a future date rebleeding occurs.9 Many of the patients, in whom choroidal hemorrhage develops, suffer from glaucoma preoperatively.8,10 Occasionally, a small or limited hemorrhagic choroidal detachment can develop postoperatively. These limited choroidal hemorrhages demonstrate the mechanical restrictions that the tangential sclera fibers provide in the suprachoroidal space, and may represent a limited or aborted form of an expulsive choroidal hemorrhage. 3 Hoffman et alii have recently reported a group of 16 patients who had such a limited choroidal hemorrhage after intracapsular cataract extraction. These hemorrhages were usually dull red in appearance, triangular in shape with the apex directed towards the optic disc, posterior to the equator, and often associated with elevation of the overlying retina. This appearance was believed to be consistent with a rupture of a branch of short or long posterior ciliary artery. Predisposing factors included myopia and ischemic or hypertensive cardiovascular disease. Patients with a rhegmatogenous retinal detachment may present with an associated choroidal detachment. This combined problem has been found to be present in 2 to 4.5% ofrhegmatogenous retinal detachments. 12,13 The choroidal detachment in these cases is usually large, serous, peripheral in location, and assumes an annular configuration. 14 These patients are usually older, and a significant number have a past history of ocular trauma or intraocular surgery.3 Choroidal detachments are also commonly associated with retinal detachment surgery occurring in up to 40% of patients after scleral buckling procedures. 15 Drainage of subretinal fluid with associated hypotony, increased episcleral venous pressure after vortex vein compression from a scleral buckle, and trauma and inflammation after cryopexy or diathermy contribute to the formation of choroidal detachments,3,4 although the retinal detachment itself could be a factor in creating suprachoroidal edema. 16 43
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LIMITED CHOROIDAL HEMORRHAGE
Fig 2. Limited choroidal hemorrhage associated with retinal detachment. Top left, wide-angle photograph. Top right, 30° fundus photograph. Fig 3. Limited choroidal hemorrhage 22 months after retinal detachment surgery. Second row left, wide-angle photograph. Second row right, 30° fundus photograph. Third row left, fluorescein angiogram, early arteriovenous phase. Third row right, fluorescein angiogram, late arteriovenous phase. Wideangle (bottom left) and 30° photographs (bottom right) six weeks later. The choroidal hemorrhage has decreased in size.
(
Choroidal detachments associated with retinal detachment surgery may be serous or hemorrhagic depending on the extent of vascular damage. The three patients reported herein had limited hemorrhagic choroidal detachments. The first patient had a limited hemorrhagic choroidal detachment immediately after cataract extraction complicated by vitreous loss requiring an anterior vitrectomy. The second patient had a limited hemorrhagic choroidal detachment associated with a retinal detachment, and the third patient presented with a limited hemorrhagic choroidal detachment 8 years after an intracapsular cataract extraction and 22 months after repair of a rhegmatogenous retinal detachment. All three patients had risk factors, which are associated with the development of hemorrhagic choroidal detachments; all were myopic and older than 68 years of age, two had chronic glaucoma requiring medical treatment, and two had systemic cardiovascular disease. In addition, two of the three patients had previous intraocular surgery, and the remaining patient had an associated retinal detachment. One patient was also on Coumadin therapy for mitral valve disease. On ophthalmoscopy, many benign and malignant lesions can simulate a choroidal malignant melanoma. 1 In 1964, Ferry17 reported that 19% of eyes with clear media enucleated for a suspected posterior uveal melanoma contained a lesion other than a malignant melanoma. In 1973, Shields and Zimmerman l8 updated Ferry's study and found that the incidence of eyes enucleated for simulating lesions was still 20%. Although the incidence of misdiagnosis is much less at referral centers, 1,19,20 lesions masquerading as a posterior uveal melanoma still frequently present diagnostic dilemmas. This can lead to unnecessary treatment such as radiotherapy or even enucleation. The three patients in this report with limited hemorrhagic choroidal detachments were all initially believed to have a choroidal malignant melanoma. Choroidal detachments have been described previously as simulating a choroidal melanoma and many eyes have been enucleated based on this erroneous diagnosis. 2,2I,22-24 Most of these eyes enucleated for a suspected choroidal melanoma had large, smooth, peripheral serous choroidal detachments occurring in association with intraocular surgery or uveitis,I,3,17,18,21 or had expulsive hemorrhagic choroidal detachments. 25 Others were reported as having blood in the suprachoroidal space l7 ,26 or "subretinal or choroidal hemorrhage.'oZ? Although a new choroidal hemorrhage may appear reddish in color, when organization of the hemorrhage occurs, the hemosiderin content and proliferation of the overlying retinal pigment epithelium may make the choroidal hemorrhage resemble a pigmented choroidal melanoma. 28,29 On the other hand, choroidal melanomas may also
present as a typical choroidal hemorrhagic detachment. Reese 30 reported a case that was thought to be a choroidal hemorrhage initially but subsequently was found to be a choroidal melanoma. He stated that it was possible that the choroidal hemorrhage had existed and masked the melanoma at the initial examination. Shields et al31 have also described a case where, in what appeared to be a typical postoperative choroidal detachment, a multilobed uveal melanoma was harbored, which was not appreciated preoperatively. Although large, multilobular, peripheral, serous choroidal detachments occurring in the immediate postoperative period are less likely to be confused with a choroidal melanoma, a hemorrhagic choroidal detachment may closely mimic a choroidal melanoma. This is especially true when the clinical findings are similar to the ones found in our patients. These patients had a limited hemorrhagic choroidal detachment which presented as a dark brown or reddish brown mass that measured 2.6 to 7.5 mm in height. Despite this considerable elevation, the lesions were discrete and well localized. Furthermore, all these limited choroidal hemorrhages were located posterior to the equator without ciliary body or peripheral choroidal involvement. Fluorescein angiography in our patients did not show any late leakage or staining, which is commonly associated with choroidal melanomas, although longstanding choroidal detachments may occasionally show leakage through defects in the retinal pigment epithelium. A serous choroidal detachment characteristically transmits light on transillumination, whereas a pigmented melanoma does not; however, transillumination of a hemorrhagic choroidal detachment will also cast a shadow. In addition, as in our patients, ultrasonography can demonstrate low to medium internal reflectivity in hemorrhagic choroidal detachments similar to that seen with a choroidal melanoma. 32 A careful history and a complete medical and ocular examination of both eyes should be performed in every case of a suspicious fundus lesion. However, the most important diagnostic test is continued observation of suspicious lesions. Serial observations over time will most likely establish the correct diagnosis.
REFERENCES 1. Shields JA, Augsburger JJ, Brown GC, Stephens RF. The differential diagnosis of posterior uveal melanoma. Ophthalmology 1980; 87:51822. 2. Shields JA, McDonald R. Improvements in the diagnosis of posterior uveal melanomas. Arch Ophthalmol 1974; 91 :259-64. 3. Fogle JA, Green WR. Ciliochoroidal effusion. In: Duane TO, Jaeger EA, eds. Clinical Ophthalmology. Philadelphia: Harper and Row, 1982; Volume 4, Chapter 63: 1-31.
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4. Brubaker RF, Pederson JE. Ciliochoroidal detachment. SUN Ophthalmol 1983; 27:281-9. 5. Schepens CL, Brockhurst RJ. Uveal effusion. 1. Clinical picture. Arch Ophthalmol1963; 70:189-201. 6. McGrand JC. Choroidal detachment in aphakic uveitis. Br J Ophthalmol 1969; 53:778-81. 7. Brubaker RF. Intraocular surgery and choroidal hemorrhage. Arch Ophthalmol 1984; 102: 1753-4. 8. Gressel MG, Parrish RK, Heuer OK. Delayed nonexpulsive suprachoroidal hemorrhage. Arch Ophthalmol1984; 102:1757-60. 9. Yanoff M. Fine BS. Surgical and nonsurgical trauma. Ocular Pathology, A Text and Atlas, 2nd ed. Vol. 138. Hagerstown: Harper & Row, 1982; 143-6. 10. Taylor OM. Expulsive hemorrhage. Am J Ophthalmol1974; 78:961-
6. 11. Hoffman P, Pollack A, Oliver M. Limited choroidal hemorrhage associated with intracapsular cataract extraction. Arch Ophthalmol1984; 102:1761-5. 12. Seelenfreund MH, Kraushar MF, Schepens CL, Freilich DB. Choroidal detachment associated with primary retinal detachment. Arch Ophthalmol1974; 91 :254-8. 13. Gottlieb F. Combined choroidal and retinal detachment. Arch Ophthalmol1972; 88:481-6. 14. Graham PA. Unusual evolution of retinal detachments. Trans Ophthalmol Soc UK 1958; 78:359-71. 15. Packer AJ, Maggiano JM, Aaberg TM, Meredith TA, Reeser FH, Kingham JD. Serous choroidal detachment after retinal detachment surgery. Arch Ophthalmol 1983; 101: 1221-4. 16. Aaberg TM. Experimental serous and hemorrhagic uveal edema associated with retinal detachment surgery. Invest Ophthalmol 1975; 14:243-6. 17. Ferry AP. Lesions mistaken for malignant melanoma of the posterior uvea. Arch Ophthalmol 1964; 72:463-9.
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18. Shields JA, Zimmerman LE. Lesions simulating malignant melanoma of the posterior uvea. Arch Ophthalmol 1973; 89:466-71. 19. Robertson OM, Campbell RJ. Errors in the diagnosis of malignant melanoma of the choroid. Am J Ophthalmol1979; 87:269-75. 20. Davidorf FH, Letson AD, Weiss ET, Levine E. Incidence of misdiagnosed and unsuspected choroidal melanomas: a 50-year experience. Arch Ophthalmol 1983; 101 :410-2. 21. Bard LA. Eyes with choroidal detachments removed for suspected melanoma. Arch Ophthalmol 1965; 73:320-3. 22. Zimmerman LE. Problems in the diagnosis of malignant melanomas of the choroid and Ciliary body. Am J Ophthalmol1973; 75:917-29. 23. Dunphy EB, Forrest AW, Leopold IH, Reese AB, Zimmerman LE. The diagnosis and management of intraocular melanomas. Trans Am Acad Ophthalmol Otolaryngol1958; 62:517-555. 24. Addison OJ, Wakelin DL. Errors in the diagnosis of choroidal malignant melanoma. Can J Ophthalmol1975; 10:205-6. 25. Chang M, Zimmerman LE, McLean I. The persisting pseudomelanoma problem. Arch Ophthalmol1984; 102:726-7. 26. Rones B, Zimmerman LE. An unusual choroidal hemorrhage simulating malignant melanoma. Arch OphthalmoI1963; 70:30-2. 27. Kirk HQ, Petty RW. Malignant melanoma of the choroid: a correlation of clinical and histological findings. Arch Ophthalmol 1956; 56:84360. 28. Reese AB. Pigmented tumors. Tumors of the Eye, 3rd ed. New York: Harper & Row, 1976; 216-22. 29. Hutchinson BT. Postoperative choroidal hemorrhage. In: Chandler, PA. Grant WM, eds. Glaucoma, 2nd ed. Philadelphia: Lea & Febiger, 1979; 316-7. 30. Reese AB. The differential diagnosis of malignant melanoma of the choroid. Arch Ophthalmol1957; 58:477-82. 31. Shields JA, Leonard BC, Sarin LK. Multilobed uveal melanoma masquerading as postoperative choroidal detachment. Br J Ophthalmol 1976; 60:386-9. 32. Shields JA. Current approaches to the diagnosis and management of choroidal melanomas. SUN OphthalmoI1977; 21:443-63.
Abstract: Considerable progress has been made during the last few years in evaluating patients with suspected choroidal melanomas but difficulties continue to persist. In this report, the authors describe three cases with an unusual localized posterior choroidal hemorrhage, which were thought to be choroidal melanomas and referred for proton beam irradiation. These limited hemorrhagic choroidal detachments presented as a dark brown mass of considerable elevation, but were discrete, well localized, and located posterior to the equator. Fluorescein angiography and ultrasonography may be of some value in differentiating these lesions from choroidal melanomas. Serial observations over time will establish the correct diagnosis. [Key words: choroidal detachment, choroidal effusion, choroidal hemorrhage, choroidal melanoma, expulsive choroidal hemorrhage, fluorescein angiography, limited choroidal hemorrhage, rhegmatogenous retinal detachment, ultrasonography.] Ophthalmology 94:41-46, 1987
Although several improvements have been made during the last few years in our ability to evaluate patients with suspected choroidal melanomas,I,2 difficulties continue to persist. Failure to differentiate a benign simulating lesion from a melanoma can lead to unnecessary treatment or enucleation. We report three cases with an unusual 10calized posterior choroidal hemorrhage, which were thought to be choroidal melanomas and were referred for proton beam irradiation.
CASE REPORTS Case 1. An 86-year-old man was referred for management of his choroidal melanoma on June 8, 1983. His past ocular history was significant for myopia and chronic open-angle glaucoma of both eyes treated with 2% pilocarpine. He had had muscle surgery as a child for right esotropia with a resultant 70-prism-diopter (PD) consecutive right exotropia and mild ambylopia in his right eye. He underwent an uneventful intracapsular cataract extrac-
From the Retina Service of the Massachusetts Eye and Ear Infirmary, and the Department of Ophthalmology: Harvard Medical School, Boston, and the Vitreo-Retina Service,t the W. K. Kellogg Eye Center, the University of Michigan Medical Center, Ann Arbor. Reprint requests to Evangelos S. Gragoudas, MD, Retina Service, Mas· sachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114.
tion of his left eye in 1978, which with aphakic correction had a visual acuity of 20/40. His past medical history was positive for a prostatectomy in 1970. The vision in his right eye deteriorated secondary to a cataract, and on May 31, 1983, an extracapsular cataract extraction under local anesthesia was performed. This was complicated by vitreous loss requiring an anterior vitrectomy. Preoperative examination of the posterior segment was reportedly normal except for pathologic cupping of the optic nerve. When the patient was seen on the first postoperative day, a "superior retinal detachment" was noted with an intraocular pressure of 18 mmHg. He was referred to a retina specialist who noted a "tumor underneath the retinal detachment." Results of a complete systemic evaluation by an internist, including a liver and brain scan, was normal. The patient was then referred for proton beam irradiation. On our initial examination, visual acuity of the right eye was counting fingers at 5 feet. Current ophthalmic medications included Cyclogyl (cyclopentolate) and a neomycin/polymyxin B/ 0.1 % dexamethasone combination. Results of an examination of the anterior segment showed a clear cornea with a well-opposed cataract wound, minimal anterior chamber inflammation, and a ruptured posterior capsule. Intraocular pressure was 26 mmHg. Results of a fundus examination showed a brown choroidallesion in the superior fundus extending from the superior temporal arcade to the equator (Fig 1, top left). Results of an examination ofthe left eye showed only retinal pigment epithelial changes in the macula. Immersion A- and B-mode ultrasonography results showed an aphakic globe with an axial length of 26.9 mm. A solid lesion was seen superiorly with a maximum height of7.5 mm (Fig 1, bottom left). Standardized A-scan ul-
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Fig 1. Limited choroidal hemorrhage after cataract extraction. Top left. wide-angle photograph. Bottom left. immersion B-mode ultrasonography of the same lesion. It is a solid-appearing lesion without choroidal excavation and maximum height of 7.5 mm. Bottom right. immersion B-mode ultrasonography 2.5 weeks later. The lesion is now only 2.5 mm in height.
trasonography results demonstrated a moderately high amplitude spike followed by attenuated spikes. No choroidal excavation was seen. On fluorescein angiography, minimal hyperfluorescence of the lesion was seen without leakage on the late pictures. Our diagnosis was a limited choroidal hemorrhage, which 0ccurred after cataract extraction, although the possibility of a choroidal malignant melanoma could not be excluded. Observation of the lesion was recommended. The patient was re-examined 21/2 weeks later. Visual acuity and intraocular pressure were unchanged. The lesion had the same color, but had decreased in size. Results from ultrasonography showed that the lesion measured only 2.5 mm in height (Fig I, bottom right). The internal texture was fairly homogenous with low reflectivity compatible with a fluid-filled space. Fluorescein angiography showed choroidal folds, and the diagnosis of a choroidal hemorrhage was established. Two weeks later, the patient's visual acuity improved to 20/70 and the intraocular pressure decreased to 16 mmHg. The choroidal hemorrhage had continued to decrease in size. The patient was last seen four months after his cataract extraction. His visual acuity was 20/40. There was now only a minimal amount of choroidal hemorrhage above the superior arcade.
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Case 2. A 68-year-old woman presented to her ophthalmologist with a one-week history of a "curtain" over the superior half of the visual field in her left eye associated with floaters and occasional photopsias. Her past medical history showed congestive heart failure and mitral valve disease, which required a commissurectomy in 1959. Current medications included Coumadin (warfarin sodium crystalline) 5 mg every other day alternating with 2.5 mg and digoxin 0 .25 mg daily. She was also on Dilantin (Phenytoin) 100 mg daily because of head trauma sustained in a motor vehicle accident 20 years previously. A retinal lesion was found in her left eye, and the patient was referred to a retina specialist who found a "pigmented elevated lesion associated with a secondary retinal detachment." He made the diagnosis of a choroidal malignant melanoma and referred the patient to us for confirmation of the diagnosis and appropriate treatment. On our initial examination, her visual acuity was 20/30 in the right eye and 20/60 in the left. Intraocular pressure was 13 mmHg in the right eye and II mmHg in the left. In the left eye, a brown-reddish subretinal elevated lesion was found in the midinferonasal quadrant (Fig 2, top left and right). An associated retinal detachment was present involving the inferior half of the retina without shifting of fluid. Immersion and contact ultra-
MORGAN AND GRAGOUDAS •
sonography results showed that the lesion had a maximum height of 2.6 mm. Results from standardized A-scan ultrasonography showed a high spike followed by rapid sound attenuation. The diagnosis was uncertain, but a choroidal melanoma could not be excluded. A period of observation was suggested. Two and a half weeks later, the visual acuity was unchanged, but the intraocular pressure was 4 mmHg. The choroidal lesion had changed color and was much smaller. A diagnosis of a retinal detachment associated with a resolving small hemorrhagic choroidal detachment was made. She was followed by the referring retina specialist until her death three months later. During that time, the lesion had continued to decrease in size. Case 3. A 72-year-old woman was referred to us on October 27, 1981, with a diagnosis ofa choroidal malignant melanoma in the right eye. Past ocular history showed bilateral intracapsular cataract extractions in August 1973 and a scleral buckling procedure in the right eye on December 17, 1979, for a rhegmatogenous retinal detachment involving the temporal retina. A silicone implant was applied from the 8- to 1:30-0'clock position under an encircling band. She also had chronic open-angle glaucoma treated with 0.5% timolol in both eyes. Her medical history included adult onset diabetes mellitus and hypertension treated with Orinase (tolbutamide) and Lasix (furosemide), respectively. One month before our examination, a persistent "headache" developed in the region of her right eye. She was seen by her ophthalmologist who found a "large melanoma tumor inferonasally" in the right eye. A metastatic workup including a brain scan was performed. On examination, her visual acuity was 20170 in the right eye and 20/40 in the left eye. Intraocular pressure was 24 mmHg in both eyes. Results from an anterior segment examination showed well-healed cataract wounds. In the inferonasal quadrant of the right eye, a dark choroidal lesion was seen extending from posterior to the circumferential buckle to approximately 2 disc diameters from the optic disc (Fig 3, second row left and right). The surface of the lesion had a smooth appearance and no retinal pigment epithelial changes were present. Results from immersion ultrasonography showed an aphakic globe with an axial length of29.4 mm. A dome-shaped homogenous mass lesion was seen just posterior to the scleral buckle with a maximum height of 5.1 mm. Standardized A-scan echography results demonstrated rapid acoustic attenuation of the internal echoes. On fluorescein angiography (Fig 3, third row left and right), there was hyperfluorescence of the lesion during the transit with no evidence of vascularity or leakage on the late films. A diagnosis of a limited choroidal hemorrhage simulating a choroidal malignant melanoma was made, and observation was suggested. The patient was re-examined six weeks later, and the dark lesion had nearly resolved (Fig 3, bottom left and right).
DISCUSSION The suprachoroidal space is a potential space which is external to the main layers of the choroid. This space is approximately 30 ~m in thickness, and consists of a fine network of collagen laminas arranged tangentially and attached to the sclera. 3 Normally, approximately 10 ~l of fluid are present in the suprachoroidal space; this acts as a lubricant and aids in the movement of the choroid during accommodation. 4 If the rate of fluid accumulation in the suprachoroidal space is greater than the exit of fluid, a choroidal detachment develops. This is most often seen after intraocular surgery where hypotony and inflam-
LIMITED CHOROIDAL HEMORRHAGE
mation are created, but may also occur spontaneously,S after trauma, 3 or in association with uveitis. 6 Occasionally, formation of a choroidal detachment may be delayed considerably after intraocular surgery. Systemic disorders such as hypertension or arteriosclerotic disease may decrease the ability of the choroidal vessels to retain fluid and promote further transudation of fluid into the suprachoroidal space. 4 Hemorrhage in the suprachoroidal space is less common, but can result from similar abnormal conditions such as hypotony, inflammation, trauma, and vascular diseases, which compromise the vasculature boarding the suprachoroidal space. 7 The elderly have an increased risk of having hemorrhagic choroidal detachments. 3 Nonexpulsive suprachoroidal hemorrhages can occur during an intraocular operation, postoperatively or spontaneously.7,8 This is usually associated with rupture of one of the long or short posterior ciliary arteries into the suprachoroidal space. The exact mechanism of delayed postoperative choroidal hemorrhage is not fully understood, but is often related to prolonged hypotony. In some instances, a small choroidal hemorrhage may occur initially which then stops spontaneously, and then at a future date rebleeding occurs.9 Many of the patients, in whom choroidal hemorrhage develops, suffer from glaucoma preoperatively.8,10 Occasionally, a small or limited hemorrhagic choroidal detachment can develop postoperatively. These limited choroidal hemorrhages demonstrate the mechanical restrictions that the tangential sclera fibers provide in the suprachoroidal space, and may represent a limited or aborted form of an expulsive choroidal hemorrhage. 3 Hoffman et alii have recently reported a group of 16 patients who had such a limited choroidal hemorrhage after intracapsular cataract extraction. These hemorrhages were usually dull red in appearance, triangular in shape with the apex directed towards the optic disc, posterior to the equator, and often associated with elevation of the overlying retina. This appearance was believed to be consistent with a rupture of a branch of short or long posterior ciliary artery. Predisposing factors included myopia and ischemic or hypertensive cardiovascular disease. Patients with a rhegmatogenous retinal detachment may present with an associated choroidal detachment. This combined problem has been found to be present in 2 to 4.5% ofrhegmatogenous retinal detachments. 12,13 The choroidal detachment in these cases is usually large, serous, peripheral in location, and assumes an annular configuration. 14 These patients are usually older, and a significant number have a past history of ocular trauma or intraocular surgery.3 Choroidal detachments are also commonly associated with retinal detachment surgery occurring in up to 40% of patients after scleral buckling procedures. 15 Drainage of subretinal fluid with associated hypotony, increased episcleral venous pressure after vortex vein compression from a scleral buckle, and trauma and inflammation after cryopexy or diathermy contribute to the formation of choroidal detachments,3,4 although the retinal detachment itself could be a factor in creating suprachoroidal edema. 16 43
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Fig 2. Limited choroidal hemorrhage associated with retinal detachment. Top left, wide-angle photograph. Top right, 30° fundus photograph. Fig 3. Limited choroidal hemorrhage 22 months after retinal detachment surgery. Second row left, wide-angle photograph. Second row right, 30° fundus photograph. Third row left, fluorescein angiogram, early arteriovenous phase. Third row right, fluorescein angiogram, late arteriovenous phase. Wideangle (bottom left) and 30° photographs (bottom right) six weeks later. The choroidal hemorrhage has decreased in size.
(
Choroidal detachments associated with retinal detachment surgery may be serous or hemorrhagic depending on the extent of vascular damage. The three patients reported herein had limited hemorrhagic choroidal detachments. The first patient had a limited hemorrhagic choroidal detachment immediately after cataract extraction complicated by vitreous loss requiring an anterior vitrectomy. The second patient had a limited hemorrhagic choroidal detachment associated with a retinal detachment, and the third patient presented with a limited hemorrhagic choroidal detachment 8 years after an intracapsular cataract extraction and 22 months after repair of a rhegmatogenous retinal detachment. All three patients had risk factors, which are associated with the development of hemorrhagic choroidal detachments; all were myopic and older than 68 years of age, two had chronic glaucoma requiring medical treatment, and two had systemic cardiovascular disease. In addition, two of the three patients had previous intraocular surgery, and the remaining patient had an associated retinal detachment. One patient was also on Coumadin therapy for mitral valve disease. On ophthalmoscopy, many benign and malignant lesions can simulate a choroidal malignant melanoma. 1 In 1964, Ferry17 reported that 19% of eyes with clear media enucleated for a suspected posterior uveal melanoma contained a lesion other than a malignant melanoma. In 1973, Shields and Zimmerman l8 updated Ferry's study and found that the incidence of eyes enucleated for simulating lesions was still 20%. Although the incidence of misdiagnosis is much less at referral centers, 1,19,20 lesions masquerading as a posterior uveal melanoma still frequently present diagnostic dilemmas. This can lead to unnecessary treatment such as radiotherapy or even enucleation. The three patients in this report with limited hemorrhagic choroidal detachments were all initially believed to have a choroidal malignant melanoma. Choroidal detachments have been described previously as simulating a choroidal melanoma and many eyes have been enucleated based on this erroneous diagnosis. 2,2I,22-24 Most of these eyes enucleated for a suspected choroidal melanoma had large, smooth, peripheral serous choroidal detachments occurring in association with intraocular surgery or uveitis,I,3,17,18,21 or had expulsive hemorrhagic choroidal detachments. 25 Others were reported as having blood in the suprachoroidal space l7 ,26 or "subretinal or choroidal hemorrhage.'oZ? Although a new choroidal hemorrhage may appear reddish in color, when organization of the hemorrhage occurs, the hemosiderin content and proliferation of the overlying retinal pigment epithelium may make the choroidal hemorrhage resemble a pigmented choroidal melanoma. 28,29 On the other hand, choroidal melanomas may also
present as a typical choroidal hemorrhagic detachment. Reese 30 reported a case that was thought to be a choroidal hemorrhage initially but subsequently was found to be a choroidal melanoma. He stated that it was possible that the choroidal hemorrhage had existed and masked the melanoma at the initial examination. Shields et al31 have also described a case where, in what appeared to be a typical postoperative choroidal detachment, a multilobed uveal melanoma was harbored, which was not appreciated preoperatively. Although large, multilobular, peripheral, serous choroidal detachments occurring in the immediate postoperative period are less likely to be confused with a choroidal melanoma, a hemorrhagic choroidal detachment may closely mimic a choroidal melanoma. This is especially true when the clinical findings are similar to the ones found in our patients. These patients had a limited hemorrhagic choroidal detachment which presented as a dark brown or reddish brown mass that measured 2.6 to 7.5 mm in height. Despite this considerable elevation, the lesions were discrete and well localized. Furthermore, all these limited choroidal hemorrhages were located posterior to the equator without ciliary body or peripheral choroidal involvement. Fluorescein angiography in our patients did not show any late leakage or staining, which is commonly associated with choroidal melanomas, although longstanding choroidal detachments may occasionally show leakage through defects in the retinal pigment epithelium. A serous choroidal detachment characteristically transmits light on transillumination, whereas a pigmented melanoma does not; however, transillumination of a hemorrhagic choroidal detachment will also cast a shadow. In addition, as in our patients, ultrasonography can demonstrate low to medium internal reflectivity in hemorrhagic choroidal detachments similar to that seen with a choroidal melanoma. 32 A careful history and a complete medical and ocular examination of both eyes should be performed in every case of a suspicious fundus lesion. However, the most important diagnostic test is continued observation of suspicious lesions. Serial observations over time will most likely establish the correct diagnosis.
REFERENCES 1. Shields JA, Augsburger JJ, Brown GC, Stephens RF. The differential diagnosis of posterior uveal melanoma. Ophthalmology 1980; 87:51822. 2. Shields JA, McDonald R. Improvements in the diagnosis of posterior uveal melanomas. Arch Ophthalmol 1974; 91 :259-64. 3. Fogle JA, Green WR. Ciliochoroidal effusion. In: Duane TO, Jaeger EA, eds. Clinical Ophthalmology. Philadelphia: Harper and Row, 1982; Volume 4, Chapter 63: 1-31.
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4. Brubaker RF, Pederson JE. Ciliochoroidal detachment. SUN Ophthalmol 1983; 27:281-9. 5. Schepens CL, Brockhurst RJ. Uveal effusion. 1. Clinical picture. Arch Ophthalmol1963; 70:189-201. 6. McGrand JC. Choroidal detachment in aphakic uveitis. Br J Ophthalmol 1969; 53:778-81. 7. Brubaker RF. Intraocular surgery and choroidal hemorrhage. Arch Ophthalmol 1984; 102: 1753-4. 8. Gressel MG, Parrish RK, Heuer OK. Delayed nonexpulsive suprachoroidal hemorrhage. Arch Ophthalmol1984; 102:1757-60. 9. Yanoff M. Fine BS. Surgical and nonsurgical trauma. Ocular Pathology, A Text and Atlas, 2nd ed. Vol. 138. Hagerstown: Harper & Row, 1982; 143-6. 10. Taylor OM. Expulsive hemorrhage. Am J Ophthalmol1974; 78:961-
6. 11. Hoffman P, Pollack A, Oliver M. Limited choroidal hemorrhage associated with intracapsular cataract extraction. Arch Ophthalmol1984; 102:1761-5. 12. Seelenfreund MH, Kraushar MF, Schepens CL, Freilich DB. Choroidal detachment associated with primary retinal detachment. Arch Ophthalmol1974; 91 :254-8. 13. Gottlieb F. Combined choroidal and retinal detachment. Arch Ophthalmol1972; 88:481-6. 14. Graham PA. Unusual evolution of retinal detachments. Trans Ophthalmol Soc UK 1958; 78:359-71. 15. Packer AJ, Maggiano JM, Aaberg TM, Meredith TA, Reeser FH, Kingham JD. Serous choroidal detachment after retinal detachment surgery. Arch Ophthalmol 1983; 101: 1221-4. 16. Aaberg TM. Experimental serous and hemorrhagic uveal edema associated with retinal detachment surgery. Invest Ophthalmol 1975; 14:243-6. 17. Ferry AP. Lesions mistaken for malignant melanoma of the posterior uvea. Arch Ophthalmol 1964; 72:463-9.
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18. Shields JA, Zimmerman LE. Lesions simulating malignant melanoma of the posterior uvea. Arch Ophthalmol 1973; 89:466-71. 19. Robertson OM, Campbell RJ. Errors in the diagnosis of malignant melanoma of the choroid. Am J Ophthalmol1979; 87:269-75. 20. Davidorf FH, Letson AD, Weiss ET, Levine E. Incidence of misdiagnosed and unsuspected choroidal melanomas: a 50-year experience. Arch Ophthalmol 1983; 101 :410-2. 21. Bard LA. Eyes with choroidal detachments removed for suspected melanoma. Arch Ophthalmol 1965; 73:320-3. 22. Zimmerman LE. Problems in the diagnosis of malignant melanomas of the choroid and Ciliary body. Am J Ophthalmol1973; 75:917-29. 23. Dunphy EB, Forrest AW, Leopold IH, Reese AB, Zimmerman LE. The diagnosis and management of intraocular melanomas. Trans Am Acad Ophthalmol Otolaryngol1958; 62:517-555. 24. Addison OJ, Wakelin DL. Errors in the diagnosis of choroidal malignant melanoma. Can J Ophthalmol1975; 10:205-6. 25. Chang M, Zimmerman LE, McLean I. The persisting pseudomelanoma problem. Arch Ophthalmol1984; 102:726-7. 26. Rones B, Zimmerman LE. An unusual choroidal hemorrhage simulating malignant melanoma. Arch OphthalmoI1963; 70:30-2. 27. Kirk HQ, Petty RW. Malignant melanoma of the choroid: a correlation of clinical and histological findings. Arch Ophthalmol 1956; 56:84360. 28. Reese AB. Pigmented tumors. Tumors of the Eye, 3rd ed. New York: Harper & Row, 1976; 216-22. 29. Hutchinson BT. Postoperative choroidal hemorrhage. In: Chandler, PA. Grant WM, eds. Glaucoma, 2nd ed. Philadelphia: Lea & Febiger, 1979; 316-7. 30. Reese AB. The differential diagnosis of malignant melanoma of the choroid. Arch Ophthalmol1957; 58:477-82. 31. Shields JA, Leonard BC, Sarin LK. Multilobed uveal melanoma masquerading as postoperative choroidal detachment. Br J Ophthalmol 1976; 60:386-9. 32. Shields JA. Current approaches to the diagnosis and management of choroidal melanomas. SUN OphthalmoI1977; 21:443-63.