HEMANGIOMA OF THE CHOROID* BY Angus L. MacLean, M.D., AND A. Edzward Mfaaumteeee, M.D. HEMANGIOMA of the choroid is a comparatively rare tumor, but one which probably occurs more often than reports in the literature would indicate. In about 50 percent of the cases reported, there has been a nevus flammeus on the ipsilateral side of the face. The choroidal tumor usually arises in the posterior portion ofthe fundus, frequently between the macula area and the disc. It is usually somewhat oval in shape. The area of choroidal involvement may be quite extensive, but in eyes with useful vision, the tumor mass has usually been limited to an area of six disc diameters or less. The color of the lesion has been variously described as grayish blue, grayish green, or pink. Sector-shaped field defects when present have aided in the diagnosis. The rate of growth of the tumor is usually slow, but if unchecked results in extensive retinal detachment from the accumulation of subretinal fluid. This is often followed by intractable secondary glaucoma. The first recognized case of hemangioma of the choroid to be reported was that of Panas and Remey in 1879 (1). Leber had pre- viously reported a case in 1869 (2), but had classified it as a spindle cell sarcoma of cavernous structure. In recent years several excel- lent reviews of the literatture have been published in conjunction with case reports (3, 4 and 5). No attempt has been made to check each case, but it appears that more than 80 cases have been described. In the majority of instances, the diagnosis has been a retrospective one, establishedonlyonhistologicexamination. The eyes lhave been removed because of suspected melanoma, or because they were blind and painful from extensive retinal detachment and intractable secondary glaucoma. In spite of the fact that the diagnosis of hemangioma of the choroid has usually been made after enucleation, there have been excellent descriptions of the clinical appearance and the course of the lesion (6, 7, and 8.) *From the Wilmer Ophthalmological Institute of the Johns Hopkins University and Hospital, Baltimore, Md. TR. AM. OPHTH. SOC., vol. 57, 1959 172 Angus L. MlacLeant and A. Edward Maumnenee There are two schools of thought as to the management of heman- gioma of the choroid. One has suggested that the lesion is so rare and so closely resembles a melanoma, that unless nevus flammeus of the same side of the face is present, the eye should be enucleated. Others have advocated eradication of the tumor mass in an attempt to save the eye. The only instance in which the latter therapy has been successfully performed was in a case reported by Schepens and Schwartz. This patient had a bilateral hemangioma of the choroid with extensive retinal detachments. In one eye the tumor was biopsied and hiistologic evidence of a hemangioma 'was obtained. All vision in this eye was eventually lost. In the remaining eye, the tumor was obliterated by transscleral diathermy and the patient regained 20/200 vision, and retained this for atleast three years afterthe operation. It is the purpose of this report to present the clinical picture and therapeutic outcome of eight cases of choroidal hemangioma. In all but one of these cases (Case 3) a clinical examination was done by one of us. Most of the patients had been referred for consultation with a diagnosis of suspected melanoma. In onlv one instance was a small facial hemangioma present. CASEREPORTS (CASE 1 The patient, a 40-year-old white man, hadhad yearly visual examina- tions. In November, 1948, his visual acuity was found to be 20/20 in each eye. On June 30, 1949, he noticed a blurring of vision in the right eye during pistol target practice. He was seen in consultation in July, 1949, at the Stanford University Hospital, and at that time was found to have a corrected visual acuity of 20/50 in the right eye and 20/15 in the left eye. There was a pie-shaped visual field defect which began at the blind spot and ex- tended upward temporally in the right eye. Just below the disc there was a lesion of approximately 3 or 4 disc diameters in size that was elevated 3 to 4 diopters. The tumor was pink in color and transillumi- nation of the mass was lighter than the remainder of the fundus. With the Friedenwald slit ophthalmoscope, no duplication of the beam could be seen. The surrounding retina was not detached. Provisional (liagnosis indicated a nonpigmented melanoma, neurofibroma of the choroid, or lhemangioma. The left eye was normal. Because of the possibility of a nonpigmented melanoma, enucleation was advised, Heinangiomtia of the Choroid 17f3 Histologic examination of the eye revealed a typical hemangioma of the choroid with cystic degeneration of the retina overlying the tumor mass in all layers, including the nerve fiber layer (Figure 1). FIGURE 1. CASE 1 MIicroscopic section of the posterior segment of the right eye, showing a typical cavernous hemangioma of the choroid in close proximity to the disc and slight separation of the retina. CASE 2 The patient, a 30-year-old white man, was first seen in consultation on February 16, 1955, at the Stanford University Hospital. He stated that three years prior to that time he had noted spots and flashes of light in front ofhis left eye. He had seen an optometrist who prescribed glasses for him. Four weeks prior to his examination, the visual acuity in his left eye had suddenly decreased. On examination the vision in the right eye could be corrected to 20/20 and in the left eye to 20/400; refractive error RE -0.50 sph., LE emmetropic. Visual field examination revealed a sector-shaped defect in the nasal field of the left eye. The right eye was normal. Ophthalmoscopic examination of the left eye revealed a honeycomb pinkmass located just above the macula area that was 3 disc diameters 174 Angus L. MacLean acnd A. Edwarsd Maumence wide and 5 to 6 disc diameters long, elevated 2 to 3 diopters (Figurc 2). There was a slight accumulation of pigment along the margin of the tumor mass. The retina was slightly detached over the entire mass and the detachment extended down into the macula. There were a few strias in this area. N,-,--,-,X..X~~~~~~~.... ..._: .. .............. X. -.... FIGURE 2. CASE 2 Fundus photograph of the left eye, showing a pink- colored hemangioma of the choroid in close prox- imity to the upper pole of the disc (Feb. 10, 1955). On examination with the slit lamp and contact lens, the retina ap- peared to be two to three times normal thickness and the lesion appeared more red and suggestive of blood vessels rather than a solid pigmented mass. No specks of pignment were noted on the surface of the lesion. A cobalt blue filter was placed in front of the beam of the Haag- Streit slit lamp and the patient given 2 c.c. of 5 percent fluorescein intravenously. A dozen small spots in the central portion of the tumor fluoresced within 30 sec. A diagnosis of hemangioma of the chioroid was made. On March 10, 1955, the tumor was coagulated with a 134 and 2 mm. electrode by means of transscleral diathermy. Following this two radon seeds each containing 2.5 mc. of radon were sutured to the sclera over the tumor mass. It was estimated that 3,000 roentgen equivalents wouldbe applied tothe tumor3mm. from theseseeds. Immediately following operation some hemorrhage was noted in the deeper part of the tissue and the patient's visual acuity fell to counting Hemangiomta of the Choroid 175 fingers at 2 feet. By May 24, 1955, the diathermy reaction had beguln to subside and the visual acuity improvedto20/300 (Figures 3 and 4). By August, 1956, thevisualacuityhadimprovedto 20/30 and has remained at that level until the time ofpublication ofthis report. FIGURE 3. CASE 2 Fundtis photograph of the left eye, showing results three months after treatment of the ttimor by trans- scleral irradiation and penetrating diathermy (May 10, 1955). FIGURE4. CASE2 Ftindus photograph of the left eye, showiiig the appearance one year after treatment of the tuimor bv transscleral irradiation and penetrating diathermy (Aug. 23, 1955). 176 Angus L. MacLean and A. Edward Maumitentee CASE3 This patient, a 34-year-old white woman, was not examined clinic- ally by either of the authors, but the histologic specimen was examined by one of us at Stanford University Hospital. The history is so typical that it is being included in this report. The patient was first seen in 1950 for routine examination. At that time she gave a history of lowered visual acuity for an indefinite period in the left eye. Her corrected vision in the right eye was 20/20 and in the left eye 20/60. The patient's vision continued to fail and in February she was seen by Dr. Arthur Kahler. At that time her visual acuity in the left eye was limited to hand motions. There was an exten- sive bullous detachment of the entire inferior retina. In the macula region there was a solid mass which measured 4 disc diameters hori- zontally and 3 vertically and was elevated 3 to 4 diopters. The lesion had a pink tinge as compared to the surrounding retina. On examina- tion with the slit lamp it appeared solid and contained a large number of blood vessels. There was no evidence of pigment on the surface ofthe mnass. The right eye was normal. The left eye was enucleated because of a suspected melanoma. On ...................|~~~~~~~~~~~~~~~~~~~~~....... FIGURE 5. CASE 3 Microscopic section of the posterior segment of the right eye, showing a typical cavernous hemangioma of the choroid with the retina attached at the upper border but detached, with high elevation, from that point to the ora below; also showing cystoid de- generation oftheretina. 'This case is presented by thc kind permission of Dr. Arthur Kaliler of Saera- mento, Calif. Hemangiorna of the Choroid 177 hiistologic examination there was a typical hemangioma of the choroid in the macula area. The inferior retina was detached by a serous coagulum. The retina overlying the tumor showed extensive cystic degeneration of all layers including the nerve fiber layer. The pigment epithelium over the tumor was thin, distorted, and some of it was missing. There was no evidence of newformed fibrous tissue between the retina and choroid (Figure 5). CASE4 This 36-year-old white male patient, was the first in this series to be seen at the Johns Hopkins Hospital. Vision of the right eye had been blurredfor several months, and there was a relative central scotoma for colors and a sector defect in the lower field extending from close to the point of fixation right to the periphery (Figure 6). The first examina- tion disclosed an oblong lesion in the upper part of the area between the macula and the disc. Under direct illumination this lesion had a greenish yellow spongy appearance, but with retroillumination it appeared streaked or honeycombed with darker lines in the lighter background. There was no obstruction to light on transillumination. It VISUAL FIEWS CASE 4 L E. 5/330 R. E. VOS 20/15 VOD 20/50 FIGURE 6. CASE 4 Visuacl fields, showing the characteristic sector-shaped defect in the right eye. Areas of ab.solute blindlness mixed with areas of relative blindness (a), and a relative central scotoma (b). 178 Angul.s L. MAacLact aiu(l A. Edward AMaulmenc FIGURE 7. CASE4 A latrge elevate(d orange-pink tumnlor, above and temporal to the disc, with slight separation of the retina at the lower dependent portion (Sept., 1955). FIGURE 8. CASE4 Fundus photograph, showing results six months after treatment of the tumor by transscleral irradiation and penetrating diathermy. A large area of scarring is seen at the upper and temporal side (Feb. 7, 1958). was generally agreed that this was not an inflammatory lesion. The eye was not enucleated, and the patient was kept under observation. The left eye was normal. In the course of a year, the lesion (Figure 7) increased in size, be- came more elevated, and the color changed to a uniform grayish pink or orange-pink. There was some separation of the retina, particuilarlv HIemtanigiotita of thte Chloroid 179 ... ...... FIGURE 9. CASE4 Funduts photograph showing the ischelnic reaction one week following treatment of the tulmor by light coagulation (Oct. 31, 1958). FIGURE 10. CASE 4 Fuindtis photograph showing the appearance three months after treatment of the tumor by light coagu- lation. The entire area of the tumor is replaced by scartissue. (Jan., 1959). at the lower dependent portion, giving the appearance of an over- hanginglowerborderabovethemaculaandthedisc. In September, 1955, a diagnosis of hemangioma of the choroid was made. The tumor was coagulated by transscleral diathermy, and a /2mc. radon seed was sutured to the episclera in this area. Figure 8 is a photograph of this fundus six months later. It shows not only the scars from the coagulation, but also evidence of a residual tumor. 180 Angus L. MacLean and A. Edward Maumenee Diathermy treatment was repeated one year later. This was also un- successful. In six months' time the extension had reached beyond the disc to the nasal side. The patient was seen by Professor Meyer-Schwickerath who con- curred in the diagnosis. Although there was some subretinal fluid, he believed that the degree ofretinal separation was still within the limits of safety and that light coagulation could be given. Since the macula was alreadyinvolved,treatmentwas given only with the idea of saving the eye. Accordingly, sparing the macula as much as possible, the entire growth was given about 25 exposures of light coagulation of moderate intensity. This treatment was followed by marked ischemic reaction whichlasted for a few weeks (Figure 9). In the course of the nextfive months, the entire growth was replacedby scartissue (Figure FRONT OUTWARD 3i/333 FIGURE 11.CAE5 Visualfielddefect.