HEMANGIOMA OF THE CHOROID* BY Angus L. MacLean, MD, AND A. Edzward Mfaaumteeee, MD

24 Pages·2005·3.53 MB·English

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.