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Elschnig's Spots and Hypertensive Choroidopathy
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6. King, G. W . : Recording pupil changes for clinical diagnosis. Electronics 32:67, 1959. 7. Stark, L. : Environmental clamping of biological systems : Pupil servomechanism. J. Optical Soc. Am. 52:925, 1962. 8. Ogle, K. N., Whisnant, R. A . and Hazelrig, J. B. : Quantitative study of pupil response to miotic drugs. Invest. Ophth. 5:176, 1966. 9. Gambill, H . D., Ogle, K. N. and Kearns, T. P. : Mydriatic effect of four drugs determined with pupillograph. Arch. Ophth. 77:740, 1967. 10. Lowenstein, O. and Loewenfeld, I. E. : Effect of physostigmine and pilocarpine on iris sphincter
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of normal man: Is it competitive or additive? Pupillographic studies. Arch. Ophth. 50:311, 1953. 11. Lowenstein, O. and Loewenfeld, I. E : Influence of retinal adaptation upon the pupillary reflex to light in normal man: I. Effect of adaptation to bright light on the pupillary threshold. Am. J. Ophth. 48 ( p t 2 ) :536, 1959. 12. Morgan, S. S. : The Effects of Certain Autonomic Drugs on the Speed of the Pupillary Response To Light Thesis, Mayo Graduate School of Medicine (University of Minnesota), Rochester, 1967.
SPOTS A N D HYPERTENSIVE
CHOROIDOPATHY
PETER H . MORSE, M . D . Baltimore, Maryland
Hypertensive choroidopathy, often called albuminuric choroiditis, is frequently obscured by concomitant hypertensive retinopathy and retinal edema. In malignant hypertension four types of fundus lesions associated with choroidal vascular changes are recognized clinically. Pale yellow or reddish patches bordered to a varying extent by pigment deposits: black isolated spots of pigment with a surrounding yellow or red halo (Elschnig's spots) ; chains of pigment flecks arranged linearly along the course of a yellow-white sclerosed choroidal vessel (Siegrist's spots) ; and yellow or red patches of chorioretinal atrophy. 1
These ophthalmoscopically visible manifestations of hypertensive choroidopathy are seen pathologically as necrosis and thinning of the pigment epithelium in some places, with proliferation and clumping in others, often overlying irregularly distributed sclerotic to obliterative changes in the choriocapillaris. Ischemic infarcts of the choriocapillaris are seen in the acute stage. These and other previously observed clinicopathologic findings are described elsewhere. " 2
8
From the Wilmer Ophthalmological Institute, Johns Hopkins Hospital and School of Medicine, Baltimore, Maryland, 21205.
The purpose of this paper is to report a case demonstrating uncommonly seen Elschnig's spots, as well as fluorescein angiograms and illustrative histopathologic findings. CASE REPORT A 35-year-old Negro woman was first seen at the Wilmer Institute in January 26, 1966, complaining of headaches, blurred vision and flickering spots in both eyes of four to five weeks' duration. Her corrected visual acuity in the right eye was 20/50 + 3 and in the left eye 20/200. Intraocular pressure was 7/5.5 or 12.2 mm H g (Schiotz) in each eye. Slitlamp examination revealed normal anterior segments. Ophthalmoscopic examination revealed clear media bilaterally with papilledema, narrowed arterioles, A - V compression, flame-shaped retinal hemorrhages, hard yellow exudates and macular star figures, more pronounced in the left eye. Her blood pressure was 270/150 mm Hg. She was admitted the same day to the Osier Medicine Service. Her past history was one of good health. In addition to her headaches and ocular complaints she had experienced polydypsia, polyuria, nocturia, and a 10-pound weight loss over the previous four to five weeks. Physical examination showed the patient to be afebrile with a blood pressure of 220/110 mm Hg. The heart had a systolic murmur with an S-4 gallop. A chest X-ray revealed the lungs to be clear but the heart showed signs of left ventricular hypertrophy. Urinalysis gave a 4-plus reaction for protein and had 10 to 12 red cells per high-powered field and a few clumps of white cells. The hematocrit was 28%, white blood cell count, 8,000, and the peripheral smear showed microangiopathic hemolytic anemia. Platelets were 132,000 and reticulocytes 1.4%. A sero-
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logic test for syphilis was nonreactive, B U N ranged from 18 mg/100cc to a high of 32 mg/100cc. Creatinine was 2.9 mg/100cc with creatinine clearance of 28 cc per minute. Two-hour postprandial glucose was 180 mg/100 cc. Twenty-fourhour urine samples contained 37 (ig of catecholamines and 2 mg of vanilmandelic acid. The patient was treated with guanethidine, reserpine, digoxin and a 4-gm salt diet with a resultant drop in the blood pressure to the level of 150/100 mm H g recumbent and 130/90 mm H g standing. The patient remained under good control until September 1966 when, after a week without medication, she experienced anorexia, nausea, emesis, headache and abdominal pain for five days. She was discovered to be unresponsive at home and was admitted to the hospital. The blood pressure was 240/170 mm H g and the results of physical examination were essentially unchanged from her previous admission. The patient still had severe hypertensive retinopathy (Grade I V ) . The hypertension was controlled with Arfonad, reserpine, and guanethidine. The B U N fell from an initial 50 mg/100 cc to 29 mg/100 cc and the creatinine from 3.7 to 2.7 mg/100 cc. Renal scan showed a decreased uptake with a smaller impression of the left kidney. A t the time of discharge the blood pressure was 212/110 mm H g recumbent and 90/65 mm H g standing, with Guanethidine treatment solely. On December 9, 1966, she was again admitted with a blood pressure of 240/105 mm Hg. Her corrected visual acuity was 20/20 and J 2 in each eye. She denied any ocular symptoms. Slitlamp examination showed normal anterior segments. Intraocular pressure was 6/5.5 or 14.6 mm H g in the right eye and 5/5.5 or 17.3 mm H g (Schiotz; in the left
Fig. 2 (Morse). Case 1. Right eye Arteriolar phase after fluorescein injection. Note the early appearance of fluorescence in the relatively depigmented halo surrounding the central pigment clump. Note also the blotchy appearance of the choroid and retinal vessels. Some drusen are beginning to fluoresce.
Fig. 1 (Morse). Case 1. Fundus photograph of the right eye showing characteristic Elschnig's spots with central pigment clumping surrounded by a lighter halo. Note other areas of relative depigmentation inferiorly.
eye. The visual fields were entirely full centrally and peripherally, with slight enlargement of the blind spots. On ophthalmoscopic examination the media were clear bilaterally. The fundus of the right eye showed early neovascularization of the disc, macular sheen, and some small, hard, yellow exudates. There was marked arteriolar narrowing with some A - V compression. In the midperiphery there were patches of pale yellow-orange color where the pigment was sparse and finely granular in appearance. Many of these areas had a dark central clump of pigment (fig. 1 ) . The left fundus showed a virtually identical picture. Intravenous fluorescein angiograms of the fundi were obtained (figs. 2 to 6 ) . The choroidal flush had a diffuse, irregular, blotchy appearance not confined to the clinically observable pigment alteration. This irregularity and uneven pattern was seen in the small retinal vessels, as well as capillary dilatation often associated with areas of capillary obliteration. N o evidence of vascular leakage was observed but the filling time of the vessels was somewhat delayed. This finding is consistent with the arteriolar narrowing and vascular compromise. Many small drusen, scarcely noticeable ophthalmoscopically, were highlighted by fluorescence. In the patches of pigment disturbance the
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Fig. 3 (Morse). Case 1, Right eye. Early venous phase showing further accentuation of the halo around the pigment spot and the irregularity of the retinal vessel filling pattern. Many more drusen are visible.
Fig. 4 (Morse). Case 1, Right eye. Late venous phase showing more fluorescence in areas of pigment disturbance but no increase in size.
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Fig. 5 (Morse). Case 1, Right eye. Photograph taken 5 minutes after fluorescein injection still demonstrating pigment defects without enlargement or leakage of the fluorescent areas.
Fig. 6 (Morse). Case 1. Right eye. Photograph taken 12 minutes after fluorescein injection showing constant size of fluorescent areas and decrease in intensity.
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Fig. 7 (Morse). Case 1. Kidney with vascular changes consisting of intimai proliferation, onion skinning, slight hyaline sclerosis and partial destruction of the muscular arterial wall. Note also focal tubular necrosis and glomerular hyalinization and sclerosis. (Hematoxylineosin, X130.)
yellow-orange halos fluoresced brightly, as one would expect, because the background choroidal fluorescence became visible in the areas of pigment rarefaction. The patient's hypertension was again partially controlled with Guanethidine. On April 30, 1967, the patient was brought to the Accident Room with a history of seizures followed by unconsciousness. She was unresponsive and apneic, with a weak pulse. The pupils were dilated and fixed with the fundi showing severe hypertensive retinopathy and subhyaloid hemorrhage. Attempted resuscitation failed, and the patient was pronounced dead. Permission for autopsy was obtained.
PATHOLOGIC FINDINGS At autopsy berry aneurysms of the basilar
and right posterior cerebral arteries were seen along with massive subarachnoid hemorrhage. There was generalized severe arteriosclerosis and arteriolosclerosis. The kidneys were markedly atrophic with large wedges of tubular atrophy and extensive intrarenal arterio- and arteriolosclerosis of the type seen in idiopathic malignant hypertension. In some arteries there was partial destruction of the muscular wall (fig. 7 ) . Striking vascular lesions were also noted in the pancreas consisting of marked destruction of the muscular wall of small and medi-
Fig. 8 (Morse). Case 1. Pancreas. Artery with virtually complete destruction of the muscular wall and marked intimai proliferation appearing as a recanalized and endothelialized thrombus. Groups of eosinophiles were seen in the arterial wall. (Hematoxylin-eosin, X27S.)
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Fig. 9 (Morse). Case 1. Choroid demonstrating intimai proliferation and hyalinization virtually obliterating arterial and arteriolar lumina. Partial destruction of the muscular wall is seen in some arteries. There is mild lymphocytic infiltration. (Hematoxylin-eosin, X l 3 0 . )
um-sized arteries. In one artery the wall and lumen were replaced by intricately re-canalized thrombi (fig. 8 ) . The arterial wall contained a number of eosinophils. No definite decision could be made as to whether or not the patient had malignant essential hypertension with severe secondary vascular changes or a peculiar primary arteritis with secondary hypertension. The possibility of pyelonephritis lenta was also considered. In the eye the choroidal changes were striking. The retina had become artifactitiously detached. The arteries showed medial
Fig. 10 (Morse). Case 1. Choroid. The pigment epithelium shows an uneven distribution of pigment granules. Drusen are prominent The choriocapillaris is focally obliterated. (Hematoxylin-eosin, X130.)
thickening, some with partial destruction of the muscular wall. Subendothelial hyalinization of the arteries and arterioles with marked intimai proliferation was widespread (fig. 9 ) . Many drusen were present (fig. 10). The pigment epithelium showed foci of clumping and proliferation in some areas with relative paucity of pigment granules in others. The Elschnig spot seen clinically (fig. 1) appeared as a clump of pigment and the surrounding pigment epithelium cells, which constitute the yellow-orange halo, had a decreased amount of pigment (fig. 12).
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Fig. 11 (Morse). Case 1. Choroid. Pathologic section conforming to the clinical appearance of an Elschnig spot (Cf. Fig. 1). There is clumping of the pigment with a relative paucity of pigment granules in the bordering epithelium on either side. The choriocapillaris is not uniformly well defined. (Hematoxylin-eosin, x400.)
Fig. 12 (Morse). Case 1. Choroid demonstrating an area of relative depigmentation of the pigment epithelium. Note retention of nuclei with sparsity of pigment granules. (Hematoxylin-eosin, X400.)
Fig. 13 (Morse). Case 1. Choroid showing focal obliteration of the choriocapillaris with overlying degeneration of the pigment epithelium. (Hematoxylin-eosin, X410.)
The choriocapillaris showed foci of fibrosis, obliteration, and residual ghost capillary outlines (figs. 11 to 13). DISCUSSION Histologic choroidal vascular changes are
seen in both benign and malignant hypertension. This choroidopathy may become clinically manifest in cases of malignant hypertension, either essential, associated with chronic glomerulonephritis, or systemic disease such as toxemia of pregnancy, scler-
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Fig. 14 (Morse). Case 2. Section showing thrombosis and infarction of the choriocapillaris with necrosis of the overlying pigment epithelium. Some serous subretinal fluid is seen as well as medial thickening of the arteries. (Hematoxylineosin, X l 6 5 . ) Courtesy of Dr. L. Zimmerman.
oderma, or other collagen disease. The early clinical changes are difficult to observe and may often be obscured by coexistent retinopathy and edema. Ultimately the underlying pathology may become ophthalmoscopically evident only as pigment alterations in the fundi. Despite the plethora of blood vessels the choroid is not invulnerable to significant vascular compromise. The vascular changes in the choroid and retina are emphasized by the fluorescein angiograms which show a blotchy and irregular filling pattern. There is also a
Fig. 15 (Morse). Case 2. Highpower view showing thrombotic obliteration of the choriocapillaris with necrosis of the pigment epithelium. The arterioles show endothelial proliferation to the point of obliteration. (Hematoxylin-eosin, X410.) Courtesy of Dr. L. Zimmerman.
slight delay in the vessel filling time which suggests a generally compromised vascular network. No evidence of vascular discontinuity or leakage was seen angiographically in the reported case which showed healed lesions. The fluorescein also highlights the multiple small drusen seen in this young patient. As would be anticipated the clumps of darker pigment, seen centrally in the Elschnig spot, did not fluoresce. However, the surrounding pale halo with sparse pigmentation (fig. 11) and the other pale areas with diffuse granular pigmentation (fig. 12) flu-
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Fig. 16 (Morse). Case 2. Highpower view showing area of thrombosed choriocapillaris (left) adjacent to a relatively uninvolved portion. Serous separation of the retina is seen. (Hematoxylin-eosin, x410.) Courtesy of Dr. L. Zimmerman.
oresced intensely because the large volume background or underlying choroidal fluorescence had an overlying deficiency of pigment which normally blocks the transmission of this fluorescence. The vascular pathology, which is progressive, shows two main phases, acute and healed. Whereas the reported case shows the healed lesions with secondary pigmentary changes, another case obtained from an autopsy eye shows the more acute phase of hypertensive choroidopathy. The patient was a 52-year-old white woman with hypertensive-cardiovascular-renal disease. She had known hypertension for five years prior to her death with a blood pressure averaging 200/140 to 200/120 mm Hg during the final year of her life. She died of congestive heart failure with uremia. Pathologic sections of her eye showed focal thrombosis of the choriocapillaris, ischemic infarcts, necrosis of the overlying pigment epithelium, and serous retinal separation (figs. 14 to 16). The pathologic findings in these cases are similar to others previously reported. However, other authors have emphasized the acute phase with necrosis of the pigment epithelium secondary to thrombosis of the choriocapillaris. This report shows the pathologic findings in the healed or late phase with
clinically manifest Elschnig's spots. Fluorescein angiograms of the fundi in cases of malignant hypertension with Elschnig's spots have not previously been reported. Clinical manifestations of malignant hypertension have not received much attention in the American ophthalmic literature and yet may be of great magnitude in the understanding of the ocular status in this disease process. SUMMARY A case of malignant hypertension with severe choroidopathy and Elschnig's spots is reported along with fluorescein angiograms of the healed lesions. Hypertensive choroidal pathology which may become clinically evident solely as pigmentary change is discussed. Wilmer Institute (21205) ACKNOWLEDGMENTS I wish to thank Dr. Bertha Klien for her guidance in this study, Mrs. Jane Ponder for preparation of the pathologic sections, Messers. Terry George and Raymond Lund for their photography, and Dr. Lorenz Zimmerman for his history and slide.
REFERENCES 1. Duke-Elder, W . S. : System of Ophthalmology. St. Louis, Mosby, vol. 9, 1966, p. 629. 2. Elschnig, A . : Die diagnostische und prognostische Bedeutung der Netzhauterkrankung bei
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Nephritis. Wien med Wschr. 54:494, 1904. 3. Fuchs, A . : Ueber Choroiditis albuminurica. Klin. Mbl. Augenh. 84:39, 1930. 4. Koyanagi, Y . : Úeber die Pathogenese der Retinitis nephritka. Klin. Mbl. Augenh. 80:436, 1928. 5. Klien, B. A . : Die Blutgefässe der Netzhaut und der Aderhaut bei allgemeiner Sklerodermie, Verhandl. Oesterr. Ophth. Ges. 6:31, 1961. 6. Klien, B. A . : Ischemic infarcts of the choroid
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(Elschnig's spots). A cause of retinal separation in hypertensive disease with renal insufficiency. Am. J. Ophth. (In press). 7. Müller, H . : Das histologische Bild der Chorioiditis allergica bei allergischer Nephritis. Arch, f. Ophth. 150:58,1950. 8. Friedman, E., Smith, T. R., Kuwabara, T. and Beyer, C. K : Choroidal vascular patterns in hypertension. Arch. Ophth. 71:842, 1964.
CHORIORETINITIS
SCLOPETARIA
R . D . RICHARDS, M . D . , C . E . WEST, M . D . AND A . A . MEISELS, M . D . Baltimore, Maryland Chorioretinitis sclopetaria is a term introduced into the German literature to describe an entity of direct choroidal and retinal trauma from a bullet wound in the orbital area. The bullet does not penetrate the globe, but either passes through the orbit between the globe and orbital wall, or lodges adjacent to the globe. The impact of the missile in the tissues adjacent to the wall of the globe causes a concussion-type injury to the globe, characterized as nonpenetrating but causing rupture of the choroid and retina with hemorrhages. Two areas of injury are usually present: the area directly adjacent to the path of the bullet, with damage from direct trauma ; and the macular area, with damage from indirect trauma. Frequently the trauma is so severe that only one large lesion involving both areas is present. Repair produces a white proliferative tissue scar with associated pigmentary disturbance. 1
CASE REPORTS CASE 1 A 50-year-old man was admitted to the University of Maryland Hospital on February 17, 1968, with gunshot wounds of the head. One wound was present in the mid-forehead and another at the infraorbital rim of the right eye (fig. 1 ) . The patient was conscious and responding but unable to give a history. From the Department of Ophthalmology, University of Maryland Medical School. Presented at the 104th Annual Meeting of the American Ophthalmological Society, Hot Springs, Virginia, May 27-29, 1968.
Fig. 1 (Richards, West and Meisels). Bullet wound, lower rim of orbit. Subconjunctival hemorrhage is also visible (Case 1 ) . Skull X-rays showed metallic fragments from a shattered bullet in the right orbit (fig. 2 ) , and additional fragments in the region of the temporal lobe and sylvian fissure within the cranial cavity. The right eyelids were swollen, and a large subconjunctival hemorrhage was present. The cornea was normal and a sluggish but reactive pupil was noted. Vision was recognition of hand movements at two feet. Ophthalmoscopic examination showed a clear vitreous. The entire lower portion of the retina was filled with retinal and choroidal hemorrhages. The area involved the inferior half of the globe, and extended to the macular area and disc (figs. 3 and 4 ) . The left eye was entirely normal, and the rest of the physical examination was normal. The patient's clinical course was uneventful, and no additional changes were noted in the retina. H e was discharged 10 days later, with ocular damage the only apparent residual from the trauma. N o neurologic abnormalities were found.
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6. King, G. W . : Recording pupil changes for clinical diagnosis. Electronics 32:67, 1959. 7. Stark, L. : Environmental clamping of biological systems : Pupil servomechanism. J. Optical Soc. Am. 52:925, 1962. 8. Ogle, K. N., Whisnant, R. A . and Hazelrig, J. B. : Quantitative study of pupil response to miotic drugs. Invest. Ophth. 5:176, 1966. 9. Gambill, H . D., Ogle, K. N. and Kearns, T. P. : Mydriatic effect of four drugs determined with pupillograph. Arch. Ophth. 77:740, 1967. 10. Lowenstein, O. and Loewenfeld, I. E. : Effect of physostigmine and pilocarpine on iris sphincter
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of normal man: Is it competitive or additive? Pupillographic studies. Arch. Ophth. 50:311, 1953. 11. Lowenstein, O. and Loewenfeld, I. E : Influence of retinal adaptation upon the pupillary reflex to light in normal man: I. Effect of adaptation to bright light on the pupillary threshold. Am. J. Ophth. 48 ( p t 2 ) :536, 1959. 12. Morgan, S. S. : The Effects of Certain Autonomic Drugs on the Speed of the Pupillary Response To Light Thesis, Mayo Graduate School of Medicine (University of Minnesota), Rochester, 1967.
SPOTS A N D HYPERTENSIVE
CHOROIDOPATHY
PETER H . MORSE, M . D . Baltimore, Maryland
Hypertensive choroidopathy, often called albuminuric choroiditis, is frequently obscured by concomitant hypertensive retinopathy and retinal edema. In malignant hypertension four types of fundus lesions associated with choroidal vascular changes are recognized clinically. Pale yellow or reddish patches bordered to a varying extent by pigment deposits: black isolated spots of pigment with a surrounding yellow or red halo (Elschnig's spots) ; chains of pigment flecks arranged linearly along the course of a yellow-white sclerosed choroidal vessel (Siegrist's spots) ; and yellow or red patches of chorioretinal atrophy. 1
These ophthalmoscopically visible manifestations of hypertensive choroidopathy are seen pathologically as necrosis and thinning of the pigment epithelium in some places, with proliferation and clumping in others, often overlying irregularly distributed sclerotic to obliterative changes in the choriocapillaris. Ischemic infarcts of the choriocapillaris are seen in the acute stage. These and other previously observed clinicopathologic findings are described elsewhere. " 2
8
From the Wilmer Ophthalmological Institute, Johns Hopkins Hospital and School of Medicine, Baltimore, Maryland, 21205.
The purpose of this paper is to report a case demonstrating uncommonly seen Elschnig's spots, as well as fluorescein angiograms and illustrative histopathologic findings. CASE REPORT A 35-year-old Negro woman was first seen at the Wilmer Institute in January 26, 1966, complaining of headaches, blurred vision and flickering spots in both eyes of four to five weeks' duration. Her corrected visual acuity in the right eye was 20/50 + 3 and in the left eye 20/200. Intraocular pressure was 7/5.5 or 12.2 mm H g (Schiotz) in each eye. Slitlamp examination revealed normal anterior segments. Ophthalmoscopic examination revealed clear media bilaterally with papilledema, narrowed arterioles, A - V compression, flame-shaped retinal hemorrhages, hard yellow exudates and macular star figures, more pronounced in the left eye. Her blood pressure was 270/150 mm Hg. She was admitted the same day to the Osier Medicine Service. Her past history was one of good health. In addition to her headaches and ocular complaints she had experienced polydypsia, polyuria, nocturia, and a 10-pound weight loss over the previous four to five weeks. Physical examination showed the patient to be afebrile with a blood pressure of 220/110 mm Hg. The heart had a systolic murmur with an S-4 gallop. A chest X-ray revealed the lungs to be clear but the heart showed signs of left ventricular hypertrophy. Urinalysis gave a 4-plus reaction for protein and had 10 to 12 red cells per high-powered field and a few clumps of white cells. The hematocrit was 28%, white blood cell count, 8,000, and the peripheral smear showed microangiopathic hemolytic anemia. Platelets were 132,000 and reticulocytes 1.4%. A sero-
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logic test for syphilis was nonreactive, B U N ranged from 18 mg/100cc to a high of 32 mg/100cc. Creatinine was 2.9 mg/100cc with creatinine clearance of 28 cc per minute. Two-hour postprandial glucose was 180 mg/100 cc. Twenty-fourhour urine samples contained 37 (ig of catecholamines and 2 mg of vanilmandelic acid. The patient was treated with guanethidine, reserpine, digoxin and a 4-gm salt diet with a resultant drop in the blood pressure to the level of 150/100 mm H g recumbent and 130/90 mm H g standing. The patient remained under good control until September 1966 when, after a week without medication, she experienced anorexia, nausea, emesis, headache and abdominal pain for five days. She was discovered to be unresponsive at home and was admitted to the hospital. The blood pressure was 240/170 mm H g and the results of physical examination were essentially unchanged from her previous admission. The patient still had severe hypertensive retinopathy (Grade I V ) . The hypertension was controlled with Arfonad, reserpine, and guanethidine. The B U N fell from an initial 50 mg/100 cc to 29 mg/100 cc and the creatinine from 3.7 to 2.7 mg/100 cc. Renal scan showed a decreased uptake with a smaller impression of the left kidney. A t the time of discharge the blood pressure was 212/110 mm H g recumbent and 90/65 mm H g standing, with Guanethidine treatment solely. On December 9, 1966, she was again admitted with a blood pressure of 240/105 mm Hg. Her corrected visual acuity was 20/20 and J 2 in each eye. She denied any ocular symptoms. Slitlamp examination showed normal anterior segments. Intraocular pressure was 6/5.5 or 14.6 mm H g in the right eye and 5/5.5 or 17.3 mm H g (Schiotz; in the left
Fig. 2 (Morse). Case 1. Right eye Arteriolar phase after fluorescein injection. Note the early appearance of fluorescence in the relatively depigmented halo surrounding the central pigment clump. Note also the blotchy appearance of the choroid and retinal vessels. Some drusen are beginning to fluoresce.
Fig. 1 (Morse). Case 1. Fundus photograph of the right eye showing characteristic Elschnig's spots with central pigment clumping surrounded by a lighter halo. Note other areas of relative depigmentation inferiorly.
eye. The visual fields were entirely full centrally and peripherally, with slight enlargement of the blind spots. On ophthalmoscopic examination the media were clear bilaterally. The fundus of the right eye showed early neovascularization of the disc, macular sheen, and some small, hard, yellow exudates. There was marked arteriolar narrowing with some A - V compression. In the midperiphery there were patches of pale yellow-orange color where the pigment was sparse and finely granular in appearance. Many of these areas had a dark central clump of pigment (fig. 1 ) . The left fundus showed a virtually identical picture. Intravenous fluorescein angiograms of the fundi were obtained (figs. 2 to 6 ) . The choroidal flush had a diffuse, irregular, blotchy appearance not confined to the clinically observable pigment alteration. This irregularity and uneven pattern was seen in the small retinal vessels, as well as capillary dilatation often associated with areas of capillary obliteration. N o evidence of vascular leakage was observed but the filling time of the vessels was somewhat delayed. This finding is consistent with the arteriolar narrowing and vascular compromise. Many small drusen, scarcely noticeable ophthalmoscopically, were highlighted by fluorescence. In the patches of pigment disturbance the
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Fig. 3 (Morse). Case 1, Right eye. Early venous phase showing further accentuation of the halo around the pigment spot and the irregularity of the retinal vessel filling pattern. Many more drusen are visible.
Fig. 4 (Morse). Case 1, Right eye. Late venous phase showing more fluorescence in areas of pigment disturbance but no increase in size.
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Fig. 5 (Morse). Case 1, Right eye. Photograph taken 5 minutes after fluorescein injection still demonstrating pigment defects without enlargement or leakage of the fluorescent areas.
Fig. 6 (Morse). Case 1. Right eye. Photograph taken 12 minutes after fluorescein injection showing constant size of fluorescent areas and decrease in intensity.
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Fig. 7 (Morse). Case 1. Kidney with vascular changes consisting of intimai proliferation, onion skinning, slight hyaline sclerosis and partial destruction of the muscular arterial wall. Note also focal tubular necrosis and glomerular hyalinization and sclerosis. (Hematoxylineosin, X130.)
yellow-orange halos fluoresced brightly, as one would expect, because the background choroidal fluorescence became visible in the areas of pigment rarefaction. The patient's hypertension was again partially controlled with Guanethidine. On April 30, 1967, the patient was brought to the Accident Room with a history of seizures followed by unconsciousness. She was unresponsive and apneic, with a weak pulse. The pupils were dilated and fixed with the fundi showing severe hypertensive retinopathy and subhyaloid hemorrhage. Attempted resuscitation failed, and the patient was pronounced dead. Permission for autopsy was obtained.
PATHOLOGIC FINDINGS At autopsy berry aneurysms of the basilar
and right posterior cerebral arteries were seen along with massive subarachnoid hemorrhage. There was generalized severe arteriosclerosis and arteriolosclerosis. The kidneys were markedly atrophic with large wedges of tubular atrophy and extensive intrarenal arterio- and arteriolosclerosis of the type seen in idiopathic malignant hypertension. In some arteries there was partial destruction of the muscular wall (fig. 7 ) . Striking vascular lesions were also noted in the pancreas consisting of marked destruction of the muscular wall of small and medi-
Fig. 8 (Morse). Case 1. Pancreas. Artery with virtually complete destruction of the muscular wall and marked intimai proliferation appearing as a recanalized and endothelialized thrombus. Groups of eosinophiles were seen in the arterial wall. (Hematoxylin-eosin, X27S.)
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Fig. 9 (Morse). Case 1. Choroid demonstrating intimai proliferation and hyalinization virtually obliterating arterial and arteriolar lumina. Partial destruction of the muscular wall is seen in some arteries. There is mild lymphocytic infiltration. (Hematoxylin-eosin, X l 3 0 . )
um-sized arteries. In one artery the wall and lumen were replaced by intricately re-canalized thrombi (fig. 8 ) . The arterial wall contained a number of eosinophils. No definite decision could be made as to whether or not the patient had malignant essential hypertension with severe secondary vascular changes or a peculiar primary arteritis with secondary hypertension. The possibility of pyelonephritis lenta was also considered. In the eye the choroidal changes were striking. The retina had become artifactitiously detached. The arteries showed medial
Fig. 10 (Morse). Case 1. Choroid. The pigment epithelium shows an uneven distribution of pigment granules. Drusen are prominent The choriocapillaris is focally obliterated. (Hematoxylin-eosin, X130.)
thickening, some with partial destruction of the muscular wall. Subendothelial hyalinization of the arteries and arterioles with marked intimai proliferation was widespread (fig. 9 ) . Many drusen were present (fig. 10). The pigment epithelium showed foci of clumping and proliferation in some areas with relative paucity of pigment granules in others. The Elschnig spot seen clinically (fig. 1) appeared as a clump of pigment and the surrounding pigment epithelium cells, which constitute the yellow-orange halo, had a decreased amount of pigment (fig. 12).
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Fig. 11 (Morse). Case 1. Choroid. Pathologic section conforming to the clinical appearance of an Elschnig spot (Cf. Fig. 1). There is clumping of the pigment with a relative paucity of pigment granules in the bordering epithelium on either side. The choriocapillaris is not uniformly well defined. (Hematoxylin-eosin, x400.)
Fig. 12 (Morse). Case 1. Choroid demonstrating an area of relative depigmentation of the pigment epithelium. Note retention of nuclei with sparsity of pigment granules. (Hematoxylin-eosin, X400.)
Fig. 13 (Morse). Case 1. Choroid showing focal obliteration of the choriocapillaris with overlying degeneration of the pigment epithelium. (Hematoxylin-eosin, X410.)
The choriocapillaris showed foci of fibrosis, obliteration, and residual ghost capillary outlines (figs. 11 to 13). DISCUSSION Histologic choroidal vascular changes are
seen in both benign and malignant hypertension. This choroidopathy may become clinically manifest in cases of malignant hypertension, either essential, associated with chronic glomerulonephritis, or systemic disease such as toxemia of pregnancy, scler-
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Fig. 14 (Morse). Case 2. Section showing thrombosis and infarction of the choriocapillaris with necrosis of the overlying pigment epithelium. Some serous subretinal fluid is seen as well as medial thickening of the arteries. (Hematoxylineosin, X l 6 5 . ) Courtesy of Dr. L. Zimmerman.
oderma, or other collagen disease. The early clinical changes are difficult to observe and may often be obscured by coexistent retinopathy and edema. Ultimately the underlying pathology may become ophthalmoscopically evident only as pigment alterations in the fundi. Despite the plethora of blood vessels the choroid is not invulnerable to significant vascular compromise. The vascular changes in the choroid and retina are emphasized by the fluorescein angiograms which show a blotchy and irregular filling pattern. There is also a
Fig. 15 (Morse). Case 2. Highpower view showing thrombotic obliteration of the choriocapillaris with necrosis of the pigment epithelium. The arterioles show endothelial proliferation to the point of obliteration. (Hematoxylin-eosin, X410.) Courtesy of Dr. L. Zimmerman.
slight delay in the vessel filling time which suggests a generally compromised vascular network. No evidence of vascular discontinuity or leakage was seen angiographically in the reported case which showed healed lesions. The fluorescein also highlights the multiple small drusen seen in this young patient. As would be anticipated the clumps of darker pigment, seen centrally in the Elschnig spot, did not fluoresce. However, the surrounding pale halo with sparse pigmentation (fig. 11) and the other pale areas with diffuse granular pigmentation (fig. 12) flu-
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Fig. 16 (Morse). Case 2. Highpower view showing area of thrombosed choriocapillaris (left) adjacent to a relatively uninvolved portion. Serous separation of the retina is seen. (Hematoxylin-eosin, x410.) Courtesy of Dr. L. Zimmerman.
oresced intensely because the large volume background or underlying choroidal fluorescence had an overlying deficiency of pigment which normally blocks the transmission of this fluorescence. The vascular pathology, which is progressive, shows two main phases, acute and healed. Whereas the reported case shows the healed lesions with secondary pigmentary changes, another case obtained from an autopsy eye shows the more acute phase of hypertensive choroidopathy. The patient was a 52-year-old white woman with hypertensive-cardiovascular-renal disease. She had known hypertension for five years prior to her death with a blood pressure averaging 200/140 to 200/120 mm Hg during the final year of her life. She died of congestive heart failure with uremia. Pathologic sections of her eye showed focal thrombosis of the choriocapillaris, ischemic infarcts, necrosis of the overlying pigment epithelium, and serous retinal separation (figs. 14 to 16). The pathologic findings in these cases are similar to others previously reported. However, other authors have emphasized the acute phase with necrosis of the pigment epithelium secondary to thrombosis of the choriocapillaris. This report shows the pathologic findings in the healed or late phase with
clinically manifest Elschnig's spots. Fluorescein angiograms of the fundi in cases of malignant hypertension with Elschnig's spots have not previously been reported. Clinical manifestations of malignant hypertension have not received much attention in the American ophthalmic literature and yet may be of great magnitude in the understanding of the ocular status in this disease process. SUMMARY A case of malignant hypertension with severe choroidopathy and Elschnig's spots is reported along with fluorescein angiograms of the healed lesions. Hypertensive choroidal pathology which may become clinically evident solely as pigmentary change is discussed. Wilmer Institute (21205) ACKNOWLEDGMENTS I wish to thank Dr. Bertha Klien for her guidance in this study, Mrs. Jane Ponder for preparation of the pathologic sections, Messers. Terry George and Raymond Lund for their photography, and Dr. Lorenz Zimmerman for his history and slide.
REFERENCES 1. Duke-Elder, W . S. : System of Ophthalmology. St. Louis, Mosby, vol. 9, 1966, p. 629. 2. Elschnig, A . : Die diagnostische und prognostische Bedeutung der Netzhauterkrankung bei
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Nephritis. Wien med Wschr. 54:494, 1904. 3. Fuchs, A . : Ueber Choroiditis albuminurica. Klin. Mbl. Augenh. 84:39, 1930. 4. Koyanagi, Y . : Úeber die Pathogenese der Retinitis nephritka. Klin. Mbl. Augenh. 80:436, 1928. 5. Klien, B. A . : Die Blutgefässe der Netzhaut und der Aderhaut bei allgemeiner Sklerodermie, Verhandl. Oesterr. Ophth. Ges. 6:31, 1961. 6. Klien, B. A . : Ischemic infarcts of the choroid
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(Elschnig's spots). A cause of retinal separation in hypertensive disease with renal insufficiency. Am. J. Ophth. (In press). 7. Müller, H . : Das histologische Bild der Chorioiditis allergica bei allergischer Nephritis. Arch, f. Ophth. 150:58,1950. 8. Friedman, E., Smith, T. R., Kuwabara, T. and Beyer, C. K : Choroidal vascular patterns in hypertension. Arch. Ophth. 71:842, 1964.
CHORIORETINITIS
SCLOPETARIA
R . D . RICHARDS, M . D . , C . E . WEST, M . D . AND A . A . MEISELS, M . D . Baltimore, Maryland Chorioretinitis sclopetaria is a term introduced into the German literature to describe an entity of direct choroidal and retinal trauma from a bullet wound in the orbital area. The bullet does not penetrate the globe, but either passes through the orbit between the globe and orbital wall, or lodges adjacent to the globe. The impact of the missile in the tissues adjacent to the wall of the globe causes a concussion-type injury to the globe, characterized as nonpenetrating but causing rupture of the choroid and retina with hemorrhages. Two areas of injury are usually present: the area directly adjacent to the path of the bullet, with damage from direct trauma ; and the macular area, with damage from indirect trauma. Frequently the trauma is so severe that only one large lesion involving both areas is present. Repair produces a white proliferative tissue scar with associated pigmentary disturbance. 1
CASE REPORTS CASE 1 A 50-year-old man was admitted to the University of Maryland Hospital on February 17, 1968, with gunshot wounds of the head. One wound was present in the mid-forehead and another at the infraorbital rim of the right eye (fig. 1 ) . The patient was conscious and responding but unable to give a history. From the Department of Ophthalmology, University of Maryland Medical School. Presented at the 104th Annual Meeting of the American Ophthalmological Society, Hot Springs, Virginia, May 27-29, 1968.
Fig. 1 (Richards, West and Meisels). Bullet wound, lower rim of orbit. Subconjunctival hemorrhage is also visible (Case 1 ) . Skull X-rays showed metallic fragments from a shattered bullet in the right orbit (fig. 2 ) , and additional fragments in the region of the temporal lobe and sylvian fissure within the cranial cavity. The right eyelids were swollen, and a large subconjunctival hemorrhage was present. The cornea was normal and a sluggish but reactive pupil was noted. Vision was recognition of hand movements at two feet. Ophthalmoscopic examination showed a clear vitreous. The entire lower portion of the retina was filled with retinal and choroidal hemorrhages. The area involved the inferior half of the globe, and extended to the macular area and disc (figs. 3 and 4 ) . The left eye was entirely normal, and the rest of the physical examination was normal. The patient's clinical course was uneventful, and no additional changes were noted in the retina. H e was discharged 10 days later, with ocular damage the only apparent residual from the trauma. N o neurologic abnormalities were found.