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Chorioretinitis Sclopetaria
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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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Fig. 3 (Richards, West and Meisels). Angiograms showing bullet fragments in the orbit and the cranial cavity.
Fig. 3 (Richards, West and Meisels). Drawing of choroidal and retinal hemorrhages one week after gunshot injury (Case 1 ) .
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Fig. 4 (Richards, West and Meisels). Composite retinal photograph one week after gunshot injury (Case 1 ) . Six weeks later ophthalmoscopic examination of the right eye showed proliferative tissue producing white strands in the injured area (fig. 5 ) . Retinal and choroidal hemorrhages were still present but decreased in extent The vitreous remained clear, and no evidence of inflammatory exudate into the vitreous was present. All the clinical aspects of chorioretinitis sclopetaria were present. CASE 2 A 24-year-old motorcycle jockey was admitted to the University of Maryland Hospital on October 1, 1967, with a shotgun wound of the face. He had been struck by pellets from a shotgun blast about 100 to 150 feet away. A t admission he had pellet wounds over the right eyebrow and orbital area, and on the left side of the face. Skull X-rays showed shotgun pellets in die face, and one in the right orbit (fig. 6 ) . Vision was 20/20 in each eye, and external appearance of the globes was normal except for a subconjunctival hemorrhage in the nasal part of the right eye. On ophthalmoscopic examination of the right eye an area of extensive retinal and choroidal hemorrhage was seen in the inferior nasal quadrant White areas thought to be choroidal ruptures were visible in the central part of the lesion (fig. 7 ) .
The clinical course was uneventful except that the vision in the right eye decreased to 20/40 the day after admission because of developing macular edema. He was discharged four days after admission, with no change in the ophthalmoscopic appearance of the eye. H e did not return for follow-up examination, so the development of proliferative scar tissue in the damaged area characteristic of chorioretinitis sclopetaria was not seen.
CASE 3 A 20-year-old man was admitted to the University of Maryland Hospital on August 20, 1966, with a 22 caliber bullet wound to the right side of the face. The wound of entrance was posterior to the lateral rim of the right orbit There was no wound of exit On admission the patient was conscious and had no neurologic abnormalities. Both globes had subconjunctival hemorrhages. The right eye was blind, with a pupil nonreactive to light The fundus was normal. The left pupil was sluggish but did react to light Visual acuity was 20/200. The left fundus showed macular edema. Skull X-rays showed the bullet had passed through the right orbit with fragments lodged in the left orbit The clinical course was uneventful
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Fig. 5 (Richards, West and Meisels). Composite retinal photograph eight weeks after original injury showing proliferative repair (Case 1 ) . and he was discharged 12 days later without any neurologic abnormality. Re-examination in March 1968 showed an esotropia of 15 degrees. The right pupil was semi-dilated and did not react to direct light The fundus was normal except for a pale disc. The eye was completely blind, and the optic atrophy was apparently due to damage to the optic nerve from passage of the bullet through the orbit. The left pupil was normal, and vision was 20/25. The macula had pigmentary changes, and additional pigmentation was present in the temporal area (fig. 8 ) . These changes were thought to be due to direct trauma to the area from one of the bullet fragments, but were not like the strands of scar tissue characteristic of chorioretinitis sclopetaria. CASE 4 A 21-year-old man was admitted to the University of Maryland Hospital on March 16, 1963, with a bullet wound in the head. The entrance wound of the 22 caliber bullet was at the left eyebrow. X -
rays of the skull showed metallic fragments in the left orbit, but no fracture was noted. The patient was conscious, with stable blood pressure and neurologic signs. The left lids were swollen, and an extensive subconjunctival hemorrhage was present The cornea was intact, and a hyphema was present N o light perception was present. The right orbit and eye were entirely normal. On the third day after admission the left eye was removed. N o perforation was found, but microscopic examination showed extensive damage to the retina and choroid, explaining the loss of vision (fig. 9 ) . The anterior chamber was filled with blood, and the iris and ciliary body were dis inserted. The vitreous was filled with a large hemorrhage. The retina was distorted and completely detached. The choroid was markedly thickened and filled with hemorrhages, and in many areas completely detached by a subchoroidal hemorrhage. The pigment epithelium was missing in many areas, and in other areas pigment proliferation was present. N o inflammatory
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Fig. 6 (Richards, West and Meisels). Skull x-rays showing shotgun pellets in right orbit and face. ( A ) Lateral view; ( B ) A - P view (Case 2 ) .
cells were noted. Although the cornea and sclera were intact, the contents of the eye were completely disorganized as a result of the trauma. These changes were compatible with the early changes described in chorioretinitis sclopetaria, but the eye was removed before the characteristic proliferative stage occurred. There were no postoperative complications, and the patient was discharged nine days after admission without evidence of neurologic abnormality.
others who had reported that destruction of the posterior ciliary arteries produced lesions in the choroid and proliferation from pigment epithelium and choroid termed chorioretinitis plastica. He thought that the direct damage to the choroid and retina from the force of the bullet, plus the injury to the cil-
DISCUSSION Goldzieher used the term chorioretinitis plastica sclopetaria in his report published in 1901, to describe the ocular injury from a bullet passing adjacent to the globe but not penetrating it. He felt the changes were so distinctive that the diagnosis could be made on ophthalmoscopic examination. The characteristic picture was an extensive white membrane with strands extending across the surface. The border was usually irregular, with an accumulation of pigment. Goldzieher emphasized that the white areas were not bare sclera, but exudative material, fusing the choroid and retina. He thought the force of the bullet was transferred to the globe, causing rupture of the choroid and hemorrhages. However, he did not think this was enough to cause the exudative white membrane. He referred to the experiments of 1
Fig. 7 (Richards, West and Meisels). Area of injury from nonpenetrating shotgun pellet, one day after injury. Large white choroidal rupture with surrounding hemorrhages, with normal retinal vessel in upper part of photograph (Case 2 ) .
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Fig. 8 (Richards, West and Meisels). Fundus photograph of the left eye showing ( A ) macular changes, and ( B ) pigment proliferation in area temporal to macula. Bullet passed through the right orbit to lodge close to the left globe. iary vessels and nerves, was responsible for the
exudative
inflammatory
called chorioretinitis sclopetaria.
response
he
Sommers
2
described
the
injury
from
a
bullet passing next to the globe as a scar from degeneration and proliferation of the
Fig. 9 (Richard, West and Meisels). Photomicrograph showing extensive damage to the choroid and retina three days after a 22 caliber bullet wound to the orbit. No perforation of the globe was found, but fragments from the bullet were scattered in the. posterior orbital region (Case 4 ) .
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retina and choroid consisting of dense connective tissue, glia, and layers of pigment epithelium. He called this chorioretinitis sclopetaria. In his discussion of trauma, he also used the term chorioretinitis proliferans. He described this as organization of connective tissue and proliferation of vessels attached to the retina which degenerated and from which glia grew into the proliferating mass. He also stated in his discussion of chorioretinitis sclopetaria that tissue proliferating from the choroid into the retina, and budlike into the vitreous body, was chorioretinitis proliferans. He did not describe any inflammatory aspect, nor did he indicate that damage to the ciliary vessels and nerves was part of the picture. 2
Samuels and Fuchs noted indirect trauma with retinal damage and choroidal rupture usually healed without significant proliferation of tissue. In contrast, direct trauma from a bullet passing close to the globe caused severe injury with marked damage to the choroid and retina, with associated extensive hemorrhage. Microscopic examination showed that the white patches seen with the ophthalmoscope were areas of necrosis of the retina, causing degenerative changes and holes. Later, as the repair of the injury took place, neuroglia proliferated, and the pigment epithelium produced a membrane partly with and partly without pigment. This membrane joined the choroid and retina, which lost most of their identifying characteristics. Inflammatory cells were not seen, and no mention of damage to ciliary structures contributing to the lesion was made. The retina surrounding the area of injury remained essentially normal, without evidence of reaction. 8
In Fuchs'* textbook, the term chorioretinitis sclopetarium was used to depict an entity of direct choroidal and retinal trauma, usually as the result of a gunshot wound to the orbit. The bullet passed through the orbit or landed between the wall of the orbit and globe, without penetrating the globe. The impact caused damage to the inner coats of the eye, usually tearing the choroid and retina,
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with resulting hemorrhages. These hemorrhages subsequently became organized, and the final result was a white or whitish-blue scar with sharp serrated borders and pigment proliferation around the edges. Included in this description, but not in reports by other authors, was a clear vitreous and a normal intraocular pressure in the involved globe. Nover referred to retinitis sclopetaria as a severe contusion from a bullet injury to the orbit leading to extensive retinal and choroidal hemorrhages which resulted in large pigmented scars and strands. Although this description was typical, the fundus picture used to illustrate retinitis sclopetaria did not have the characteristic changes described for this entity. Velhagen gave essentially the same description, and used an illustration that did present the typical fundus changes. 5
8
Not all the German ophthalmologists used the term sclopetaria. The first description was by Cohn, in his book dealing with war injuries, published in 1872. His description of the fundus changes was similar to that given by Goldzieher in 1901. Also in Cohn's book was a descrition by Waldeyer of a choroidal focus of 1.5 mm diameter in the posterior pole. Microscopic examination showed exudative inflammatory changes derived from the choroid, with the choroid and retina fused into one layer. He made the anatomic diagnosis of chronic chorioretinitis with the development of a plastic exudate on the inner surface of the retina, associated with fibrinous changes and atrophy of the retina and choroid. 7
The same entity was also described by von Szily in his atlas published in 1917. He did not use the term sclopetaria, but called the process chorioretinitis proliferans. However, his description of the ophthalmoscopic appearance of the lesion and the fundus drawings included left no doubt he was writing about the same condition. He also referred to the reports by Cohn and Goldzieher. In his extensive book was included a report of microscopic examination. In this case, the choroid was condensed, and pigment prolif8
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eration was present extending into the sclera. The retina was largely replaced by a tissue containing hyaline, connective tissue, new vessels, and pigment epithelium. Retinal remnants were visible at the edges. In his discussion of the cases he reported, von Szily wrote that he thought the absorption and organization of the hemorrhages played only a secondary role in relation to the active proliferation of the scar tissue. He also thought damage to the ciliary vessels and nerves might be responsible and referred to experiments by others who produced tissue proliferation after damage to ciliary structures. He did not find inflammatory cells, but did suggest a dystrophic disturbance of the choroid might be responsible for the proliferation of scar tissue in addition to the direct trauma. In Duke-Elder's Text-book of Ophthalmology, the term chorioretinitis proliferans is used. The condition is described as choroidal and retinal laceration with hemorrhage from the direct impact of the^projectile, and with retinal edema and choroidal rupture in the macular area from the force of the concussion traveling through the globe. The macular and peripheral lesions are often confluent in a single area of destruction because of the extent of the force. Mesoblastic elements from the choroid take part in the organization of the damaged area with fibrous tissue to form dense white bands. These do not project into the vitreous, and because they attach the damaged retina and choroid securely together, retinal detachment rarely follows. No suggestion is made of damage to ciliary vessels and nerves contributing to the lesion. Doherty used the term traumatic proliferating choroidoretinitis of Lagrange for the same entity, emphasizing the proliferation with formation of fibrous tracts, the choroid being the productive factor in this unique lesion. 9
9
globe, and the velocity and size of the missile determine the extent of the damage to the choroid and retina. The injury to the inner coats of the eye can be so severe, even without perforation of the sclera, that all vision is lost at once, as in Case 4. Characteristically the injury is severe but limited to a specific area, as in Cases 1 and 2. The damage can be so slight that little evidence of the injury is present, as in Case 3. Case 1 had all of the clinical aspects of chorioretinitis sclopetaria, with localized retinal and choroidal damage from a bullet passing adjacent to the globe without perforating it, and proliferation of white scar tissue in the healing phase. Case 2 had the characteristic localized damage from a shotgun pellet, but the proliferation of scar tissue was not seen since the patient could not be located for follow-up examination. Case 3 had a bullet wound to the orbit, but not close enough to the globe to produce the typical findings. Case 4 had such extensive damage the globe was removed before the characteristic proliferative repair could occur. SUMMARY
The term chorioretinitis sclopetaria is used to describe direct choroidal and retinal trauma from a missile wound to the orbit. Repair is characterized by proliferation of tissue. Strands of white scar tissue fill the center of the damaged area, and pigment proliferation is present around the edges. The globe is not perforated. University of Maryland Hospital (21201) ACKNOWLEDGMENT W e thank Doctor F. C. Blodi for his assistance with the references.
10
Although various names are used, it is clear that the same entity is being described. Bullets and other high velocity missiles penetrating the orbits cause the characteristic changes. The proximity of the missile to the
REFERENCES 1. Goldzieher, W . : Beitrag zur pathologie der orbitalen Schussverletzungen. Z. Augenh. 6:277, 1901. 2. Sommers, I. G. : Histology and Histopathology of the Eye and its Adnexa. New York, Grune and Stratton, 1949, p. 73. 3. Samuels, B. and Fuchs, A. : Clinical Pathology of the Eye. New York, Hoeber, 1952, pp. 22, 341. 4. Fuchs, E. : Lehrbuch der Augenheilkunde. Edited by M . Salzmann, Leipzig and Wien, Franz Deuticke, 1926, ed. 15, p. 691.
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5. Nover, A . : The Ocular Fundus: Methods of Examination and Typical Findings. Translated by F. Blodi, Philadelphia, Lea and Febiger, 1966, p. 130. 6. Velhagen, K. : Der Augenarzt. Leipzig, Georg Thieme, 1963, Band V, p. 825. 7. Cohn, H . : Schussverletzungen des Auges. Breslau, Ferdinand Enke, 1872, p. 24.
RADIATION
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8. von Szily, A . : Atlas der Kriegsaugenheilkunde. Stuttgart, Ferdinand Enke, 1917, p. 254. 9. Duke-Elder, W . S.: Textbook of Ophthalmology. St. Louis, Mosby, 1954, vol. 6, p. 5836. 10. Doherty, W . B. : Some of the most important ocular and orbital wounds in war. Am. J. Ophth. 25:135, 1942.
RETINOPATHY
PERCIVAL H . Y . CHEE, M . D . Honolulu, Hawaii
For many years ophthalmologists concerned with radiation treatment to the eyes have recognized chorioretinal lesions due to radiation. In the English language literature these fundus lesions are mentioned in passing only by those few authors who are experienced in radiation treatment of intraocular malignancies. " The "radiation syndrome" of the fundus occurs typically after a latent period and has a variable picture which may include papillitis, retinal pigment epithelial atrophy, exudates, and microaneurysms or telangiectases. Four cases are presented to focus attention on this entity. 1
8
CASE 1 A 53-year-old white woman examined at the Bascom Palmer Eye Institute in August 1963 gave a history of radiation therapy in April 1960 to the right sinuses for carcinoma. The total dose was approximately 7,000r. Following irradiation, the right cornea ulcerated and was treated with a tarsorraphy. However, glaucoma developed and because of intractable pain the eye was enucleated. The patient did well until January 1963, when there was a sudden onset of blurred vision in the left eye. This was thought to be an optic neuritis and was treated with corticosteroids with some improvement. The patient was referred to the Bascom Palmer Eye Institute for follow-up care. The right orbit was anophthalmic. Visual acuity in the left eye was 20/25, and a mild trichiasis was present. Fields were full to a 1/330 white target and tension was normal. The fundus showed slight temporal blurring of the disc margin with scattered exudates in the posterior fundus. Abnormal vessels could be visualized in the lower temporal quadrant. The patient was followed-up and in January 1964 a few microaneurysms were seen, with an increase in From the Bascom-Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida 33136.
Fig. 1 (Chee) Case 1. Microaneurysms and neovascularization of the posterior pole. January 1964. abnormal vessels (fig. 1 ) . By September 1965 the changes had progressed and the vision had dropped to 20/80 secondary to a serous detachment of the macula. Superonasally and superior to the disc there were abnormal vessels. By January 1966 the vision had improved to 20/25. The patient did well until November 1966, when the vision dropped to 20/200. The fundus lesion had progressed ; there was a macular star with microaneurysms temporal to the disc and abnormal vessels temporal to the macula (figs. 2 and 3 ) . Because of the progression of the lesions, the patient had photocoagulation temporal to the macula by Dr. Edward Maumenee at the Wilmer Institute in February 1967 (figs. 4 and 5 ) , and vision improved to about 20/40. When last seen in January 1968, the macula area was unchanged and the vision was still about 20/40. The untreated areas superior and nasal to the disc were unchanged.
CASE 2 This 22-year-old white woman, first seen at the Bascom Palmer Eye Institute in April 1966, had
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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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Fig. 3 (Richards, West and Meisels). Angiograms showing bullet fragments in the orbit and the cranial cavity.
Fig. 3 (Richards, West and Meisels). Drawing of choroidal and retinal hemorrhages one week after gunshot injury (Case 1 ) .
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Fig. 4 (Richards, West and Meisels). Composite retinal photograph one week after gunshot injury (Case 1 ) . Six weeks later ophthalmoscopic examination of the right eye showed proliferative tissue producing white strands in the injured area (fig. 5 ) . Retinal and choroidal hemorrhages were still present but decreased in extent The vitreous remained clear, and no evidence of inflammatory exudate into the vitreous was present. All the clinical aspects of chorioretinitis sclopetaria were present. CASE 2 A 24-year-old motorcycle jockey was admitted to the University of Maryland Hospital on October 1, 1967, with a shotgun wound of the face. He had been struck by pellets from a shotgun blast about 100 to 150 feet away. A t admission he had pellet wounds over the right eyebrow and orbital area, and on the left side of the face. Skull X-rays showed shotgun pellets in die face, and one in the right orbit (fig. 6 ) . Vision was 20/20 in each eye, and external appearance of the globes was normal except for a subconjunctival hemorrhage in the nasal part of the right eye. On ophthalmoscopic examination of the right eye an area of extensive retinal and choroidal hemorrhage was seen in the inferior nasal quadrant White areas thought to be choroidal ruptures were visible in the central part of the lesion (fig. 7 ) .
The clinical course was uneventful except that the vision in the right eye decreased to 20/40 the day after admission because of developing macular edema. He was discharged four days after admission, with no change in the ophthalmoscopic appearance of the eye. H e did not return for follow-up examination, so the development of proliferative scar tissue in the damaged area characteristic of chorioretinitis sclopetaria was not seen.
CASE 3 A 20-year-old man was admitted to the University of Maryland Hospital on August 20, 1966, with a 22 caliber bullet wound to the right side of the face. The wound of entrance was posterior to the lateral rim of the right orbit There was no wound of exit On admission the patient was conscious and had no neurologic abnormalities. Both globes had subconjunctival hemorrhages. The right eye was blind, with a pupil nonreactive to light The fundus was normal. The left pupil was sluggish but did react to light Visual acuity was 20/200. The left fundus showed macular edema. Skull X-rays showed the bullet had passed through the right orbit with fragments lodged in the left orbit The clinical course was uneventful
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Fig. 5 (Richards, West and Meisels). Composite retinal photograph eight weeks after original injury showing proliferative repair (Case 1 ) . and he was discharged 12 days later without any neurologic abnormality. Re-examination in March 1968 showed an esotropia of 15 degrees. The right pupil was semi-dilated and did not react to direct light The fundus was normal except for a pale disc. The eye was completely blind, and the optic atrophy was apparently due to damage to the optic nerve from passage of the bullet through the orbit. The left pupil was normal, and vision was 20/25. The macula had pigmentary changes, and additional pigmentation was present in the temporal area (fig. 8 ) . These changes were thought to be due to direct trauma to the area from one of the bullet fragments, but were not like the strands of scar tissue characteristic of chorioretinitis sclopetaria. CASE 4 A 21-year-old man was admitted to the University of Maryland Hospital on March 16, 1963, with a bullet wound in the head. The entrance wound of the 22 caliber bullet was at the left eyebrow. X -
rays of the skull showed metallic fragments in the left orbit, but no fracture was noted. The patient was conscious, with stable blood pressure and neurologic signs. The left lids were swollen, and an extensive subconjunctival hemorrhage was present The cornea was intact, and a hyphema was present N o light perception was present. The right orbit and eye were entirely normal. On the third day after admission the left eye was removed. N o perforation was found, but microscopic examination showed extensive damage to the retina and choroid, explaining the loss of vision (fig. 9 ) . The anterior chamber was filled with blood, and the iris and ciliary body were dis inserted. The vitreous was filled with a large hemorrhage. The retina was distorted and completely detached. The choroid was markedly thickened and filled with hemorrhages, and in many areas completely detached by a subchoroidal hemorrhage. The pigment epithelium was missing in many areas, and in other areas pigment proliferation was present. N o inflammatory
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Fig. 6 (Richards, West and Meisels). Skull x-rays showing shotgun pellets in right orbit and face. ( A ) Lateral view; ( B ) A - P view (Case 2 ) .
cells were noted. Although the cornea and sclera were intact, the contents of the eye were completely disorganized as a result of the trauma. These changes were compatible with the early changes described in chorioretinitis sclopetaria, but the eye was removed before the characteristic proliferative stage occurred. There were no postoperative complications, and the patient was discharged nine days after admission without evidence of neurologic abnormality.
others who had reported that destruction of the posterior ciliary arteries produced lesions in the choroid and proliferation from pigment epithelium and choroid termed chorioretinitis plastica. He thought that the direct damage to the choroid and retina from the force of the bullet, plus the injury to the cil-
DISCUSSION Goldzieher used the term chorioretinitis plastica sclopetaria in his report published in 1901, to describe the ocular injury from a bullet passing adjacent to the globe but not penetrating it. He felt the changes were so distinctive that the diagnosis could be made on ophthalmoscopic examination. The characteristic picture was an extensive white membrane with strands extending across the surface. The border was usually irregular, with an accumulation of pigment. Goldzieher emphasized that the white areas were not bare sclera, but exudative material, fusing the choroid and retina. He thought the force of the bullet was transferred to the globe, causing rupture of the choroid and hemorrhages. However, he did not think this was enough to cause the exudative white membrane. He referred to the experiments of 1
Fig. 7 (Richards, West and Meisels). Area of injury from nonpenetrating shotgun pellet, one day after injury. Large white choroidal rupture with surrounding hemorrhages, with normal retinal vessel in upper part of photograph (Case 2 ) .
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Fig. 8 (Richards, West and Meisels). Fundus photograph of the left eye showing ( A ) macular changes, and ( B ) pigment proliferation in area temporal to macula. Bullet passed through the right orbit to lodge close to the left globe. iary vessels and nerves, was responsible for the
exudative
inflammatory
called chorioretinitis sclopetaria.
response
he
Sommers
2
described
the
injury
from
a
bullet passing next to the globe as a scar from degeneration and proliferation of the
Fig. 9 (Richard, West and Meisels). Photomicrograph showing extensive damage to the choroid and retina three days after a 22 caliber bullet wound to the orbit. No perforation of the globe was found, but fragments from the bullet were scattered in the. posterior orbital region (Case 4 ) .
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retina and choroid consisting of dense connective tissue, glia, and layers of pigment epithelium. He called this chorioretinitis sclopetaria. In his discussion of trauma, he also used the term chorioretinitis proliferans. He described this as organization of connective tissue and proliferation of vessels attached to the retina which degenerated and from which glia grew into the proliferating mass. He also stated in his discussion of chorioretinitis sclopetaria that tissue proliferating from the choroid into the retina, and budlike into the vitreous body, was chorioretinitis proliferans. He did not describe any inflammatory aspect, nor did he indicate that damage to the ciliary vessels and nerves was part of the picture. 2
Samuels and Fuchs noted indirect trauma with retinal damage and choroidal rupture usually healed without significant proliferation of tissue. In contrast, direct trauma from a bullet passing close to the globe caused severe injury with marked damage to the choroid and retina, with associated extensive hemorrhage. Microscopic examination showed that the white patches seen with the ophthalmoscope were areas of necrosis of the retina, causing degenerative changes and holes. Later, as the repair of the injury took place, neuroglia proliferated, and the pigment epithelium produced a membrane partly with and partly without pigment. This membrane joined the choroid and retina, which lost most of their identifying characteristics. Inflammatory cells were not seen, and no mention of damage to ciliary structures contributing to the lesion was made. The retina surrounding the area of injury remained essentially normal, without evidence of reaction. 8
In Fuchs'* textbook, the term chorioretinitis sclopetarium was used to depict an entity of direct choroidal and retinal trauma, usually as the result of a gunshot wound to the orbit. The bullet passed through the orbit or landed between the wall of the orbit and globe, without penetrating the globe. The impact caused damage to the inner coats of the eye, usually tearing the choroid and retina,
N O V E M B E R , 1968
with resulting hemorrhages. These hemorrhages subsequently became organized, and the final result was a white or whitish-blue scar with sharp serrated borders and pigment proliferation around the edges. Included in this description, but not in reports by other authors, was a clear vitreous and a normal intraocular pressure in the involved globe. Nover referred to retinitis sclopetaria as a severe contusion from a bullet injury to the orbit leading to extensive retinal and choroidal hemorrhages which resulted in large pigmented scars and strands. Although this description was typical, the fundus picture used to illustrate retinitis sclopetaria did not have the characteristic changes described for this entity. Velhagen gave essentially the same description, and used an illustration that did present the typical fundus changes. 5
8
Not all the German ophthalmologists used the term sclopetaria. The first description was by Cohn, in his book dealing with war injuries, published in 1872. His description of the fundus changes was similar to that given by Goldzieher in 1901. Also in Cohn's book was a descrition by Waldeyer of a choroidal focus of 1.5 mm diameter in the posterior pole. Microscopic examination showed exudative inflammatory changes derived from the choroid, with the choroid and retina fused into one layer. He made the anatomic diagnosis of chronic chorioretinitis with the development of a plastic exudate on the inner surface of the retina, associated with fibrinous changes and atrophy of the retina and choroid. 7
The same entity was also described by von Szily in his atlas published in 1917. He did not use the term sclopetaria, but called the process chorioretinitis proliferans. However, his description of the ophthalmoscopic appearance of the lesion and the fundus drawings included left no doubt he was writing about the same condition. He also referred to the reports by Cohn and Goldzieher. In his extensive book was included a report of microscopic examination. In this case, the choroid was condensed, and pigment prolif8
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CHORIORETINITIS SCLOPETARIA
eration was present extending into the sclera. The retina was largely replaced by a tissue containing hyaline, connective tissue, new vessels, and pigment epithelium. Retinal remnants were visible at the edges. In his discussion of the cases he reported, von Szily wrote that he thought the absorption and organization of the hemorrhages played only a secondary role in relation to the active proliferation of the scar tissue. He also thought damage to the ciliary vessels and nerves might be responsible and referred to experiments by others who produced tissue proliferation after damage to ciliary structures. He did not find inflammatory cells, but did suggest a dystrophic disturbance of the choroid might be responsible for the proliferation of scar tissue in addition to the direct trauma. In Duke-Elder's Text-book of Ophthalmology, the term chorioretinitis proliferans is used. The condition is described as choroidal and retinal laceration with hemorrhage from the direct impact of the^projectile, and with retinal edema and choroidal rupture in the macular area from the force of the concussion traveling through the globe. The macular and peripheral lesions are often confluent in a single area of destruction because of the extent of the force. Mesoblastic elements from the choroid take part in the organization of the damaged area with fibrous tissue to form dense white bands. These do not project into the vitreous, and because they attach the damaged retina and choroid securely together, retinal detachment rarely follows. No suggestion is made of damage to ciliary vessels and nerves contributing to the lesion. Doherty used the term traumatic proliferating choroidoretinitis of Lagrange for the same entity, emphasizing the proliferation with formation of fibrous tracts, the choroid being the productive factor in this unique lesion. 9
9
globe, and the velocity and size of the missile determine the extent of the damage to the choroid and retina. The injury to the inner coats of the eye can be so severe, even without perforation of the sclera, that all vision is lost at once, as in Case 4. Characteristically the injury is severe but limited to a specific area, as in Cases 1 and 2. The damage can be so slight that little evidence of the injury is present, as in Case 3. Case 1 had all of the clinical aspects of chorioretinitis sclopetaria, with localized retinal and choroidal damage from a bullet passing adjacent to the globe without perforating it, and proliferation of white scar tissue in the healing phase. Case 2 had the characteristic localized damage from a shotgun pellet, but the proliferation of scar tissue was not seen since the patient could not be located for follow-up examination. Case 3 had a bullet wound to the orbit, but not close enough to the globe to produce the typical findings. Case 4 had such extensive damage the globe was removed before the characteristic proliferative repair could occur. SUMMARY
The term chorioretinitis sclopetaria is used to describe direct choroidal and retinal trauma from a missile wound to the orbit. Repair is characterized by proliferation of tissue. Strands of white scar tissue fill the center of the damaged area, and pigment proliferation is present around the edges. The globe is not perforated. University of Maryland Hospital (21201) ACKNOWLEDGMENT W e thank Doctor F. C. Blodi for his assistance with the references.
10
Although various names are used, it is clear that the same entity is being described. Bullets and other high velocity missiles penetrating the orbits cause the characteristic changes. The proximity of the missile to the
REFERENCES 1. Goldzieher, W . : Beitrag zur pathologie der orbitalen Schussverletzungen. Z. Augenh. 6:277, 1901. 2. Sommers, I. G. : Histology and Histopathology of the Eye and its Adnexa. New York, Grune and Stratton, 1949, p. 73. 3. Samuels, B. and Fuchs, A. : Clinical Pathology of the Eye. New York, Hoeber, 1952, pp. 22, 341. 4. Fuchs, E. : Lehrbuch der Augenheilkunde. Edited by M . Salzmann, Leipzig and Wien, Franz Deuticke, 1926, ed. 15, p. 691.
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5. Nover, A . : The Ocular Fundus: Methods of Examination and Typical Findings. Translated by F. Blodi, Philadelphia, Lea and Febiger, 1966, p. 130. 6. Velhagen, K. : Der Augenarzt. Leipzig, Georg Thieme, 1963, Band V, p. 825. 7. Cohn, H . : Schussverletzungen des Auges. Breslau, Ferdinand Enke, 1872, p. 24.
RADIATION
N O V E M B E R , 1968
8. von Szily, A . : Atlas der Kriegsaugenheilkunde. Stuttgart, Ferdinand Enke, 1917, p. 254. 9. Duke-Elder, W . S.: Textbook of Ophthalmology. St. Louis, Mosby, 1954, vol. 6, p. 5836. 10. Doherty, W . B. : Some of the most important ocular and orbital wounds in war. Am. J. Ophth. 25:135, 1942.
RETINOPATHY
PERCIVAL H . Y . CHEE, M . D . Honolulu, Hawaii
For many years ophthalmologists concerned with radiation treatment to the eyes have recognized chorioretinal lesions due to radiation. In the English language literature these fundus lesions are mentioned in passing only by those few authors who are experienced in radiation treatment of intraocular malignancies. " The "radiation syndrome" of the fundus occurs typically after a latent period and has a variable picture which may include papillitis, retinal pigment epithelial atrophy, exudates, and microaneurysms or telangiectases. Four cases are presented to focus attention on this entity. 1
8
CASE 1 A 53-year-old white woman examined at the Bascom Palmer Eye Institute in August 1963 gave a history of radiation therapy in April 1960 to the right sinuses for carcinoma. The total dose was approximately 7,000r. Following irradiation, the right cornea ulcerated and was treated with a tarsorraphy. However, glaucoma developed and because of intractable pain the eye was enucleated. The patient did well until January 1963, when there was a sudden onset of blurred vision in the left eye. This was thought to be an optic neuritis and was treated with corticosteroids with some improvement. The patient was referred to the Bascom Palmer Eye Institute for follow-up care. The right orbit was anophthalmic. Visual acuity in the left eye was 20/25, and a mild trichiasis was present. Fields were full to a 1/330 white target and tension was normal. The fundus showed slight temporal blurring of the disc margin with scattered exudates in the posterior fundus. Abnormal vessels could be visualized in the lower temporal quadrant. The patient was followed-up and in January 1964 a few microaneurysms were seen, with an increase in From the Bascom-Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida 33136.
Fig. 1 (Chee) Case 1. Microaneurysms and neovascularization of the posterior pole. January 1964. abnormal vessels (fig. 1 ) . By September 1965 the changes had progressed and the vision had dropped to 20/80 secondary to a serous detachment of the macula. Superonasally and superior to the disc there were abnormal vessels. By January 1966 the vision had improved to 20/25. The patient did well until November 1966, when the vision dropped to 20/200. The fundus lesion had progressed ; there was a macular star with microaneurysms temporal to the disc and abnormal vessels temporal to the macula (figs. 2 and 3 ) . Because of the progression of the lesions, the patient had photocoagulation temporal to the macula by Dr. Edward Maumenee at the Wilmer Institute in February 1967 (figs. 4 and 5 ) , and vision improved to about 20/40. When last seen in January 1968, the macula area was unchanged and the vision was still about 20/40. The untreated areas superior and nasal to the disc were unchanged.
CASE 2 This 22-year-old white woman, first seen at the Bascom Palmer Eye Institute in April 1966, had