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Transient Vessel Wall Sheathing in Acute Retinal Vein Occlusions A. J. E

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Transient Vessel Wall Sheathing in Acute Retinal Vein Occlusions A. J. E TRANSIENT VESSEL WALL SHEATHING IN ACUTE RETINAL VEIN OCCLUSIONS A. J. E. FOSS/ M. P. HEADON/ A. M. HAMILTON2, S. LIGHTMAN2 Reading and London SUMMARY before presentation he had noted a blind spot in the tem­ Three cases are reported which had features similar to, poral fieldof his left eye, which had lasted for one day and and evolved in a pattern consistent with central retinal had resolved. vein occlusions and a fourth case is reported which The vision was 6/5 in the right eye and 6/60 in the left behaved as a hemispheric vein occlusion. However, they eye with a afferent pupillary defect. There were cells+ in differed from classic retinal vein occlusions by having the anterior chamber and fine keratic precipitates were prominent sheathing of the retinal venous vasculature at noted. There were no cells in the vitreous. The intraocular presentation, which in all four cases resolved within three pressures were normal in both eyes. The dramatic findings weeks. There was no evidence for any of these cases were on fundal examination (see Fig. la). All the retinal having an inflammatory vasculitis. The significance of veins showed sheathing with the tributary venules termi­ this transient sheathing is uncertain. nating in retinal haemorrhages. The optic disc was swol­ len and there was pronounced cystoid macular oedema. Three cases are reported of a condition that behaved like There were also widespread scattered retinal haemor­ ischaemic central retinal vein occlusion (CRVO) and a rhages. A few cells only were noted in the anterior fourth with a similar appearance, but with the features of a vitreous. hemispheric vein occlusion. They all differed from classic Fluorescein angiography at this stage showed diffuse retinal vein occlusions by their striking fundal appearance venous leakage and macular oedema (Fig. lb). at presentation, with sheathing of the walls of the involved Full assessment was performed by a general physician retinal venous system. Sheathing of retinal vessels is a and no evidence of a systemic disorder, particularly Beh­ recognised late phenomenon in both central and branch get'S disease or sarcoidosis, could be found on history, full retinal vein occlusions, but is not a recognised feature at physical examination or investigation, which included full presentation in the absence of inflammation. blood count, biochemistry profile, autoantibody screen Such a fundal appearance at presentation suggests a dif­ (including ANA and ANCA) , immunology, virology ferential diagnosis of retinal vasculitis, reticulum cell sar­ (including herpes viruses), SACE, VDRL, chest radio­ coma, frosted branch angiitis or papillophlebitis. graph and urine analysis. A CT scan of his sinuses and However, the sheathing resolved within three weeks and orbits showed normal enhancement and a B-scan showed otherwise the features and clinical course were similar to no abnormalities in either the retina or the optic nerve. retinal vein occlusions. He was started on prednisolone at 60 mg/day andhad a PATIENTS reducing course over ten days. The vision in his left eye Case One improved to 6/12 and the sheathing had disappeared by one week, but the haemorrhages persisted and a star exu­ A 23-year-old man presented to casualty with a one-day date formed at the macula (see Fig. lc). By four weeks the history of blurring of vision in his left eye. Two months vision had deteriorated to 6/36 and the fundal appearances previously he had had a vesicular rash of uncertain cause were those of a classical CRVO with cotton wool spots. that affected his back, arm and legs. This had cleared By four months, iris rubeosis had developed and flu­ within a week without obvious sequelae. Two weeks orescein angiography demonstrated severe ischaemia From Royal Berkshire Hospital, Reading' and Moorfields Eye (Fig. ld). He received extensive pan-retinal photocoag­ Hospital, London ECIV 2P02. Correspondence to: A. J. E. Foss, Moorfields Eye Hosptial, City ulation, but developed rubeotic glaucoma. His vision is Road, London ECIV 2PO. currently perception of light only. Eye (1992) 6, 313-316 314 A. J. E. FOSS ET AL. Fig. la. The fundal appearance of case one at presentation, showing dilatation and sheathing of the retinal veins, widespread retinal haemorrhage and swelling of the optic nerve Fig. Ie. The fundal appearance one week later, showing the head. resolution of the sheathing and the formation of a macular star. Fig. lb. The accompanying fluorescein angiogram shows Fig. ld. A fluorescein angiogram performed four months continuous leakage offluorescein from the venous system. later, showing the development of severe ischaemia. Case Two the end of the third week (Fig. 2c). The cystoid macular A 32-year-old man presented with a twenty-four hour oedema, however, persisted and the visual acuity history of severe left visual loss. He was otherwise per­ remained unaltered at 6/60. His condition was unchanged fectly healthy. His vision was 6/5 in the right eye and 6/60 six months later. in the left. There were no signs of ocular inflammation and Case Three the intraocular pressures were normal. Fundal examin­ ation demonstrated engorgement of all the retinal veins A 54-year-old man presented to the casualty department with sheathing of the inferior retinal veins and tributaries with a twelve hour history of severe visual loss in the right (Fig. 2a). There was swelling of the optic disc, cystoid eye. The preceding day had been very hot and that evening macular oedema and widespread scattered retinal haemor­ he drank five pints of beer. He slept heavily face down and rhages. A fluorescein angiogram demonstrated leakage of woke with his fist pressed against his right eye. The vision the inferior retinal veins in a continuous manner in the right eye was counting fingers and 6/5 in left eye (Figs. 2b). with a right afferent pupillary defect. The anterior segment A full assessment by a general physician found no other was normal with normal intraocular pressure. The fundal abnormality. A CT scan of the brain and orbits was picture was that of a CRVO but the striking feature was the normal. sheathing of the vein walls, which was virtually continu­ Over the next two weeks the visual acuity remained ous. There were scattered haemorrhages, cotton wool unchanged, but the sheathing had completely vanished by spots, disc swelling and macular oedema. VESSEL SHEATHING IN VEIN OCCLUSION 315 (a) (c) Fig. 2a. The fundal appearance of case two at presentation, showing the dilatation of the retinal veins, sheathing of the inferior retinal veins and widespread retinal haemorrhages in the inferior half of the fundus. The sheathing of the inferior retinal veins is continuous and many of the sheathed venules are further outlined with haemorrhage. Fig. 2b. The accompanying fluorescein angiogram shows continuous leakage offluorescein from the inferior retinal veins. Fig. 2e. This shows complete resolution of the sheathing, retinal vein dilatation and haemorrhages in case two, by the third week. There are some resolving deposits of hard exudate in (b) the macular region. He was a non-smoker and normotensive. Full history The patient was admitted and commenced on 60 mg of and examination was performed by a general physician prednisolone per day. She was fully examined and investi­ and all investigations were normal, including a FBC, bio­ gated by physicians and no evidence for a systemic vas­ chemical screen, autoantibodies and a chest radiograph. culitis could be found, but a right carotid bruit was noted. Fluorescein angiography performed two days later Fundus fluorescein angiography suggested an ischaemic showed that there was leakage of dye from the venous CRVO and pan-retinal photocoagulation was performed system in a contiguous manner and not in the patchy man­ and steroids gradually withdrawn. The sheathing resolved ner characteristic of retinal vasculitis. Areas of capillary over eight days. In view of the poor vision at a level that non-perfusion were seen and pan-retinal photocoagula­ could not be accounted for by a CRVO alone, a CT scan tion was carried out. His vision remained poor due to cys­ was performed and demonstrated a large frontal men­ toid macular oedema. ingioma extending into both orbits. She was referred to the The sheathing was a transient phenomenon and had neurosurgeons who removed the tumour, but she died resolved by ten days, after which the fundal appearance post -operativel y. resembled a classical CRVO. DISCUSSION Case Four Four cases have been presented, which behaved like ret­ A 75-year-old lady presented to casualty with a two-day inal vein occlusions, but in which transient retinal wall history of loss of vision in her right eye. The left eye had sheathing was present from the onset. The sheathing was had no perception of light since childhood, which had continuous with periodic interruptions, which respected been attributed to meningitis. She had no relevant pre­ vious medical or ocular history. Examination revealed per­ a-v crossings, or could be attributed to overlying haemor­ ception of light vision in the right eye only, with a few cells rhage. The venules often ended in an area of haemorrhage. in the anterior chamber and the anterior vitreous. The Some of the venules had haemorrhage running parallel to ocular pressure was normal. Fundal examination showed a their walls, outlining the sheathing and giving the vessels a swollen disc and veins. The venous walls showed continu­ red layer lying just outside, and contiguous with, the ous sheathing. There were widespread retinal haemor­ sheathing. Fluorescein angiography showed uniform leak­ rhages and marked macular oedema. The left eye was age from the vein walls. In all cases the sheathing resolved phthisical. within three weeks. All four cases had an acute onset and 316 A. J. E.
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