bilateral scleritis with marginal after cataract...

British Jolurnal of Ophthalmology, 1980, 64, 170-174

Bilateral necrotising scleritis with marginalcorneal ulceration after cataract surgery in apatient with vasculitisSTEPHEN E. BLOOMFIELD,' CARL G. BECKER,2CHARLES L. CHRISTIAN,3 AND JACK S. NAUHEIM4From the Departments of'Ophthalmology and 2Pathology, New York Hospital-Cornell Medical Center,the 3Department of Medicine, Hospital for Special Surgery, and the 4Department of Ophthalmology,State University of New York at Stony Brook, USA

SUMMARY The onset of bilateral necrotising scleritis and marginal corneal ulceration after cataractsurgery are described in a patient with diffuse vasculitis syndrome. The finding of immune complexesin this patient's tissue biopsy suggests that they are the cause of a localised primary vasculitistriggered by surgical inflammation. Clinically, this patient's marginal corneal ulceration precededthe scleral necrosis. A past history of orbital inflammation may have been due to a posteriorscleritis. The corneal ulcerations responded to conjunctival debridement and steroids. The scleralnecrosis was unresponsive to the 2 anti-inflammatory preparations tried, and the patient eventuallywent into remission while off all treatment.

Necrotising scleritis is the most serious and destruc-tive form of scleral disease. It is rare and oftenassociated with a variety of systemic disorders. Thiscase report documents the onset of bilateral necro-tising scleritis and marginal corneal ulceration aftercataract surgery in a patient with a diffuse vasculitissyndrome. Immune complexes found in the con-junctiva and episcleral tissue may have been instru-mental in triggering the inflammatory process.

Case report

A 67-year-old white female with a vasculitis syn-drome presented for ophthalmological evaluationof painless, progressive loss of vision in both eyes.Her systemic disease began 3 years earlier withfever, chest pain, anaemia, and evidence of renalfailure. A renal biopsy showed 'focal lobularglomerulitis'. Early in the course of her illnesschronic serous otitis of the right ear requiredtreatment with a Teflon tube tympanectomy.Methylprednisone therapy was instituted and main-tained continuously. The association of a vasculitissyndrome after acute otitis media has been reported.'At the time of ophthalmological consultation

Correspondence to Stephen E. Bloomfield, MD, 525 East68th Street, New York, New York 10021, USA.

(March 1977) the dose of methylprednisone was48 mg and 32 mg on alternate days.Two years before this eye examination she had

what was diagnosed as a left orbital inflammation,which resolved without sequelae on systemiccorticosteroids. That episode started with a limita-tion of elevation of the globe and was accompaniedby lid oedema and chemosis. An ultrasonogram atthat time revealed thickening of the extraocularmuscles.Her general physical examination when first

seen at the New York Hospital-Cornell MedicalCenter showed a thin, frail-appearing womanconfined to a wheelchair because of severe weaknessand pain due to fractures of the left pubic ramus.Her blood pressure was 160/90 mmHg, there wereecchymotic skin lesions over the extremities, andshe had a mild left hemiparesis. Notable laboratorydata were: haematocrit 23; serum creatinine2-2 mg/100 ml (194 sumol/l) a trace of protein inthe urine; latex fixation test negative; hepatitis Bsurface antigen negative.

Ocular examination showed a best correctedvisual acuity of counting fingers at 5 ft (1 5 m) onthe right and hand motions on the left. Schirmertesting showed an adequate tear volume in botheyes, though slit-lamp examination revealed somefilament formation on the left cornea, suggestive

170

on 27 May 2018 by guest. P

rotected by copyright.http://bjo.bm

j.com/

Br J O

phthalmol: first published as 10.1136/bjo.64.3.170 on 1 M

arch 1980. Dow

nloaded from

http://bjo.bmj.com/

Bilateral necrotising scleritis with marginal corneal ulceration after cataract surgery

1A 1B

Fig. lA Marginal corneal ulceration and infiltration of the dight eye. B: Similar corneal ulceration and earlyscleral necrosis of the left eye. There is prominent vessel formation in the limbal area.

of a tear volume deficit. There was no cornealinfiltration or inflammation of the anterior chamber.There were dense lens opacities in both eyes, theleft greater than the right. There was no sign ofpresent or past scleral inflammation. The intraocularpressure was 17 mmHg in each eye. Indirect ophthal-moscopy through the dense lens opacities revealeda normal disc, vessels, and macular area in eacheye.The patient underwent an uncomplicated left

intracapsular lens extraction on 18 May 1977. Thewound was closed with 5 buried 8-0 silk suturesbeneath a limbal flap. She had an uncomplicatedpostoperative course and was maintained onatropine 1% drops 4 times a day, chloramphenicoldrops 4 times a day, and dexamethasone phosphatedrops twice a day for 5 weeks. The second or righteye underwent a similar uncomplicated cataractextraction 6 weeks after the first operation. Thesame technique with silk sutures was used. Threemonths after her last operation she was comfortableand seeing 20/25 in both eyes from her aphakicspectacles. The eyes were uninflamed except forsome injection at each limbal area.She returned 3 weeks later complaining of discom-

fort in both eyes. Examination of the right eyerevealed erosion of a suture at the 11 o'clock posi-tion 1 mm from the limbus. There was also someperipheral corneal infiltrate from the 11-1 o'clockposition. Examination of the left eye showed onlyerosion of a suture through the conjunctiva at the10 o'clock position of the limbus. The superiorlimbal conjunctiva of each eye appeared somewhatoedematous. We thought we might be dealing witha peripheral staphylococcus corneal infection and/or

a suture abscess. The exposed sutures were removedand topical chloramphenicol treatment was insti-tuted. Culture of each eye showed only a few colo-nies of Staphylococcus epidermidis.One week later the patient returned with more

corneal infiltrate on the right and the start ofinfiltrate on the left. Reculture showed no orga-nisms. She was started on topical dexamethasoneevery 2 hours. A week later she returned still veryuncomfortable, with the process of peripheralcorneal infiltration continuing. The limbal conjunc-tiva looked more inflamed and oedematous. Theremaining buried sutures were removed in theoffice at the slit-lamp, and the patient continuedon topical corticosteroids every 2 hours while awakeand chloramphenicol eye drops 4 times a day.The remaining sutures were removed because itwas thought they might be playing a role in thecontinuation of this uncontrolled inflammation.She returned 5 days later with more peripheralcorneal infiltrate, loss of corneal stroma in eacheye, and scleral melting on the left, radiating fromthe original 10 o'clock suture tract (Figs. IA andB).At this time we decided to debride the oedema-

tous, inflamed, and necrotic-appearing limbalconjunctiva for therapeutic and diagnostic purposes.The patient was admitted to the New York Hospitaland taken to the operating room. The friableconjunctiva and episcleral tissue over each limbaarea were surgically resected in the form of anellipse concentric to the limbus. The superior 180°from the limbus to 4 mm posterior was removedin each eye. The tissue was placed in appropriatemedia and sent for histological and immuno-

171



j.com/

Br J O


arch 1980. Dow

nloaded from

http://bjo.bmj.com/

Stephen E. Bloomfield, Carl G. Becker, Charles L. Christiani, and Jack S. Naitheim

2A 2B

Fig. 2A Advanced scleral necros:s with healing of the marginal corneal ulcerations in the left eye. A characteristicallywhite avascular rim of necrosis is present. B: Fluorescein stain shows residual area of scleral necrosis in the right eye.

fluorescent studies. Postoperatively the patient wasplaced on hourly topical dexamethasone in eacheye around the clock for 1 week, which was gradu-ally tapered off as the corneal infiltrate disappeared.Three weeks after rpmoval of the conjunctiva thecorneas cleared of irifiltrate, developed epithelium,and the patient became much more comfortable.The scleral melting continued in the left eye insi-diously (Fig. 2A).

It was difficult to convince the patient that shestill had a serious problem, since she was nowcomfortable and the eyes were less injected. Shewas started on oral indomethacin, 100 mg per dayin divided doses, to control the scleral necrosis.The topical corticosteroids were continued oncea day in each eye. The scleral necrosis continued.After 1 month the patient was started on oxyphen-butazone, 600 mg per day in divided doses. Thescieral necrosis continued on the left and becameworse on the right. This medication was discon-tinued when no effect was shown after 3 weeks.No other medication was given at this time becauseof a drop in the patient's platelet count. The scleralnecrosis continued for 1 month while the patientwas off all medication and then suddenly stoppedin the left eye and slowed in the right (Fig. 2B).She has now been stable for the last 6 months.

HISTOPATHOLOGICAL FINDINGSMicroscopically the arteries, arterioles, and veinsin the conjunctival-episcleral biopsy were sur-rounded by a cellular infiltrate consisting of poly-morphonuclear neutrophils, lymphocytes, eosino-phils, and in some areas plasma cells. The wallsof some vessels were extensively infiltrated by

inflammatory cells (Figs. 3A and B). In addition,margination and diapedesis of polymorphonuclearneutrophils were present.

Frozen sections of the biopsy which had beenembedded in gelatin were treated with fluores-ceinated rabbit antisera to human IgG, IgA, IgM,IgE, C3, and fibrinogen. Examination of thesesections revealed focal granula deposits of IgG(Fig. 3C) IgM, and C3 (Fig. 3D) in the walls ofconjunctival vessels, indicating that immune com-plexes had been deposited. In addition fibrin wasdeposited focally in vessel walls, presumably atsites of injury (Fig. 3E).

Discussion

Necrotising anterior scleritis is a rare and destructivedisease resulting in necrosis and disappearance ofthe scleral envelope of the eye.2 Its occurrenceafter cataract extraction is unusual. The finding ofimmune complexes in the inflamed tissue suggeststhat they are the cause of localised vasculitis in thispatient.

In the biopsied conjunctival tissue of this patientpolymorphonuclear neutrophils and eosinophilswere adherent to the endothelium of many vesselsand were seen between endothelial cells, presumablyattempting to phagocytose immune complexesdeposited in the lining and in the walls of thesevessels. It has been shown that, when polymorpho-nuclear leucocytes attempt to ingest immunecomplexes on nonphagocytosable surfaces such asvascular membranes, lysosomal enzymes are re-leased into the surrounding tissues.3 4The question arises as to why immune complexes

172



j.com/

Br J O


arch 1980. Dow

nloaded from

http://bjo.bmj.com/

Bilateral necrotising scieritis with marginal corneal ulceration after cataract surgery........ :,....X.W:.

i.

"'' .. _S i.rA#

3A 3B

3D

were deposited in conjunctival-episcleral vessels inthis patient, who at this time had no clinical evi-dence of immune complex deposition in othervascular beds, such as renal glomeruli. We have no

answer to this, but we can offer 2 hypotheses.The first hypothesis concerns the nature of the

blood vessels of the external eye in this patient.Corneal and scleral necrosis began after the patientunderwent surgery for cataracts. New vesselsproliferated during healing of the wound occurred,and it is in this new area that immune complexeswere deposited (Fig. 2B). This may indicate that

Fig. 3A The wall of the smallvein is infiltrated by inflammatorycells. Polymorphonuclear neutrophilsare adherent to the endothelium.Haemaioxyiin and eosin. x 385.B: Polymorphonuclear leucocytesare adherent to the endotheliumof a venule and can be seenemigrating between endothelialcells. Haematoxylin and eosin.x 385. C: Frozen section ofconjunctival biopsy treated withfluoresceinated rabbit antiserum toIgG. Granular immunofluorescentdeposits of IgG are present in the wallof a small blood vessel. x 306. D:Frozen section of conjunctival biopsytreated with fluoresceinated rabbitantiserum to humnan C3. Granularimmunofluorescent deposits of C3 arepresent in the walls of a small bloodvessel. x 306. E: Frozen section ofconjunctival biopsy treated withfluoresceinated antiserum to humanfibrinogen. The wall of an injuredvessel is insulated by fibrin. x 306.

new vessels are more susceptible to deposition ofcirculating immune complexes. If this is true, itwould explain the progressive nature of the disease,since a situation would be created in which injurywould follow reparative vessel growth as long asimmune complexes remained in the circulation.According to this hypothesis patients with circu-lating immune complexes would be at particularrisk of developing the scleral necrosis syndrome,not because of defective healing but because ofincreased vulnerability of new vessels.The second hypothesis, which is not exclusive

173



j.com/

Br J O


arch 1980. Dow

nloaded from

http://bjo.bmj.com/

Stephen E. Bloomfield, Carl G. Becker, Charles L. Christian, and Jack S. Nauheim

of the first, concerns the nature of the patient'scirculating immune complexes. Although we couldnot demonstrate cryoglobulinaemia during theperiod of her illness described above, cryoglobulin-aemia had been demonstrated in the past duringperiods of clinically active systemic disease. Cryo-globulin complexes might be expected to be deposi-ted selectively in superficial vessels of the externaleye, where the temperature would be lower thanin vessels of internal organs. The two hypothesestogether suggest the progression of scleral necrosisin this patient may have been a result of synergybetween the increased susceptibility of new bloodvessels to immune complex deposition and thethermal optimum for precipitation of cryoglobulins.

This hypothesis also suggests that patients witha history of rheumatoid arthritis, lupus erythema-tosus, or other diseases associated with circulatingimmune complexes might be at a special risk ofdeveloping scleral melting after ophthalmic surgeryand should be examined for the presence of circu-lating complexes by a variety of techniques nowavailable.Our patient presented initially with discomfort

and signs of marginal corneal ulceration. Withresolution of the corneal involvement the symptomssubsided, but the destructive scleral process con-tinued in an insidious manner, revealing the correctdiagnosis of necrotising scleritis.The corneal changes are similar to a Mooren's

ulcer, which one might consider in the diagnosis inthe absence of scleral involvement. Both types ofulcers are painful and gradually progressive.Mooren's ulceration, however, is not associatedwith any systemic disease or progressive scleralulceration.5The patient's past episode of left orbital cellulitis,

presenting first with a limitation of elevation, mayhave been due to an unrecognised posterior scleritis.Inflammation of the posterior sclera is a difficultdiagnosis to make and is deduced from its secon-dary effects on neighbouring tissues. The patient

presents with pain, limitation of ocular movements,chemosis, and sometimes lid oedema. The indirectconsequence of inflammation provides the clue todiagnosis, but its appearance will often simulateorbital cellulitis or pseudotumour formation. Ber-telsen6 confesses to this mistake in diagnosis in 6out of 12 patients.The treatment of deep scleritis at various stages

is controversial. Watson and Hayreh2 believe thatscleritis is medically treatable provided the dosagesof the drugs are adequate to suppress the inflam-matory change. Aronson and Elliot disagree.7Many anti-inflammatory agents are now avail-

able, but only a few are effective in the treatment ofscleral disease. These include corticosteroids, indo-methacin, and oxyphenbutazone. Some difficultcases have responded to cytotoxic agents.8 Ourpatient did not respond to indomethacin or oxy-phenbutazone. She underwent a spontaneousremission after being off systemic medications for1 month. The controversy surrounding the treat-ment of deep scleritis is probably due to its unpre-dictable course.

References

'Sargent JS, Christian CL. Necrotising vasculitis afteracute serous otitis media. Ann Intern Med 1974; 81: 195-9.2Watson PG, Hayreh SS. Scleritis and episcleritis. Br JOphthalmol 1976; 60: 163-91.3Henson PM, Oades ZG. Stimulation of human neutrophilsby soluble and insoluble immunoglobulin aggregates.J. Clin Invest 1975; 56: 1053-61.4Henson PM. In: Bayer Symposium VI. ExperimentalModels of Chronic Inflammatory Diseases. New York:Springer, 1977; 94-106.5Ferry AP, Leopold IH. Marginal corneal ulcer as a present-ing manifestation of Wegener's granuloma. Trans AmAcad Ophthalmol Otolaryngol 1970; 74: 1276-82.6Bertelsen TI. Acute sclerotenonitis and ocular myositiscomplicated by papillitis and retinal detachment andglaucoma. Acta Ophthalmol 1960; 38: 136-52.7Aronson SB, Elliot JH. Scleritis. In: Golden B; ed. OcularInflammatory Disease. Springfield: Thomas, 1974; 43.8Jampol LM, West C, Goldberg MF. Therapy of scleritiswith cytotoxic agents. Am J Ophthalmol 1978; 86: 266-71.

174



j.com/

Br J O


arch 1980. Dow

nloaded from

http://bjo.bmj.com/

bilateral scleritis with marginal after cataract...

Documents