British Journal of Ophthalmology, 1985, 69, 836-840

Clinically unsuspected phacoanaphylaxis afterextracapsular cataract extraction with intraocular lensimplantationMARK S McMAHON, JAYNE S WEISS, KLAUS G RIEDEL,AND DANIEL M ALBERT

From the David G Cogan Eye Pathology Laboratory ofthe Massachusetts Eye and Ear Infirmary, HarvardMedical School, Boston, Massachusetts, USA

SUMMARY We describe the histopathological findings of an enucleation specimen from a clinicallyundiagnosed case of phacoanaphylaxis which occurred after extracapsular cataract extraction withintraocular lens implantation. Phacoanaphylaxis, a granulomatous inflammatory response toliberated lens protein, is potentially curable by prompt removal of residual lens material. This caseemphasises that the diagnosis of phacoanaphylactic endophthalmitis should be considered in anycase of extracapsular cataract extraction complicated by granulomatous intraocular inflammation.

In 1919 Straub' discussed the possibility thatliberated lenticular protein could incite uveitis. In1922 Verhoeff and Lemoine2 introduced the term'endophthalmitis phacoanaphylactica' for this lens-induced intraocular inflammation. Riise3 demon-strated that phacoanaphylaxis following extra-capsular cataract extraction could be dramaticallyarrested by prompt removal of residual lens material.We describe the histopathological findings of anenucleation specimen from a clinically undiagnosedcase of phacoanaphylaxis which occurred after extra-capsular cataract extraction. This case emphasisesthat phacoanaphylactic endophthalmitis should besuspected in any eye with persistent unexplaineduveitis after extracapsular cataract extraction.

Case report

The patient was an 80-year-old female who under-went extracapsular cataract extraction in 1983 withimplantation of a Simcoe anterior chamber lens. Fivedays postoperatively the patient had iris bombe, andlaser iridotomy was performed to relieve the pupil-lary block. The patient continued to experiencesevere intraocular inflammation, suspected to beinfectious endophthalmitis. This did not respond totreatment, and the eye became increasingly painful,Correspondence to Daniel M Albert, MD, Massachusetts Eye andEar Infirmary, 243 Charles Street, Boston, Massachusetts 02114,USA.

with only light perception vision. Six weeks aftercataract extraction the eye was enucleated.

PATHOLOGICAL FINDINGSGross examination revealed a shrunken, distortedeye which measured 24 mm in antero-posteriordiameter. The sclera contained foci of haemorrhage,the cornea was cloudy, and the iris was poorlyvisualised. On opening the eye the vitreous was seento be replaced by a gelatinous, dark red-browncoagulum containing a 0-5 cm roughly oval grey-white mass involving the lens remnants (Fig. 1).On microscopic examination the cornea was noted

to contain a well-healed full-thickness scar withchronic inflammatory cells, red blood cells, andextracellular pigment granules. The basal cell layer ofthe epithelium was noted to be oedematous.Bowman's membrane was interrupted peripherallyby a fibrovascular pannus containing chronic inflam-matory cells. Descemet's membrane was frag-mented at the site of the cataract incision.Endothelial cells were decreased in number and aretrocorneal membrane was present. The anteriorchamber was shallow and contained chronic inflam-matory cells, red blood cells, and pigment granules.The iris was infiltrated by plasma cells, lymphocytes,and epithelioid cells. The iris was adherent peripher-ally to the retrocorneal membrane, which closed theangle on one side. Posteriorly the iris was adherent toan inflammatory mass which enveloped residual lens

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Clinically unsuspectedphacoanaphylaxis after extracapsular cataract extraction

Fig. 1 Low magnification viewshowingphthisical eye.

> Inflammatory mass (arrow) can be} visualised posterior to iris.

(Haematoxylin and eosin, x 7-5).

Fig. 2 Inflammatory masscontaining lens capsule andliquefied lens cortex material.(Haematoxylin and eosin, x28).

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Mark S McMahon, Jayne S Weiss, Klaus G Riedel, and DanielM Albert

cortex and capsule (Figs. 2, 3). The capsule wasthickened and folded. Remnants of lens corticalmaterial demonstrated liquefaction and were infiltra-ted by eosinophils, polymorphonuclear leucocytes,and lymphocytes (Fig. 4). Further peripherally,epithelioid cells, giant cells, cystic spaces, and redblood cells were present (Fig. 5). The ciliary bodywas atrophic, hyalinised, and encased in the inflam-matory mass.The retina, which was attached in part to the

inflammatory mass, was totally detached, dis-organised, and atrophic. Subretinal serous fluidcontained blood, non-granulomatous chronic-inflam-matory cells, polymorphonuclear leucocytes, pig-ment granules, and pigment-laden macrophages.The retinal pigment epithelium was absent in someareas. The choroid was partly detached and infil-trated by non-granulomatous chronic inflammatorycells. The sclera was folded and thickened.

Discussion

Endophthalmitis phacoanaphylactica is the termintroduced by Verhoeff and Lemoine2 for the lens-induced endophthalmitis which was previously des-cribed by Straub.' Phacoanaphylaxis is generally heldto be an autoimmune phenomenon which occurs inresponse to the release of sequestered 'non-selfantigens, usually as a result of rupture of the lenscapsule or possibly leakage of lens protein throughthe capsule. Marak and coworkers45 believe that thisconcept does not explain why lens injury onlyirregularly leads to antilens antibody production norwhy phacoanaphylactic endophthalmitis is such arare disease. They contend that T cell tolerance isnormally maintained by small amounts of circulatinglens protein and that the pathogenesis of phacoana-phylaxis involves reversal of acquired tolerance at theT cell level. This tolerance may be altered by trauma,possibly through the adjuvant effect of wound con-tamination.6

Clinically the onset can occur as early as 1 to 14days after leakage of lens material, and is manifest bygranulomatous uveitis in the injured eye centredaround the region of the lens. The inflammation maydevelop insidiously or acutely with congestion, a hazycornea, white keratic precipitates, and an intensecellular exudation into the anterior chamber.7The clinical diagnosis of phacoanaphylaxis, par-

ticularly in an eye with an intraocular lens implant, isdifficult, and on routine examination the conditionmay be indistinguishable from granulomatous uveitisof other causes, or endophthalmitis. The fact that theinflammation is centred round the lens may bedetected 'by ultrasound. Paracentesis typicallyreveals evidence of chronic granulomatous inflam-

Fig. 3 The iris (arrow) is shown to be adherentposteriorlyto the inflammatory mass. The iris is infiltrated byplasmacells, lymphocytes, and epithelioid cells. (Haematoxylin andeosin, x80).

mation rather than the acute inflammation ofbacterial endophthalmitis. It should be kept in mind,however, that fungal endophthalmitis can causegranulomatous inflammation. A high index ofsuspicion for phacoanaphylaxis in eyes with residuallens material and granulomatous inflammation isadvised. Should such an eye. not respond to steroidand/or antibiotic treatment, prompt removal of theresidual lens would be necessary for definitive diag-nosis and treatment.

Histopathologically the inflammatory reaction inphacoanaphylaxis is centred round the damaged lens,which is extensively infiltrated by polymorpho-nuclear leucocytes and eosinophils. Surrounding thelens is a zone of epithelioid and giant cells. Theadjacent iris and ciliary body are inflamed andinfiltrated with lymphocytes and plasma cells.7The clinician must be able to differentiate

phacoanphylaxis from other complications of catar-act surgery such as phacolytic glaucoma, sympatheticophthalmia, or endophthalmitis. In phacolytic

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Fig. 4 High magnification ofacuteinflammatory reaction aroundfolded lens capsule (arrows) andliquefied lens cortex material(asterisk). (Haematoxylin andeosin, x200).

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glaucoma, macrophages engorged with lens materialbecome lodged in the trabecular meshwork. Theclinical picture consists of a severe increase of intra-ocular pressure with cells in the anterior chamber.Keratic precipitates and posterior synechiae, oftenseen with phacoanaphylaxis, are typically absent.Sympathetic ophthalmia is a bilateral, granuloma-tous panuveitis which usually occurs two weeks8 ormore after accidental or iatrogenic injury. Theinjured eye appears irritated and injected shortlyafter surgery, culminating in a picture of persistentbilateral uveitis characte,jsed by 'mutton fat' keraticprecipitates, iris and pupillary nodules, posterior

synechiae, and the appearance of yellow-whiteDalen-Fuchs nodules in the periphery of the fundus.Approximately one-fourth of phakic eyes enucleatedfor sympathetic ophthalmia show evidence of phaco-anaphylaxis.9 The signs and symptoms of bacterialendophthalmitis are generally present 18-72 hoursafter surgery and include increasing pain, hyper-aemia, chemosis, lid and corneal oedema, anteriorchamber and vitreous inflammation, and a decreasedor absent retinal reflex.

If phacoanaphylaxis is left untreated, an unremit-ting downhill course of chronic inflammation mayensue, ultimately leading to synechiae, cyclitic mem-

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brane, retinal detachment, and phthisis bulbi. In anycase of extracapsular cataract extraction complicatedby a granulomatous intraocular inflammation thediagnosis of phacoanaphylaxis should be seriouslyconsidered. The ability to differentiate phacoana-phylaxis from phacolytic glaucoma,"' sympatheticophthalmia," or endophthalmitis has importanttherapeutic implications.The present report is to the best of our knowledge

the first case occurring after an intraocular lensimplant. Since the majority of these procedures are atpresent performed with extracapsular extractions,lens cortex may be left in the eye. The occurrence ofphacoanaphylaxis is therefore not surprising andmust be considered in this type of surgery.

References

1 Straub M. On inflammation of the eye caused by resorption ofcrystalline lens matter in eye lymph. Amsterdam: de Bussy, 1919.

2 Verhoeff FH, Lemoine AN. Endophthalmitis phacoanaphylac-tica. AmJ Ophthalmol 1922; 5: 737-45.

3 Riise P. Endophthalmitis phacoanaphylactica. AmJ Ophthalmol1965; 60: 911-5.

4 Marak GE, Font RA, Alepa P. T-cell function in lens-inducedendophthalmitis. XXIII Concilium Ophthalmologicum Acta,Part II. Kyoto, 1978: 950.

5 Marak GE, Font RA, Weigle WO. Pathogenesis of lens-inducedendophthalmitis. In: Silverstein AM, O'Connor GR, eds.Immunology and immunopathology of the eye. Paris: Masson,1979: 135.

6 Schlaegel TF, O'Connor GR. Hypersensitivity uveitis. In:Duane TD, ed. Clinical ophthalmology. Philadelphia: Harperand Row, 1982: 45.

7 Hogan MJ, Zimmerman LE. Ophthalmic pathology. 2nd ed.Philadelphia: Saunders, 1962: 155.

8 Lubin JR, Albert DM, Weinstein M. Sixty-five years of sympa-thetic ophthalmia: a clinicopathologic review of 105 cases (1913-1978). Ophthalmology (Rochester) 1980; 87: 109-21.

9 Blodi FC. Sympathetic uveitis as an allergic phenomenon.Ophthalmology (Rochester) 1959; 63: 642.

10 Jaffe NS. Cataract surgery and its complications. St Louis:Mosby, 1981.

11 Easom HA, Zimmerman LE. Sympathetic ophthalmia andbilateral phacoanaphylaxis. Arch Ophthalmol 1964; 72: 9-15.

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