British Journal of Ophthalmology 1996; 80: 409-412

Infectious keratitis with comeal perforationassociated with comeal hydrops and contact lenswear in keratoconus

Eric D Donnenfeld, Amilia Schrier, Henry D Perry, Herbert J Ingraham,Richard Lasonde, Arthur Epstein, Bruce Farber

AbstractBackground-Corneal perforation is anuncommon complication associated withkeratoconus. The first cases of infectiouskeratitis and corneal perforation associ-ated with corneal hydrops and contactlens wear are reported in two keratoconuspatients.Methods-A retrospective chart reviewand histopathological examination werecarried out.Results-Both patients progressed tocorneal perforation and emergency pene-trating keratoplasty. One patient culturedFusarium and the second patient Serratiamarcesens. Both patients wore contactlenses against medical advice.Conclusions-The tear in Descemet'smembrane, stromal oedema, and epithe-lial bedewing associated with cornealhydrops results in loss of the epithelial-endothelial barrier ofthe cornea, creatinga conduit for infectious organismsthrough the cornea. Acute hydrops associ-ated with epithelial keratitis, stromalswelling, and a Descemet's membranetear may be a significant risk factor forinfectious keratitis and corneal perfora-tion. Contact lenses should not be wornduring an active corneal hydrops owing tothe increased risk for severe infectiouskeratitis and corneal perforation.(BrJ' Ophthalmol 1996; 80: 409-412)

Department ofOphthalmology, NorthShore UniversityHospital, Manhasset,New York and CornellUniversity MedicalCollege, New York,USAE D DonnenfeldA SchrierH D PerryR LasondeA EpsteinB Farber

Department ofOphthalmology,Geisinger MedicalCenter, Danville,Pennsylvania, USAH J Ingraham

Correspondence to:Eric Donnenfeld, MD, LionsEye Bank for Long Island atNorth Shore UniversityHospital, Manhasset, NY11030, USA.Accepted for publication19 January 1996

Infectious keratitis associated with contact lenswear is a well described entity.1-15 Risk factorsfor contact lens associated microbial keratitisinclude poor hygiene, tear film abnormalities,extended wear contact lens use, and epithelialdefects. Severe keratitis is generally associatedwith Gram negative infection2 4 5 7-10 and/or acompromised host. Corneal perforation is anextremely rare complication of contact lensrelated microbial keratitis. A large series ofcontact lens associated comeal ulcers5 foundone corneal perforation in 54 consecutivepatients in which the contact lens was worn forvisual rehabilitation.

Corneal hydrops, a rupture of Descemet'smembrane with secondary stromal and epithe-lial oedema, is a dramatic occurrence in kera-toconus.16 The symptoms are related to theacute break in Descemet's membrane withstromal and epithelial oedema. Visual acuity isacutely compromised and patients may com-plain of pain and photophobia. Resolution of

the epithelial and stromal oedema generallyoccurs over a period of months, often withconcomitant improvement in visual acuity. Wehave recently managed two keratoconuspatients with infectious keratitis who pro-gressed to corneal perforation despite aggres-sive antimicrobial therapy. Both patients gave ahistory of keratoconus with contact lens wearin the presence of an acute corneal hydrops.

MethodsA thorough retrospective chart review wasundertaken on both patients. In addition, ahistopathological examination of corneal speci-mens was performed.

Case reports

CASE 1A 51-year-old woman presented with a 1 dayhistory of acute pain and decreased vision inthe right eye. Previous ocular history was perti-nent for keratoconus, managed with a gaspermeable contact lens in the right eye. Sixweeks before presentation, the patient haddeveloped acute corneal hydrops in the righteye with epithelial and stromal oedema. Shewas managed with sodium chloride 5% dropsfour times daily, prednisolone acetate 1% fourtimes daily, and chloramphenicol 0 5% dropstwice daily. The steroids were tapered and dis-continued after 10 days; the patient wasadvised not to wear contact lenses. Four weeksbefore presentation, the patient travelled to the

Figure 1 Case 1, day 1. Photograph showing deepstromal corneal infiltrate.

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Figure 2 (Case 1, day 1. .ilit-lamp photograph showingnormal anterior corneal stroma with inflammatory cellsradiatingfrom the endothelium into the anterior chamber.

_~~~~~~~~.

.I.;

Figure 3 Case 1, day 7. Photograph showing centralcorneal infiltrate with central perforation and 15% inferiorhypopyon.

Caribbean, wore the contact lenses, experi-enced pain, and treated herself with pred-nisolone acetate 1% every 2 hours andchloramphenicol 0'5% drops four times daily.Following this, the patient wore contact lensesagain, experienced acute pain, and once again,treated herself with chloramphenicol 0-5%and prednisolone acetate 1% drops in the righteye.On presentation, ocular examination

revealed a visual acuity of 20/200 in the righteye and 20/20 in the left eye. The conjunctivawas 3+ diffusely injected in the right eye. Theepithelium was clear and intact. There was a1-5 mm endothelial plaque immediatelybeneath the tear in Descemet's membrane withinflammatory cells radiating into the anteriorchamber (Figs 1 and 2). The overlying stromawas compact and not inflamed. The intraocu-lar pressure was 16 mm Hg in both eyes. The

Figure 4 Case 1. Photomicrograph (X36). Corneal pathology revealing severe cornealthinning with inflammatory stromal infiltrates and adherent hypopyon.

lens was clear. The vitreous was clear. Theposterior pole was normal.The patient underwent anterior chamber

paracentesis with removal of the inflammatoryplaque. Gram, Giemsa, and silver stains werenegative. Topical treatment was initiated con-sisting of chloramphenicol 0-5% drops fourtimes daily and amphotericin 0 1% drops every2 hours. After 24 hours, Fusarium was identi-fied on three culture media, later speciated toFusarium solani. The patient was started onamphotericin O.4%/O drops every 15 minutes,natamycin 5% drops every hour, fluconazole100 mg orally twice daily, and doxycycline100 mg orally twice daily. The patient experi-enced increasing hypopyon formation, pro-gressive corneal thinning (Fig 3), and went onto corneal perforation on day 10 of treatment.An emergency 8 mm penetrating kerato-

plasty was performed. The hypopyon wascultured. Postoperatively the patient was main-tained on natamycin 5% drops four times dailyand fluconazole 100 mg once a day, which wastapered over a 2 month period. Prednisoloneacetate 1% drops were started on postoperativeday 5. Intraocular and corneal cultures werenegative. Histology of the cornea revealed anadherent hypopyon characteristic of fungalkeratitis (Fig 4). Silver stain demonstrated thepresence of organisms throughout the cornealstroma, despite 10 days of antifungal therapy.Six months postoperatively, following sutureremoval, the patient had an uncorrected visionof 20/25 in the right eye.

CASE 2A 36-year-old man was referred for evaluationwith complaints of decreased vision and painin the left eye of 1 day duration. Ocular historywas significant for keratoconus in both eyes,managed with rigid gas permeable contactlenses for 6 years. There was a history of acutehydrops with epithelial bedewing in the left eyefor 1 month, which was treated by the referringphysician with tobramycin 0'30/o/dexametha-sone 0-1% ophthalmic solution four timesdaily. During this time, the patient continuedto wear contact lenses against medical advice.On ocular examination, the visual acuity was

20/80 in the right eye with contact lens correc-tion, and hand motion in the left eye. The con-junctiva in the left eye was 3+ injected with amarked limbal flush. The corneal examinationrevealed a 7 mm inferior paracentral cornealulcer with 5 mm of central comeal thinning.The surrounding cornea was noted to have amarked stromal infiltrate and a significantpurulent discharge (Fig 5).Pneumotonometry of the periphery was 16.

The patient was Seidel negative. There was a50/o inferior hypopyon and a pupillary mem-brane was noted. B-scan ultrasound wasnormal. Diagnostic corneal scrapings for cul-ture and sensitivity, as well as Gram, Giemsa,and Papanicolaou stains were obtained.The patient was empirically started on cefa-

zolin drops, 100 mg/ml, and tobramycin drops,14 mg/ml, alternating every half hour. Thepatient was given one subconjunctival injection

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Infectious keratitis with corneal perforation associated with corneal hydrops and contact lens wear in keratoconus

Figure S Case 2. Cornea had marked stromal infiltrateand a significant punrlent discharge.

of tobramycin 80 mg, and cefazolin 200 mg.Serratia marcesens was cultured from the corneawithin 48 hours. Antibiotic therapy was modi-fied to tobramycin 14 mg/ml drops every hour.The hypopyon resolved, but the patient hadprogressive corneal thinning. Despite 10 daysof aggressive therapy, the patient went on to a4 mm corneal perforation.An emergency 7*5 mm penetrating kerato-

plasty was performed. The patient wasmaintained on chloramphenicol 05°/0 anddexamethasone phosphate 0-1% ophthalmicsolutions postoperatively. Pathology of thecornea adjacent to the area of thinningrevealed a marked inflammatory infiltrate andstromal extracellular fluid with a loss of normalcollagen lamellae (Fig 6). Six months post-operatively, with sutures in place, the patientcorrected to 20/30 visual acuity.

ResultsBoth patients progressed to corneal perforationdespite aggressive antimicrobial therapy. Bothpatients also required emergency penetratingkeratoplasty. One patient cultured Fusariumand the second patient Serratia marcesens. Ahistopathological examination in both eyesrevealed significant corneal thinning, consistent

Figure 6 Case 2. Photomicrograph (X36) of keratoplasty specimen adjacent to the area ofperforation, revealing inflammatory cellular infiltrate in the corneal stroma contiguous tothe area of extracellular stromal oedema.

with the diagnosis of keratoconus. In addition,there were marked inflammatory infiltratesand loss of normal collagen lamellae. In thecase associated with Fusarium viable fungalorganisms were visible on pathology andcultured, despite 10 days of aggressive anti-fungal therapy. Both patients did well withpenetrating keratoplasty and achieved goodvisual acuity with a mean follow up of 11months.

DiscussionThese two cases demonstrate contact lenswear, epithelial defects, and corticosteroids asrisk factors for infectious keratitis. Contact lenswear in the presence of an epithelial defectpermits the adherence of micro-organisms tothe lens, prolonging the contact time ofpathogens to the corneal surface.'3 Cornealswelling associated with contact lens wearalone may cause enough epithelial disruptionto precipitate corneal infection.'2 13 In the twocases presented, there was epithelial bedewingsecondary to corneal hydrops and contact lenswear, allowing an entry site for the infectiousorganisms.

Spontaneous corneal perforation associatedwith corneal hydrops in the absence of infec-tion has recently been described by Ingraham,et al.'7 Following penetrating keratoplasty forvisual rehabilitation, pathology of the involvedcornea revealed a prominent fistula. The fistulatracked directly from the tear in Descemet'smembrane through the entire stroma toBowman's membrane. This fistula could easilyact as a conduit, enabling organisms to trackinto the deep stroma and anterior chamber. Asthe infection progressed, the fistula could cer-tainly enlarge sufficiently to become an overtperforation.The normal corneal stroma is composed of

collagen fibres. The collagen fibres arearranged in lamellar sheets, which lie atoblique angles to one another. Each lamella iscontinuous across the cornea. In stromaloedema, the interlamellar spaces are widened,and irregularity of the collagen lamellaeoccurs.'8 We believe that the stromal oedemaassociated with corneal hydrops produces achange in the normal architecture of thecorneal lamellae, facilitating the passage oforganisms through the cornea. In keratoconus,there is also a decrease in the number ofstromal lamellae associated with the cornealthinning.16 Decreased number of collagenlamellae with concomitant disruption may beresponsible for the rapid penetration of infec-tious organisms through the keratoconiccornea with acute hydrops.These two cases demonstrate stromal

oedema and tears in Descemet's membrane tobe significant risk factors for corneal perfora-tion in infectious keratitis. In both patients, theulcerative keratitis and perforation occurreddirectly over the area of hydrops. The acutetear in Descemet's membrane in hydropsallows not only access offluid from the anteriorchamber into the corneal stroma, but alsocauses a loss of the barrier function of

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Descemet's membrane and endothelium. The

resultant stromal oedema produces a change inthe normal architecture of collagen lamellae,weakening the integrity of the cornea.

Infectious keratitis and corneal perforationassociated with bandage contact lens wearhave been reported in patients with bullouskeratopathy.5 19 Spontaneous corneal perfora-tion is a rare, but well documented occurrencein corneal hydrops.17 The combination ofcorneal oedema and a rupture of Descemet'smembrane creates a conduit, allowing infec-tious organisms to pass readily from the sur-

face of the eye into the anterior chamber.In conclusion, we feel contact lens wear in

the face of acute hydrops increases the risk ofsevere infectious keratitis and corneal perfora-tion.This study was supported in part by the Lions ClubInternational Foundation, Oakbrook, Illinois.

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