Britishournal ofOphthalmology, 1990,74,325-327 325

ORIGINAL ARTICLES Br J Ophthalmol: first published as 10.1136/bjo.74.6.325 on 1 June 1990. Downloaded from

Ocular : case report and review ofthe literature

Danny J H Kauffmann, Gary P Wormser

Abstract she was admitted to a community hospital. Lyme disease is an emerging new spirochaetal A physician removed fragments ofa presumed disease in which ocular complications may insect stringer from her right foot accompanied arise. We have seen a 45-year-old woman who byawhitemucoid discharge. Herwhite bloodcell developed unilateral endophthalmitis leading count was 22 x 109/l with 73% polymorphonuclear to blindness during the course of this disease. leucocytes and 18% band forms, and the erythro- Ocular tissue showed the characteristic cyte sedimentation rate was 48 mm/h. She was spirochete. A literature review shows that the treated empirically with intravenous cefazolin, commonest ocular manifestation of Lyme 6 g a day for seven days, but continued to have disease is a mild conjunctivitis, but other daily fevers up to 40- 10C (104-20F). She was then symptoms may include periorbital oedema, changed to oral tetracycline, 500 mg four times a oculomotor palsies, uveitis, papilloedema, day for seven more days. She responded well to papillitis, interstitial , and others. this regimen. Ophthalmologists treating patients from Two weeks later, on 25 August 1982, a sting on Lyme disease endemic areas need to be aware the left thigh by a bee caused localerythema. Two of the protean clinical manifestation of this days afterwards she awoke with a painful red left disease. eye. She had no eye complaints previously. The patient treated herself with sulphafurazole (sul- fisoxazone)drops, but sought an ophthalmologist Lyme disease, a tick-borne spirochaetal infec- when the vision decreased in the left eye and

tion,' often begins with a characteristic rash, periorbital oedema developed. She was noted to http://bjo.bmj.com/ erythema chronicum migrans.3 Ocular manifes- haveiritisandposteriorsynechiaeandwas treated tations have been infrequently reported, but, as with topical atropine 1% and Predforte 1% the disease is increasing in frequency and occur- (dexamethasone) drops, with resultant breaking ring in new geographical areas,4 more cases with of the synechiae. However, the iritis persisted, ocular involvement can be expected. We report in and despite subconjunctival injection oftriamci- detail the eye findings in a patient previously nolone 40 mg hypopyon developed along with

reported on who developed severe, unilateral vitritis. Oral prednisone 60 mg per day was on September 29, 2021 by guest. Protected copyright. endophthalmitis five weeks after erythema begun. The visual acuity nevertheless worsened, chronicum migrans.' Spirochaetes were found in and the prednisone dose was increased to 100 mg the vitreous in specimens obtained at surgery. In daily. She was then transferred to Westchester addition we review the literature ofthe ocular and County Medical Center. neuro-ophthalmic manifestations of Lyme Her visual acuity was 20/20 right eye and hand disease. motions left eye. The results of ocular examina- tion including tension were normal in the right eye. Theleft eye showedmild conjunctival ecchy- Case report mosis and ciliary vessel injection. There was mild On 15 July 1982 a 45-year-old woman from West- periorbital oedema. The ocular rotations were chester County, New York, developed severe full, though splinting ofthe left eye was noted in headache, light headedness, chills, and fever. extreme fields ofgaze. There was no measurable Three days later she also had nausea and vomit- proptosis. The showed microscopic epi- New York Medical ing. On 21 July she noted red lesions on her right thelial oedema with stippled fluorescein College, Valhalla, New staining. York 10595, USA foot and upper arms that looked like insect bites. There was a severe inflammatory reaction in the Department of In addition a large annular erythema with an anterior chamber, with 3+ flare and cells, and a Ophthalmology indurated centre was noted on the right thigh. 20% hypopyon was present. After dilatation of D J H Kauffmann This lesion expanded and developed a bright red the pupil pigment residua were noted on the Division of Infectious outer border with central clearing. She remem- anterior lens surface from the previously treated Diseases, Department of bered having been bitten by many flying insects posteriorsynechiae. Thelenswasotherwiseclear. Medicine during the 4 no G P Wormser July weekend but recalled tick The ocular tension was 18 mmHg. The left bites. She was treated with diphenhydramine Correspondence to: D J H fundus could not be seen, and no red reflex was Kauffmann, MD, 1 Stone hydrochloride 50 mg four times a day and a obtained throughthe cellulardebris in the media. Place, Bronxville, New York cortisone skin ointment for what was to 10708, USA. thought Ophthalmic A and B scan ultrasound revealed be a local skin allergy to insect bites. On 30 July, Accepted for publication diffuse debris in the vitreous cavity, with marked 9 January 1990 because ofdaily fevers, malaise, and skin lesions, thickening of the choroid consistent with a 326 Kauffmann, Wormser

TABLE I Ophthalmic and severe panophthalmitis. The retina was attached. which continuously maintained 20/20 vision neuro-ophthalmic The initial impression was severe uveitis ofun- throughout the course ofher disease.

manifestations ofLyme Br J Ophthalmol: first published as 10.1136/bjo.74.6.325 on 1 June 1990. Downloaded from disease known aetiology. Prednisone was continued at 100 mg daily and tapered to 60 mg over the next Optic neuritis Discussion Optic perineuritis seven days. Roentgenograms of the chest, skull, Ischaemic optic neuropathy sinuses, orbits, and sacroiliac joints were normal. Our patient presented with a uniocular uveitis Optic atrophy due to Lyme disease which to a Cranial nerve palsies Serological tests for (FTA-abs, VDRL), progressed Argyll-Robertson pupil antinuclear antibodies, angiotensin converting panendophthalmitis and ultimate destruction of Pupillary areflexia toxo- vision.5 Other of Homer's syndrome enzyme, cryoglobulins, rheumatoid factor, useful uniocular examples Lyme Cortical blindness cara, toxoplasmosis, histoplasmosis, and cyto- related uveal tract involvement include case Papilloedema tuberculin reports of patients with and Conjunctivitis megalovirus were negative. A 5 TU iritis,7 uveitis,89 Keratitis skin test was negative. Serum creatinine and liver choroiditis with exudative retinal detachment.'0 Iritis on 17 The visual outcome of the other reported cases Iridocyclitis enzymeswerenormal. Serumobtained Sep- Panuveitis tember 1982 showed an IgM antibody titre to the was much better. A patient with iritis had spon- Vitritis an titreof 1:512 taneous resolution, and visual acuity in the others Choroiditis Lyme spirochaete of1 :64and IgG Exudative retinal detachments (normal for both < 1:64) determined by indirect improved after the use of specific systemic anti- Retinal vasculitis immunofluorescence. biotics with8 or without9'0 concomitant ocular Retinal haemorrhages Periorbital oedema Eightdaysafteradmission tohospitalandwhile steroids. on prednisone 60mgdaily the patient developed a Additional ocular manifestations of Lyme rapid rise in intraocular pressure in the left eye to disease include conjunctivitis, photophobia, 40 mmHg along with 5 mm proptosis, a conjunc- periorbital oedema, cranial nerve palsies, kera- tival purulent discharge, and the rapid onset of a titis, pupillary abnormalities, papilloedema, dense cataract. Computerised tomography optic neuritis, and optic atrophy (Table I).3 9 11-24 showed no evidence of a lesion to explain the The commonest ocular finding in Lyme disease proptosis. Orbital cellulitis was suspected, and appears to be conjunctivitis. Steere etalin a study nafcillin 12 g/day plus gentamicin 80 mg every of314patientswitherythemachronicum migrans eight hours was given intravenously along with noted conjunctivitis in 35 (11%), photophobia in topical neomycin-polymixin B (Neosporin) and 19 (6%), and periorbital oedema in 10 (3%).3 Un- dexamethasone. The ocular tension was control- fortunately the authors did not elaborate further. ledwithacetazolamide 1 g orally daily and timolol By inference and from our anecdotal experience 0-5% topically. Conjunctival cultures were nega- conjunctivitis isnotveryprominentinthis disease tive for bacteria, fungi, and mycobacteria. and is short-lived. One week later, 19 days after admission, a len- Neurological complications in Lyme disease sectomy and vitrectomy were performed and occur in approximately 10% of cases, and over purulentvitreousmaterial was removed. Intravit- halfofpatients with them have cranial nerve pal- real gentamicin 0-2 mg in 0-1 ml and chloram- sies, most commonly involving the seventh phenicol 0-2 in 0-1 ml were injected and nerve.'2 Oculomotor weakness is infrequent, but

[tg http://bjo.bmj.com/ systemic were maintained, while sys- may be due to cranial nerve palsy ofthe III, IV, or temic steroids were tapered. Cultures and smears VI cranial nerves. Such palsies may occur indivi- for bacteria, fungi, and acid fast organisms were dually, orin combinationwith one anotherand/or negative, and cytological examination of a fresh with other neurological abnormalities. 12 14 Diplo- vitreous specimen revealed only white cells. One pia is the typical ocular complaint in these in- weeklater a second vitrectomy to remove increas- stances. Like other neuropathies, oculomotor ing amounts of vitreous debris was performed. weakness usually resolves within two weeks to Examination ofthe material by dark field micro- five months after onset.'2 14 Whether antibiotics on September 29, 2021 by guest. Protected copyright. scopy was negative for spirochaetes. improve the rate of recovery has not been Pathological examination of the lens and vit- established.25 One patient with an Argyll-Robert- reous contents showed lens fragments and nec- son pupil'2 and another with a reversible Homer's roticcellswithaheavyinfiltrationofmononuclear syndrome26 have been reported on, and optic cells. Later the eye tissue was stained by the atrophy has been noted in several patients (see Dieterle method.6 Despite the advanced cell nec- below).9 12 27 28 rosis, occasional intact spirochaetes, morphologi- Papilloedema of diverse causes has been seen callycompatible withthe Lyme spirochaete, were occasionally.'2 162022 It has been observed in a demonstrated in vitreous material. minority of Lyme disease patients with menin- After surgery the patient continued to have gitis. 14 22 It is then typically bilateral and may be mucous secretions from the eye and experienced symptomatic, leading to complaints of blurred occasional myalgias and arthralgias. A good red vision. It is of interest that the intracranial reflex was obtained, vision returned to hand pressure isoften not raised, so that the pathogene- motions, and on 13 October she was discharged sis of this condition is obscure. Too few data are from hospital. However, over the ensuing three available to assess the natural history ofpapilloe- months she developed a dense cyclitic membrane dema in Lyme disease meningitis, but in her eye and lost all functional visual activity, therapyappears to hasten the resolution ofcertain with the eye becoming phthisical. On 28 October other clinical signs.2 In one report resolution of IgM antibody to the spirochaete was no longer papilloedema was accompanied by the develop- detectable, and the titre of specific IgG had ment ofpigment epithelial mottling at the fovea,'6 decreased to 1:64. By May 1983 neither specific and in two other cases optic atrophy ensued. 12 IgM not IgG antibody was found. After one year Papilloedema has also been reported in several the patient was given a cosmetic contact lens shell Lyme disease patients who had increased intra- covering the phthisical left eye. She developed no cranial pressure but no evidence of meningitis.'5 manifestation of Lyme disease in her right eye, Sixth nerve palsy was sometimes present in these OcularLyme disease: case reportand review ofthe literature 327

patients. Cranial tomography failed to reveal worsen the condition.3 Similarly, steroids may mass or in a lesions enlarged ventricles resulting have contributed to the marked severity of the Br J Ophthalmol: first published as 10.1136/bjo.74.6.325 on 1 June 1990. Downloaded from diagnosis of Lyme disease-associated pseudo- ocular infection in our patient. tumor cerebri for some of these patients.'5 Anti- Lyme disease is an emerging infectious disease biotics plus additional medications for reducing which may cause eye lesions requiring treatment. intracranial pressure have been useful thera- Ophthalmologists caring for patients from ende- peutically. mic areas need to be aware of its protean clinical Anecdotal cases of optic neuritis'420 and optic manifestations. atrophy92728 possibly due to Lyme disease have been et The authors thank Mrs Shirley M Gamble and Ms Susan reported. Bertuch al'8 described a patient Friedlander for their valuable assistance in preparing this manu- who developed progressive temporal pallor ofthe script and Dr Jorge Benach for performing the Lyme serological right optic disc six months after onset ofan illness tests. presumed to be Lyme disease. In addition Shech- 1 Steere AC, Grodzicki RL, Kornblatt AN, etal. The spirochetal ter'7 described a patient who developed papillitis etiology of Lyme disease. N EnglJ Med 1983; 308: 733-40. 2 Steere AC, Malawista SE, Hardin JA, Ruddy S, Askenase PW, of the right eye with an inferior altitudinal visual Andiman WA. Erythema chronicum migrans and Lyme defect three months after the onset of Lyme arthritis: the enlarging clinical spectrum. Ann Intern Med 1977; 86:685-98. disease. Despite a course of high dose systemic 3 Steere AC, Bartenhagen NH, Craft JE, et al. The early clinical corticosteroids, the left eye ofthis patient also be- manifestations of Lyme disease. Ann Intern Med 1983; 99: 76-2. came involved. The process eventually stabilised 4 SchmidGP. Theglobaldistribution ofLymedisease. RevInfect after a course of antibiotics plus corticosteroids. Dis 1985; 7: 41-50. S Steere AC, Duray P, Kauffmann DJH, Wormser G. Unilateral The author's diagnosis in this latter case was blindness caused by infection with the Lyme disease spiro- ischaemic optic neuropathy due to Lyme disease. chete, Borrelia burgdorferi. Ann InternMed 1985; 103: 382-4. 6 Van Orden AE, Greer PW. Modification of the Dieterle spiro- Other late ophthalmic manifestations of Lyme chete stain.JHistotech 1977; 1: 51-3. disease have been described. Corneal abnormali- 7 Eichenfield AH, Goldsmith DP, Benach JL, et al. Childhood Lyme arthritis: experience in an endemic area. J Pediatr ties presumed to be due to Lyme disease have 1986; 109:753-8. been described in four patients who developed a 8 Wong T. The onset of bilateral uveitis in an elderly man with fever, headache,andarash. OphthalmicSurg 1989; 20: 154-6. form of interstitial keratitis six months to as 9 Winward KE, Smith JL. Ocular disease in Caribbean patients long as five years after the diagnosis of Lyme with serologic evidence of Lyme borreliosis. J7 Clin Neuro Ophthalmol 1989; 9: 65-70. disease.'8192' None of these patients had other 10 Bialasiewicz AA, Ruprecht KW, Naumann GOH, Blenk H. symptoms attributable to Lyme disease when the Bilateral diffuse choroiditis and exudative retinal detach- ments withevidenceofLymedisease. AmJ Ophthalmol 1988; ocular disease was demonstrated, and all four had 105: 419-20. previously received antibiotic therapy for Lyme 11 Pachner AR, Steere AC. The triad ofneurologic abnormalities disease. The keratitis was of Lyme disease: meningitis, cranial neuritis, and radi- characterised by scat- culoneuritis. Neurology 1985; 35: 47-53. tered corneal opacities that occurred randomly 12 Reik L, Burgdorfer W, Donaldson JO. Neurologic abnormali- ties in Lyme disease without erythema chronicum migrans. within the corneal stroma from Bowman's mem- AmJMed 1986; 81: 73-7. brane to Descemet's membrane, either unilater- 13 Ackermann R, Horstrup P, Shmidt R. Tick-borne meningo- polyneuritis (Garin-Bujadoux, Bannwarth). Yalek BiolMed http://bjo.bmj.com/ ally or bilaterally. Bilateral involvement of the 1984; 57:485-90. cornea was noted in one case in which the corneal 14 ReikL,SteereAC,BartenhagenNH,ShopeRE,Malawista SE. Neurologic abnormalities of Lyme disease. Medicine 1979; stroma was involved asymmetrically.'9 In one 58:281-94. patient minimal neovasculatisation was seen.'9 15 Raucher HS, Kaufman DM, Goldfarb J, Jacobson RI, Rose- Two patients man B, WolffRR. Pseudotumor cerebri and Lyme disease: a were asymptomatic, one of whom new solution. Pediatr 1985; 107: 931-3. was found to have diminished visual acuity on 16 WuG, LincoffH, Ellsworth RM, Haik B. Opticdiscedemaand routine school screening; the Lyme disease. Ann Ophihalmol 1986; 18: 252-5. other two patients 17 Shechter SL. Lyme disease associated with optic neuropathy. on September 29, 2021 by guest. Protected copyright. complained of decreased vision. The keratitis AmJMed 1986; 81:143-5. responded 21 18 Bertuch WA, Rocco E, Schwartz EG. Eye findings in Lyme to topical corticosteroids'9 but disease. Conn Med 1987; 51: 151-2. apparently not to systemic or tropical anti- 19 Baum L, BarzaM, Weinstein P, Groden J, Aswad M. Bilateral biotics,'8 keratitisasamanifestationofLymedisease. AmJ Ophthalmol which supports the conjecture that this 1988; 105: 75-7. process has an immunological rather than an 20 Farris BK, Webb RM. Lyme disease and optic neuritis.J Clin infectious origin. Neuro Ophthalmol 1988; 8: 73-8. 21 Orlin SE, LaufferJL. Lymediseasekeratitis. AmJ Ophthalmol Eye involvement in Lyme disease shares cer- 1989; 107:678-9. tain similarities with that seen in 22 Jacobson DM, Frens DB. Pseudotumor cerebri syndrome syphilis. Syphi- associated with Lyme disease. AmJ Ophthalmol 1989; 107: litic uveitis or iritis occurs in about 4% ofpatients 81. with secondary syphilis, but may also follow 23 AabergTM. TheexpandingophthalmologicspectrumofLyme disease. AmJ Ophthalmol 1989; 107: 77-80. inadequately treated primary syphilis or may be a 24 Markowitz LE, Steere AC. Lyme disease during pregnancy. part of tertiary Keratoiritis the JAMA 1986; 255: 3394-6. syphilis."0 is 25 Clark JR, Carlson RD, Sasaki CT, Pachner AR, Steere AC. commonest manifestation, but choroiditis, lenti- Facial paralysis in Lyme disease. Laryngoscope 1985; 95: cular opacities, retinitis, and panophthalmitis 1341-4. 26 Glauser TA, Brennan PJ, Galetta SL. Reversible Homer's may occur.30 The vitreous often shows multiple syndrome and Lyme disease. Clin Neuro Ophthalmoll989; yellow-grey exudates, which tend to spread 9:225-8. along 27 Schaltenbrand G. Durch arthropoden ubertragene Infektionen retinal vessels.3' An important differentiating der Haut und des Nervensystems. Munch Med Wochenschr feature of syphilitic keratitis from that so far 1966; 108: 1557. 28 Schaltenbrand G. Durch arthropoden ubertragene Erkran- reported in Lyme disease is the early and massive kungen des Haut und des Nervensystems. VerhDtsch GesInn neovascularisation of the cornea in 19 Med 1966; 72: 975. syphilis. 29 Steere AC, Pachner AR, Malawista SE. Neurologic abnormali- Most authors suggest treatment of ocular ties of Lyme disease: successful treatment with high dose with intravenous intravenous penicillin. Ann InternMed 1983; 99: 767-72. syphilis high-dose penicillin, 30 Moore JE. Syphilitic iritis. AmJOphthalmol 1931; 14: 110-22. sometimes accompanied by systemic cortico- 31 Ross WH, Sutton HFS. Acquired syphilitic uveitis. Arch steroids.3'32 steroids alone be Ophthalmol 1980; 98: 495-8. However, may 32 Belin MW, Baltch AL, Hay PB. Secondary syphilitic uveitis. permissive to the spirochaete and are likely to AmJ Ophthalmol 1981; 92: 210-4.