CAT SCRATCH DISEASE (CSD) IN PATIENTS WITH STELLATE NEURORETINITIS: 3 CASES. DE SCHRYVER I.* STEVENS A.M.*, VEREECKE G.*, KESTELYN PH.* valence dans la population générale. La maladie des SUMMARY griffes du chat connait une évolution bénigne chez This case series describes three patients with a simi- les patients immunocompétents. Néanmoins, une lar clinical picture: unilateral abrupt visual loss, op- cure d antibiotiques sous forme de doxycycline est tic nerve edema, and a macular star exudate. In all conseillée. cases we found significant antibody titers to Bar- tonella henselae, the causative agent of cat scratch KEY- WORDS disease. Cat scratch disease seems to be the most common cause of stellate neuroretinitis, formerly Cat scratch disease- neuroretinitis- B. known as Leber s idiopathic stellate retinopathy. A henselae- Leber s stellate retinopathy- review of the pertinent literature shows that sero- macular star logic evidence of B. henselae is sufficient to con- firm the diagnosis given the low incidence of signi- MOTS-CLÉS ficant titers in the general population. Cat scratch disease is usually a self limiting disorder in immu- Maladie des griffes du chat- neurorétinite- B. nocompetent patients, but treatment with doxycy- henselae- rétinite stellaire pseudo- cline is recommended. néphritique de Leber- exsudat maculaire en étoile RÉSUMÉ Nous décrivons une série de trois patients qui pré- sentent un tableau clinique similaire: une perte bru- tale de l acuité visuelle dans un oeil, un oedÅme pa- pillaire et des exsudats en étoile autour de la macu- la. Chez ces trois patients la sérologie met en évi- dence un taux élevé d anticorps contre Bartonella henselae, l agent étiologique de la maladie des grif- fes du chat. La maladie des griffes du chat semble Ä™tre la cause la plus fréquente de la neurorétinite stellaire, connue sous le nom de rétinite stellaire pseudonéphritique de Leber dans l ancienne littéra- ture. Une revue de la littérature apprend que la sé- ropositivité pour B. henselae suffit en général pour affirmer le diagnostic, étant donné la faible séropré- zzzzzz * Department of Ophthalmology, University Hospital Ghent, Belgium received: 25.07.02 accepted: 28.09.02 Bull. Soc. belge Ophtalmol., 286, 41-46, 2002. 41 INTRODUCTION Cat scratch disease (CSD) is a subacute, self- limiting infection caused by the gram-negative bacillus Bartonella henselae (figure 1). Most patients develop a mild to moderately severe flu-like illness associated with regional lym- phadenopathy. Ocular involvement occurs in only 5% to 10% of patients with CSD and in- cludes Parinaud s oculoglandular syndrome, neuroretinitis, and focal retinochoroiditis. A re- view of the literature indicates a prevalence of neuroretinitis in documented CSD between 1 and 2% (6,20), although one series reported a prevalence as high as 26% (24). Encephalitis, osteomyelitis, and hepatosplenic disease are rare complications of CSD. We report 3 cases of neuroretinitis with a posi- Fig 1. Gram-negative bacilli Bartonella henselae in a vis- tive serology for B. henselae. ceral lymph node.Photograph taken by Dr. C. Van Den Broecke, Department of Anatomopathology, University Hos- pital Ghent. PATIENTS AND RESULTS examination of the right eye revealed no par- Case 1 ticularities. Examination of the left eye showed A 50-year old caucasian woman presented with disc edema, especially in the superior segment, sudden visual loss in the left eye. She reported with minimal macular edema (fig. 2a). Visual a febrile illness with mild headaches and my- field testing revealed a mild left central scoto- algia, 2 weeks prior to the visual loss. She owned ma. She was not given treatment initially and a hostel for lost animals. on examination 4 days later the visual acuity Physical examination revealed an erythema- in her left eye had improved to 20/30. Oph- tous papule at the left hand and a tender lymph thalmoscopy of the left eye now showed disc node in the left elbow. edema surrounded by flame-shaped hemor- Best corrected visual acuity was 20/20 in the rhages and a macular star pattern of lipid exu- right eye and 20/70 in the left eye. Biomicros- dates. Additionally, there was a white retinal fo- copy was unremarkable in both eyes. She had cus superotemporal to the fovea (Fig. 2b). a left relative afferent pupillary defect. Fundus Fig 2b. Disc edema with flame-shaped hemorrhages, ma- Fig 2a. Severe disc edema with minimal macular edema and a retinal focus superotemporal to the fovea. cular star exudates and a retinal focus superotemporal to the fovea. 42 Fig 3. Staining of the optic disc and the superotemporal Fig 4. Disc edema with flame-shaped hemorrhages, ve- focus on fluorescein angiography. nous dilation and macular star exudates. Note the retinal focus superotemporal to the disc. Fluorescein angiography of the left eye de- monstrated staining of the optic disc (especially left eye was normal. Examination one week lat- superiorly) and of the superotemporal focus er showed a similar clinical picture with mac- (Fig. 3). Color vision, contrastsensitivity and ular star exudates (Fig. 4). A general examina- electroretinography of both eyes were within tion revealed no scratch or papule, nor any lym- normal limits. Visually evoked potentials of the phadenopathy. Basic investigations including a left eye showed slightly increased latencies. complete blood examination, chest X-ray, ab- Several basic investigations were all found to dominal ultrasound and neuroimaging showed be normal: a complete blood examination, chest no abnormalities. Serology for B. henselae was X-ray, abdominal ultrasound, angiotensin-con- positive at a dilution of 1/512. The patient was verting enzyme level, neurological examina- treated with doxycycline 100 mg/day for 6 tion including lumbar puncture and neuroim- weeks. At a follow-up visit one month later the aging. The erythrocyte sedimentation rate was fundus findings were essentially unchanged but slightly elevated. Serologic tests for Lyme dis- the visual acuity in the right eye was restored ease, syphilis and toxoplasmosis were nega- to 20/20. tive. Serological indirect immunofluorescence tests detected antibodies to B. henselae at a Case 3 dilution of 1/256, confirming the diagnosis of neuroretinitis caused by CSD. She was treated A 42-year old caucasian man complained of with oral ciprofloxacin 1g/day for 2 weeks. sudden blurred vision in the right eye. He men- Follow-up 2 months later showed complete res- tioned a recent episode of malaise and had a olution of the fundus lesions and recovery of the cat at home. visual acuity in the left eye to 20/20. Visual acuity was counting fingers in the right eye and 20/20 in the left eye. No afferent pu- pillary defect was detected. Slit-lamp exami- Case 2 nation revealed no inflammation. Fundus ex- An 18-year old caucasian man was referred to amination of the right eye showed disc edema our department for sudden visual loss in the with a focal lesion temporal to the disc and a right eye. He reported no viral prodrome and peripapillary serous retinal detachment (Fig. 5). had no cats. Best corrected visual acuity was Examination of the left eye was normal. 20/70 in the right eye and 20/20 in the left eye. Fluorescein angiography demonstrated late leak- Biomicroscopy was unremarkable in both eyes. age from the optic nerve and the temporal fo- An afferent pupillary defect was present in the cus. Serologic testing was negative for Lyme right eye. Fundus examination of the right eye disease and toxoplasmosis. Bartonella IgM ti- revealed disc edema surrounded by retinal he- ters were positive at a dilution of 1/128. The morrhages, venous dilation and a focal lesion patient was treated with doxycycline 100mg/ superotemporal to the disc. The fundus of the day for two weeks. Follow-up 1 month later 43 id fly and ticks are potential vectors in the trans- mission of the Bartonella species (10). B. henselae seropositivity in cats varies de- pending on the geographic region and the cli- mate conditions (15). The frequency of infec- tion in cats seems to be higher in regions with more fleas (7). Typically, a scratch by a cat, especially a kit- ten, is followed after a variable incubation pe- riod of three to ten days by unilateral regional lymphadenopathy. An erythematous papule or pustule may be present at the site of inocula- tion. The infection may be asymptomatic or Fig 5. Disc edema, peripapillary serous retinal detach- cause a systemic illness with fever, malaise, ment and a macular star. Note again the retinal focus tem- night sweats and painful regional lymphadeno- poral to the disc.- pathy. Occasionally CSD may cause a more se- showed formation of a macular star with re- vere picture with involvement of the central and gression of the disc edema and the temporal peripheral nervous system, liver, spleen, lung, focus. Two months after the initial visit the vi- bone, skin and eye (18). sual acuity of the right eye had improved to 20/ The eye is the most commonly affected non- 20. The macular star, still present at that time, lymphatic organ and many ophthalmic mani- faded away over the next month. festations of CSD have been reported: neurore- tinitis, chorioretinitis, serous detachment of the macula, optic neuritis, anterior and posterior DISCUSSION uveitis and Parinaud s oculoglandular syndrome CSD is a self-limited infectious disease that pri- (2). The most common association appears to marily affects the lymphatic system. It appears be the oculoglandular syndrome of Parinaud, to affect patients of any age with a higher pre- affecting approximately 5% of symptomatic valence among youngsters and without a pre- patients and characterized by fever, granulo- dilection for either sex. Domestic cats are a ma- matous conjunctivitis and regional lymphade- jor reservoir for B. henselae, the causative agent nopathy (2). of CSD. This agent was first described by Wear Neuroretinitis, formerly known as Leber s (id- and coworkers in 1983 (22). They identified a iopathic) stellate retinopathy, is characterized pleomorphic gram-negative bacillus in a lymph by unilateral acute visual loss, optic disc ede- node from a patient with CSD using the Whar- ma, lipid exudation in the macula arranged in tin-Starry silver method. In 1990 Relman and a star configuration and one or more foci of coworkers identified a new pathogen as a cause chorioretinitis (8). of bacteremia, bacillary angiomatosis and ba- Gass demonstrated in 1977 that the tissue pri- cillary splenitis: Rochalimaea henselae (13). mary involved appears to be the optic nerve This previously unknown organism was close- head vasculature. Abnormal permeability of the ly related to the causative agent of trench fe- small capillaries on the optic nerve results in ver, at that time named Rochalimaea quin- leakage of lipid-rich exudate in the subretinal tana and it was suggested that it be named space. He hypothesized that the macular star Rochalimaea henselae (16,23). is due to reabsorption of the serous compo- Brenner and coworkers revealed that the Roch- nent of this exudate leaving lipid precipitates alimaea henselae species were closely related in the outer plexiform layer of the macular re- to Bartonella bacilliformis, the agent of oroya gion. These observations led him to the con- fever and verruga peruana in Peru and Ecua- clusion that this clinical entity should prefer- dor (1). A new reclassification in 1993 changed entially be called neuroretinitis rather than a re- the genus Rochalimaea henselae in the Bar- tinopathy or a maculopathy (5). tonella species. Studies suggested that not only Neuroretinitis secondary to CSD is typically uni- fleas, but also the human body louse, the sar- lateral, although bilateral cases have been de- 44 scribed (21). Generally, patients present with the percentage of neuroretinitis caused by CSD, abrupt unilateral visual loss. Most patients have based upon a positive serology. They found a features of optic neuropathy. seropositivity for Bartonella henselae in 65.3% Anterior chamber cells and flare may be present of tested patients with neuroretinitis (19). This and posterior vitreous cells are common. All pa- result is much higher than the 3% incidence in tients have optic edema and a peripapillary se- the general population, found by Regnery and rous detachment. Nerve fiber layer splinter he- coworkers, implying that CSD is one, if not the morrhages are ocasionally seen. After 1 to 2 single most important cause of neuroretinitis. weeks when the serous fluid resolves, a mac- The optimal treatment for CSD is unknown due ular star begins to precipitate. These lipid exu- to a lack of adequate clinical trials. B. hense- dates disappear after a few to several months lae seems to be sensitive in vitro to a number and may leave retinal epithelial pigment de- of antibiotics: erythromycin, doxycycline, cipro- fects behind. floxacin, rifampicin and trimethoprim-sul- Foci of retinitis or choroiditis have been de- famethoxazole. Doxycycline (100mg orally twice scribed (17). They can be multiple or isolated. daily) is preferred: it has better ocular and cen- Our three patients presented with an isolated tral nervous system penetration. It is usually focus in association with disc edema and a ma- given 2 to 4 weeks in immunocompetent pa- cular star. In two cases the focus appeared peri- tients and 4 months in immunocompromised papillary. Complications of focal chorioretinitis patients (9). The benefit of antibiotic therapy include branch retinal artery (3,17) and vein in immunocompetent patients with CSD has occlusions (3), and localized serous detach- never been demonstrated and before the iden- ment (25). tification of B. henselae, most immunocompe- Several other infectious diseases may produce tent patients tended to do well without any treat- a similar clinical picture and should be ruled ment. out: syphilis, Lime disease, leptospirosis, toxo- plasmosis, toxocariosis and tuberculosis. 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