Abstract
We report an atypical presentation of ocular cat scratch disease (CSD) in an 8-year-old Caucasian male who presented with localised retinal arterial vasculitis and associated retinal oedema. His history of headaches, frequent contact with a kitten and a high Bartonella henslelae titre confirmed the diagnosis of CSD. Over an 18-month follow-up period, his best corrected visual acuity in the affected eye improved from 20/30−2 to 20/25+3 without treatment; however, the affected retinal artery remained sheathed.
BACKGROUND
Cat scratch disease (CSD) is a protean disease whose ocular manifestations comprise a spectrum of ocular syndromes that have been characterised as “ill-defined and considered idiopathic.”1 Posterior segment manifestations commonly include neuroretinitis (disc oedema with macular star exudation) and retinal or choroidal white lesions.2 A wide range of other findings include panuveitis, vitreitis, arterial and venous vascular occlusion(s), peripapillary angiomatosis, vitreous haemorrhage and serous retinal detachment.1–5 While Bartonella henslelae has an affinity for vascular endothelia there is only one documented case of vasculitis from CSD to our knowledge.6 The vasculitis was diffuse, bilateral and multifocal with an associated pars planitis.7 We describe an unusual case of localised retinal arterial vasculitis with associated retinal oedema in a 8-year-old Caucasian male and his clinical course during a 18-month follow-up. We believe this is the first case report of a patient presenting with localised arterial vascular sheathing due to B henslelae.
CASE PRESENTATION
An 8-year-old Caucasian male was referred to Wake Forest University Eye Center. He had noticed a “dark spot” in the vision of his right eye and complained of headaches a couple of months previous to presentation. There was a kitten in the home that he had frequent contact with. He had no history of cat bites or scratches. He also denied fevers or lymphadenopathy. His best corrected visual acuity (BCVA) was 20/30−2 in the right eye and 20/25+2 in the left eye. There was no vitreous inflammation in the affected right eye and his optic nerve was not oedematous. His left eye was completely normal. The proximal portion of the right superior temporal artery appeared to be sheathed with oedematous retina surrounding the affected segment and its first branch (fig 1). Optical coherence tomography (OCT) reflected an oedematous segment of retina with a diffuse non-specific thickening of all retinal layers (fig 2). Fluorescein angiography demonstrated hyperfluorescence/clouding of the affected segment of artery, but no obstruction (fig 3). The adjacent edge of the optic nerve stained late (fig 4).
Figure 1.
Colour fundus photo of the right eye at the initial presentation demonstrates segmental sheathing of the proximal portion of the superior temporal artery. There is “cloudy” oedema of the surrounding retina also visible. The red line through the photograph demonstrates the orientation of the subsequent optical coherence tomography image.
Figure 2.
Corresponding 10 mm optical coherence tomography scan with proportional process. The orientation of the scan is shown with the red line in photo 1 obliquely through the affected area. The scan shows thickened retina and a suggestion of sensory fluid.
Figure 3.
Corresponding fluorescein angiogram in the arteriovenous phase showing affected segment of the right superior temporal artery to be patent; laminar flow in the superior temporal vein is still visible.
Figure 4.
Recirculation phase of the fluorescein angiogram suggests staining of the vessel wall and the edge of the optic disc.
INVESTIGATIONS
A variety of lab values were normal or negative, including rheumatoid factor, herpes simplex virus 1 and 2, toxoplasmosis IgM and IgG, acetylneuraminic acid, rubella, lupus, protein C, c-reactive protein, homocysteine, lyme titre, toxocara titre, microhaemagglutination treponema pallidum and rapid plasma reagin. His B henslelae IgG titre was 1:256 and his human leucocyte antigen (HLA)-B27 was positive.
OUTCOME AND FOLLOW-UP
His clinical presentation changed little over succeeding months. Eighteen months later, his BCVA in the right eye improved to 20/25+3 and his retinal oedema appeared less by slit lamp exam, but his OCT consistently reflected some retinal thickening. The proximal segment of artery remained sheathed (fig 5). His second B henslelae IgG titre remained at 1:256, and then 5 months later the titre decreased to 1:64.
Figure 5.
Colour fundus photo of the right eye 18 months after initial presentation. Vision is improved to 20/25+3 and no specific treatment was given.
DISCUSSION
The incidence of CSD has been estimated at 9.3/100 000 population with 80% of cases occurring in children.3 Our case appears to be an unusual presentation because, while arterial and venous vascular occlusions have been reported as posterior segment complications of CSD, there has only been one reported case of vasculitis due to CSD.5,7 The previously reported case showed bilateral, diffuse, multifocal involvement with a pars planitis. We have been unable to find a case in the literature of localised retinal vasculitis similar to our patient where a small segment of one vessel was sheathed.
The absence of significant macular oedema, the limited segment of arterial sheathing and small degree of visual affectation militated against treating the patient with systemic antibiotics, which have proven complications. Antibiotics have not been shown to provide a benefit in treating ocular CSD of the posterior segment in immunocompetent patients. In their case series of 19 untreated eyes and 16 treated eyes, Solley et al concluded that “there appeared to be no difference in the final visual acuity in treated patients compared to untreated patients.” Immunocompetent patients can recover excellent visual acuity without antibiotic treatment as did our patient.2
The persistence of arterial vascular sheathing and retinal thickening, documented by both fundus photography and OCT after a full 18 months of follow-up, are possibly unique features of this case, but the absence of similar reported cases makes comparison impossible.
Finally, while the positive B henslelae antibodies (IgG) with the clinical history are persuasive in the diagnosis of CSD in this case, the relevance of the positive HLA-B27 remains obscure.
LEARNING POINTS
Ocular cat scratch disease can present with localised retinal arterial vasculitis and associated retinal oedema.
Immunocompetent patients with ocular cat scratch disease of the posterior segment can recover excellent visual acuity without antibiotics as treatment has not been shown to provide a clinical benefit in these patients.
Footnotes
Competing interests: none.
Patient consent: Patient/guardian consent was obtained for publication.
REFERENCES
- 1.Ormerod LD, Dailey JP. Ocular manifestations of cat-scratch disease. Curr Opin Ophthalmol 1999; 10: 209–16 [DOI] [PubMed] [Google Scholar]
- 2.Solley WA, Martin DF, Newman NJ, et al. Cat scratch disease: posterior segment manifestations. Ophthalmology 1999; 106: 1546–53 [DOI] [PubMed] [Google Scholar]
- 3.Ormerod LD, Skolnick KA, Menosky MM, et al. Retinal and choroidal manifestations of cat-scratch disease. Ophthalmology 1998; 105: 1024–31 [DOI] [PubMed] [Google Scholar]
- 4.Khurana RN, Albini T, Green RL, et al. Bartonella henselae infection presenting as a unilateral panuveitis simulating Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol 2004; 138: 1063–5 [DOI] [PubMed] [Google Scholar]
- 5.Cohen SM, Davis JL, Gass DM. Branch retinal arterial occlusions in multifocal retinitis with optic nerve edema. Arch Ophthalmol 1995; 113: 1271–6 [DOI] [PubMed] [Google Scholar]
- 6.Schwartzman W. Bartonella (Rochalimaea) infections: beyond cat scratch. Annu Rev Med 1996; 47: 355–64 [DOI] [PubMed] [Google Scholar]
- 7.Soheilian M, Markomichelakis N, Foster CS. Intermediate uveitis and retinal vasculitis as manifestations of cat scratch disease. Am J Ophthalmol 1996; 122: 582–4 [DOI] [PubMed] [Google Scholar]