Ocular Involvement

Ocular involvement, which occurs in 5%–10% of patients with CSD, includes Parinaud oculoglandular syndrome (unilateral granulomatous conjunctivitis and regional lymphadenopathy) in approximately 5% of patients and a wide array of posterior segment and neuro-ophthalmic findings. Entities to be considered in the differential diagnosis of Parinaud oculoglandular syndrome include tularemia, tuberculosis, syphilis, sporotrichosis, and acute Chlamydia trachomatis infection (see also BCSC Section 8, External Disease and Cornea, for discussion on Parinaud oculoglandular syndrome).

The best-known posterior segment manifestation of B henselae infection is neuroretinitis, a constellation of findings that includes abrupt vision loss, unilateral optic disc swelling, and macular star formation. This syndrome, formerly known as idiopathic stellate maculopathy and later renamed Leber idiopathic stellate neuroretinitis, is now known to be caused by B henselae infection in approximately two-thirds of cases. It occurs in 1%–2% of patients with CSD. Focal or multifocal retinitis may also be identified. Table 10-3 lists other entities that may cause neuroretinitis. Visual acuity loss varies between 20/25 and 20/200 or worse and follows the onset of constitutional symptoms by approximately 2–3 weeks. Although the presentation is most often unilateral, bilateral cases of neuroretinitis have been reported and are frequently asymmetric. Optic disc edema, associated with peripapillary serous retinal detachment, has been observed 2–4 weeks before the appearance of the macular star and may be a sign of systemic B henselae infection. The development of the macular star is variable (Fig 10-15) and may be partial or incomplete, usually resolving in approximately 8–12 weeks.

Patients with Bartonella-associated neuroretinitis may exhibit some degree of anterior chamber inflammation and vitritis. Discrete, focal, or multifocal retinal and/or choroidal lesions measuring 50–300 μm are common posterior segment findings. Both arterial and venous occlusive disease, as well as localized neurosensory macular detachments, have been described in association with focal retinitis. Other posterior segment ocular complications include epiretinal membranes, inflammatory mass of the optic nerve head, peripapillary angiomatosis, intermediate uveitis, retinal white dot syndromes, orbital abscess, isolated optic disc swelling, and panuveitis. Immunosuppressed individuals may display a retinal vasoproliferative response, leading to single/multiple angiomatoid lesions involving retina/choroid.

Table 10-3 Differential Diagnosis of Neuroretinitis

Figure 10-15 Left, color fundus photograph of the left eye of a patient with cat-scratch disease shows neuroretinitis with optic disc involvement associated with a macular star. Punctate retinal infiltrates are also visible superiorly (arrows). Upper right, spectral-domain optical coherence tomography scan delineates the inner retinal infiltrate (arrow). Lower right, scan reveals subretinal fluid (peripapillary serous retinal detachments, arrows), intraretinal exudates, vitreous inflammatory infiltration, and optic disc and macular edema.

(Courtesy of Daniel V. Vasconcelos-Santos, MD, PhD.)

• Chi SL, Stinnett S, Eggenberger E, et al. Clinical characteristics in 53 patients with cat scratch optic neuropathy. Ophthalmology. 2012;119(1):183–187.

Excerpted from BCSC 2020-2021 series: Section 9 - Uveitis and Ocular Inflammation. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.