Year : 2000 | Volume
: 48 | Issue : 1 | Page : 47--9
Papilloedema with peripapillary retinal haemorrhages in an acquired immunodeficiency syndrome (AIDS) patient with cryptococcal meningitis
RR Battu, J Biswas, N Jayakumar, HN Madhavan, N Kumarsamy, S Solomon
Medical and Vision Research Foundation, 18 College Road, Chennai-600 006, India
R R Battu
Medical and Vision Research Foundation, 18 College Road, Chennai-600 006
|How to cite this article:|
Battu R R, Biswas J, Jayakumar N, Madhavan H N, Kumarsamy N, Solomon S. Papilloedema with peripapillary retinal haemorrhages in an acquired immunodeficiency syndrome (AIDS) patient with cryptococcal meningitis.Indian J Ophthalmol 2000;48:47-9
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Battu R R, Biswas J, Jayakumar N, Madhavan H N, Kumarsamy N, Solomon S. Papilloedema with peripapillary retinal haemorrhages in an acquired immunodeficiency syndrome (AIDS) patient with cryptococcal meningitis. Indian J Ophthalmol [serial online] 2000 [cited 2023 Sep 30 ];48:47-9
Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?2000/48/1/47/14855
Cryptococcosis is the most common fungal infection of the eye in patients with acquired immunodeficiency syndrome (AIDS). Cryptococcus neoformans, an yeast like fungus, has a marked predilection for the brain and meninges. Ocular involvement is usually secondary to intracranial involvement and may manifest as papilloedema, optic atrophy, and ophthalmoplegia. Primary ocular manifestations include chorioretinitis, choroiditis, retinovitreal abscess, neuroretinitis, and endophthalmitis.
The diagnosis in this case was confirmed by the demonstration of Cryptococcus neoformans in the cerebrospinal fluid using Nigrosin stain and by growing the organism on blood agar.
A 29-year-old female was seen in the uveitis clinic in June 1997. She gave a history of blurred vision and bifrontal headache of one week's duration. She was already known to have tested positive for Human Immunodeficiency Virus (HIV) type-I by Enzyme-Linked Immune Assay (ELISA) using Immunocomb II (Orgenics II, Israel) and Tridot test (J Mitra, India). She had contracted HIV through her husband who was a daily wage labourer and had promiscuous sexual habits. She was also being treated for pulmonary tuberculosis. On examination her vision was 6/6 in both eyes and anterior segment and fundus examination were normal. She was asked to come for a follow-up examination after 3 months. Subsequent examinations at 3 and 6 months were completely within normal limits. However, she complained of a mild blurring of vision and bifrontal headache 8 months after the first examination, in February 1998. Her vision in both eyes was 6/6, anterior segment examination and right fundus were normal. The left fundus revealed blurred disc margin and prominent peripapillary retinal haemorrhages [Figure:1]. Investigations revealed a total WBC count of 5200 cells per mm,3 absolute CD4 count of 37 cells per mm3 (normal range:-290-2600), absolute CD8 count of 102 cells per mm3 (normal range: 190-2120) and CD4/CD8 ratio of 0.37 (normal range: 0.60 - 2.80).
CT scan of the brain was normal. Nigrosin stain of the cerebrospinal fluid (CSF) revealed the presence of a Cryptococcus organism. Culture revealed Cryptococcal neoformans [Figure:2]. The patient was started on intravenous Amphotericin B for 5 days and oral Fluconazole 150 mg twice daily for 10 days. When examined in March 1998 while still on Fluconazole, the patient had developed retinal haemorrhages in the temporal periphery of the left eye, and optic disc oedema with peripapillary haemorrhages in the right eye [Figure:3]. On continued treatment, the patient became totally asymptomatic, and a month later, the retinal haemorrhages and disc oedema regressed completely. On last follow up after 3 months she maintained a vision of 6/6 in both eyes. Fundus examination of both eyes revealed normal optic disc and resolution of the retinal haemorrhages.
Ophthalmic manifestations are seen in up to 70% of patients infected with HIV. These can be classified into four groups: (1) a non-infectious microangiopathy, referred to as HIV retinopathy; (2) opportunistic ocular infections like Cytomegalovirus, Herpes zoster, Toxoplasma gondii, Mycobacterium tuberculosis, Cryptococcus neoformans, Mycobacterium avium-intracellulare, Pneumocystis carinii, Histoplastum capsulatum and Candida; (3) ocular neoplasms which include Kaposi's sarcoma and lymphoma, and (4) neuro-ophthalmic lesions secondary to cryptococcal meningitis, zoster ophthalmicus, viral encephalitis and central nervous system lymphoma.
Measurement of T-lymphocyte subsets, especially absolute CD4 counts, have become an essential part of staging HIV infections. CD4 counts of 250-500 cells per mm3 are associated with oral candidiasis and disseminated tuberculosis; counts of 150-200 cells per mm3 are associated with Kaposi's sarcoma, lymphoma and cryptosporidiosis; counts of 75-125 cells per mm3 are associated with cryptococcosis, Pneumocystis carinii, ulcerated Herpes simplex, toxoplasmosis and oesophageal candidiasis; and counts less than 50 cells per mm3 are associated with Cytomegalovirus retinitis.
Cryptococcus neoformans is an encapsulated yeast and reproduces by budding. Serotypes A and D are commonly associated with clinical disease and infection is usually acquired by inhalation of aerosolised organisms from contaminated soil. Cryptococcal infections are common in immunocompromised patients and can cause a variety of clinical syndromes including meningitis, prostatitis, ocular involvement, pneumonia, skin and soft tissue abscesses, bone infection, myocarditis and disseminated multiorgan disease. The most common clinical syndrome is meningoencephalitis and the predilection for central nervous system in humans is probably related to the relative absence of antibody, complement activity or other soluble anticryptococcal factors in the cerebrospinal fluid and the presence of substances that enhance local cryptococcal proliferation.
Ophthalmic complications occur in 36-40% of cases of cryptococcosis and include papilloedema, optic atrophy and ophthlamoplegias. Other documented intraocular features of the infections include chorioretinitis, choroiditis, retinovitreal abscess, neuroretinitis, and endophthalmitis.
Cryptococcal meningitis in HIV patients can cause either rapid or slow visual loss. Rapid visual loss is usually due to optic neuritis with direct invasion of the optic nerve by Cryptococcus neoformans. Papilloedema and raised intracranial pressure may cause a slow progressive visual loss. This typically begins later in the course of the disease and may lead to severe visual impairment. Early treatment to reduce the intracranial pressure and specific anti-cryptococcal treatment may help prevent this catastrophic visual loss.
HIV retinopathy may also present as retinal haemorrhages though their distribution is mainly in the posterior pole of the fundus. Hence it is necessary to consider cryptococcal meningitis in the differential diagnosis whenever an AIDS patient presents with peripapillary retinal haemorrhages.
Combined therapy with oral fluconazole and intravenous amphotericin B has been the recommended treatment of choice for patients with disseminated or meningeal cryptococcosis. Fluconazole is a relatively new azole compound with minimal side effects and is especially useful in the overall management of patients with AIDS where it is desirable to use relatively non-toxic agents. Fluconazole has been found to be beneficial in the resolution of papilloedema caused by cryptococcal meningitis.
Our report indicates that papilloedema with retinal haemorrhages can be the presenting feature in a patient of cryptococcal meningitis with AIDS. Early diagnosis and prompt treatment with a combination of intravenous Amphotericin B and Fluconazole can lead to resolution of retinal lesions.
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