Ординатура / Офтальмология / Английские материалы / Textbook of Vitreoretinal Diseases and Surgery_Natarajan, Hussain_2008

Textbook of Vitreoretinal Diseases and Surgery

infection with the human papilloma virus (HPV), which may explain its increased prevalence in immunosuppressed individuals with HIV disease. Smaller lesions can be excised followed with cryotherapy invasive lesions necessitate enucleation or exenteration. More recently, topical medications, including mitomycin-C,60 5-fluorouracil,61 and interferon, have been used successfully for treatment of various types of ocular surface squamous neoplasia.

Non-Hodgkin Lymphoma (NHL)

NHL is the second most common opportunistic neoplasm in HIV-infected individuals. Since the introduction of HAART, there has been a substantial reduction in the incidence of NHL in HIVinfected individuals and some studies suggest there is no difference in the incidence of NHL in non HIV and HIV patients.62

Lymphoma can occur in the eyelids, conjunctiva and orbits, where it can cause painful proptosis. Primary ocular lymphoma in immunocompetent individuals often presents with unilateral or bilateral decreased vision, vitritis, and retinal or choroidal infiltrates. In HIV-infected individuals, NHL should be suspected in patients of any age who have prominent vitreous humor cells, with or without subretinal material. Intraocular lymphoma usually occurs when CD4+ T-lymphocyte counts are less than 50 cells/ml.

Intraocular lymphoma can also cause anterior chamber cellular reactions.63,64 Lesions suspicious for lymphoma should be biopsied, and systemic evaluation for non-ocular sites of disease should be undertaken. Treatment consists of radiation or chemotherapy.

Our Experience with Ocular Complications of AIDS at Minto Eye Hospital

The most common ophthalmic lesions seen causing morbidity was CMV retinitis and herpes zoster ophthalmicus. A total of 184 cases of AIDS patients were seen between 2000-2003. HIV retinopathy was another commonest posterior segment lesion seen in 18 (10.28%) cases .13 (72.22 %) were males and 5(27.77%) cases were females. Unilateral in 8 (50.00%) and bilateral in 7(38.88%). one case (5.55%) presented with ischemic optic neuropathy. CMV retinitis was seen in 18 cases (10.28% overall) (25.35% among those with ocular findings, i.e. 71 cases) seen in 34 eyes of 18 patients, predominantly seen in 16(88.8%) males cases and 2(11.22%) female cases. In 4 (22.22%) CMV retinitis was the AIDS defining diagnosis. 1(50%) female case presented with CMV retinitis as first presentation of HIV disease. Bilateral in 16(88.8%), 9 (50%) cases presented with end stage disease. 4 cases died within 4-9 weeks of diagnosis. 9 (50%) cases were lost to follow up. Average age of cases was 34.77 years overall and 30 years in females (2 cases). No children manifested with CMV retinitis. 12 (66.66%) cases had classic pizza pie fundus appearance, 2(11.11%) cases frosted branch angiitis appearance was seen, CMV papillitis was seen in 1 case, and mixed presentation was seen in 3 cases. Average CD4 + counts among 11 cases in which CD4+ counts were done were 87.53 cells/microliter. 5 cases died due to various systemic disorders during the period of follow up. CD4+ counts done in 5 cases gave an average count of 214.5 cells/microliter cases. Average age at presentation among with HIV retinopathy was 28.13 years. Acute retinal necrosis was seen in 4(2.28%) cases ,all were males,100% unilateral,

288 Cutaneous zoster history was seen in 3 cases. 2 cases had herpes zoster ophthalmicus. 1 case of intravenous drug abuse was associated with ARN. One case died shortly after diagnosis due to

Ocular Manifestations of HIV/AIDS

systemic illnesses. In 1 case ARN was aids defining diagnosis. One case of immune recovery vitritis was seen in a 28 year old case with improvement in CD4+ counts from 22 cells/ cumm to 384 cells/ cumm 3 months after starting HAART.

Forty-nine cases had anterior segment lesions, herpes zoster ophthalmicus 18 (10.28%) cases was the commonest anterior segment disease. Male : female ratio was 14:4, no children had HZO. Average age at presentation of HZO was 33.33 years. HZO was the first presentation of HIV in 6 (33.33%) cases. Cornea was involved in 5 cases during acute stage. Complete recovery with standard treatment was seen in 10 cases. Two cases had recurrent epithelial defects/sterile corneal ulceration. Two cases developed HZO keratouveitis. Corneal ulceration with opacity and vascularization occurred ultimately in 5 cases. 2 cases devoloped acute retinal necrosis within 1-1½ years of herpes zoster ophthalmicus. Steven Johnson syndrome with conjunctival involvement as a result of drug reaction to nevirapine and sulphonamides was seen in 2 cases (1.14%). Other lesions included Molluscum contagiosum seen in one female patient, herpes simplex keratitis and blepharitis was seen in 2(1.14%) cases each.

Neuro-ophthalmic lesions were seen in the study included optic atrophy 5(2.85%) cases due to cryptococcal and tuberculous meningitis in 3 cases and drug toxicity due to anti-tubercular drugs. Papilledema due to cryptococcal meningitis was seen in 4 cases. Extraocular muscle palsy 6th nerve paralysis due to meningitis was seen in one case. Facial palsy due to suspected Progressive multifocal leukoencephalopathy (PML) was seen in one case.

CORRELATION WITH CD4+ CELL COUNTS

CD4+ counts were determined in 38 cases and CD4+ counts ranged from 24 to 370 median CD4+ count was 128.21 cells/microlitre. Out of the 38 cases had ocular lesions related to HIV, in whom CD4+ counts were done 13 cases had CMV Retinitis showed very low CD4+ cell counts with an average CD4+ cell count of 87.53 cells/microlitre. Follow-up examination was done in 60 cases. The duration of follow-up ranged between 12 to18 months. 20 patients died during the period of follow up because of multiple systemic infections of these patients had CMV retinitis.

VISUALACUITY

The visual acuity at initial presentation was in the range of 6/6-6/12 in 122 (n=175, 69.71%) cases. Main causes for deterioration in vision among the patients were CMV retinitis (4 cases), optic atrophy (2 cases), corneal ulcer (1 case), herpetic eye disease (HZO, HSV, ARN) (4 cases) and drug toxicity of optic nerve (1 case).

Conclusion

Ocular Manifestations of HIV/AIDS

30.Kassuto S, Doweiko JP. Syphilis in the HIV era. Emerg Infect Dis 2004;10:1471–3.

31.Lynn WA, Lightman S. Syphilis and HIV: a dangerous combination. Lancet Infect Dis 2004; 4: 456–66.

32.Aldave AJ, King JA, Cunningham ET Jr. Ocular syphilis. Curr Opin Ophthalmol 2001; 12: 433-41.

33.Spoor TC, Ramocki JM, Nesi FA, Sorscher M. Ocular syphilis 1986. Prevalence of FTA-ABS reactivity and cerebrospinal fluid findings. J Clin Neuroophthalmol 1987; 7: 191–5, 196–7.

34.de Souza EC, Jalkh AE, Trempe CL, Cunha S, Schepens CL. Unusual central chorioretinitis as the first manifestation of early secondary syphilis. Am J Ophthalmol 1988; 105: 271–6.

35.Browning DJ. Posterior segment manifestations of active ocular syphilis, their response to a neurosyphilis regimen of penicillin therapy, and the influence of human immunodeficiency virus status on response. Ophthalmology 2000; 107: 2015–23.

36.Arruga J, Valentines J, Mauri F, Roca G, Salom R,Rufi G. Neuroretinitis in acquired syphilis. Ophthalmology 1985; 92: 262–70.

37.US Centers for Disease Control and Prevention. Sexually transmitted diseases treatment guidelines 2002. MMWR Recomm Rep 2002; 51: 1–78.

38.Macher AM, Bardenstein DS, Zimmerman LE, et al. Pneumocystis carinii choroiditis in a male homosexual with AIDS and disseminated pulmonary and extrapulmonary P. carinii infection. [Letter] N Engl J Med 1987;316:1092.

39.Rosenblatt MA, Cunningham C, Teich SA, Friedman AH: Choroidal lesions in patients with AIDS. Br J Ophthalmol 1990;74:610–4.

40.Freeman WR, Gross JG, Labelle J, et al. Pneumocystis carinii choroidopathy. A new clinical entity. Arch Ophthalmol 1989;107:863-7.

41.Dugel PU, Rao NA, Forster DJ, et al. Pneumocystis carinii choroiditis after long-term aerosolized pentamidine therapy. Am J Ophthalmol 1990;110:113–7.

42.Chess J, Marcus DM: Zoster-related bilateral acute retinal necrosis syndrome as presenting sign in AIDS. Ann Ophthalmol 1988;20:431–5, 438.

43.Hellinger WC, Bolling JP, Smith TF, Campbell RJ. Varicella-zoster virus retinitis in a patient with AIDS-related complex: case report and brief review of the acute retinal necrosis syndrome. Clin Infect Dis 1993;16:208-12.

44.Holland GN. The progressive outer retinal necrosis syndrome. Int Ophthalmol 1994;18:163-5.

45.Woods AD, Caputo MK. Neuro-ophthalmic manifestations of AIDS. Optom Clin 1996; 5:113-52.

46.Friedman DI. Neuro-ophthalmic manifestations of human immunodeficiency virus infection. Neurol Clin 1991;9:5572.

47.Clark RA, Greer D, Atkinson W, Valainis GT, Hyslop N. Spectrum of Cryptococcus neoformans infection in 68 patients infected with human immunodeficiency virus. Rev Infect Dis 1990;12:768-77.

48.Rex JH, Larsen RA, Dismukes WE, Cloud GA, Bennett JE. Catastrophic visual loss due to Cryptococcus neoformans meningitis. Medicine 1993;72:207-24.

49.Manzardo C, Del Mar Ortega M, Sued O, García F, Moreno A, Miró JM. Central nervous system opportunistic infections in developed countries in the highly active antiretroviral therapy era. J Neurovirol. 2005;11 Suppl 3:7282.

50.Karavellas MP, Lowder CY, Macdonald C, et al. Immune recovery vitritis associated with inactive cytomegalovirus retinitis: a new syndrome. Arch Ophthalmol 1998; 116:169-75.

51.Goldberg DE, Wang H, Azen SP, Freeman WR. Long term visual outcome of patients with cytomegalovirus retinitis treated with highly active antiretroviral therapy. Br J Ophthalmol 2003;87:853–5.

52.Nguyen QD, Kempen JH, Bolton SG, et al. Immune recovery uveitis in patients with AIDS and cytomegalovirus retinitis after highly active antiretroviral therapy. Am J Ophthalmol 2000;129:634-9.

53.Robinson MR, Reed G, Csaky KG, et al. Immune-recovery uveitis in patients with cytomegalovirus retinitis taking highly active antiretroviral therapy. Am J Ophthalmol 2000;130:49-56.

54.Karavellas MP, Azen SP, Macdonald JC, et al. Immune recovery vitritis and uveitis in AIDS: clinical predictors, sequelae, and treatment outcomes. Retina 2001;21:1–9.

55.Song MK, Azen SP, Buley A, et al. Effect of anti-cytomegalovirus therapy on the incidence of immune recovery uveitis in AIDS patients with healed cytomegalovirus retinitis. Am J Ophthalmol 2003;136:696–702.

56.Kempen et al. Studies of Ocular Complications of AIDS Research Group. Risk of immune recovery uveitis in patients with AIDS and cytomegalovirus retinitis. Ophthalmology 2006;113:684–94

57.Kuppermann BD, Holland GN. Immune recovery uveitis. Am J Ophthalmol 2000;130:103–6.

58.Holland GN. AIDS and ophthalmology: the first quarter century. Am J Ophthalmol 2008;145:397-408.

59.Lewallen S, Shroyer KR, Keyser RB, Liomba G. Aggressive conjunctival squamous cell carcinoma in three young Africans. Arch Ophthalmol 1996;114:215-8.

Textbook of Vitreoretinal Diseases and Surgery

Introduction

Central serous chorioretinopathy (CSC) was first described by von Graefe in 1866 as central recurrent retinitis.1 Gass in 1967 named it Idiopathic central serous chorioretinopathy.2 Klein and Maumenee were the first to postulate that the source of subretinal fluid was from the choriocapillaris. Central angiospastic chorioretinopathy is another term applied to this condition.

CSC is essentially an idiopathic condition. It is a circumscribed serous detachment of the neurosensory retina which occurs over an area of leakage from choriocapillaris through the retinal pigment epithelium (RPE). The leakage beneath the retina results from disturbed fluid equilibrium causing its elevation and producing macular detachment with subsequent distortion of vision.

There are two main types of CSC, based on nature of RPE leak. The more common type termed acute, typical or classic CSC is seen in younger patients with focal leaks from RPE, mild to moderate visual loss and a benign self limiting course. A second uncommon type with widespread alterations of RPE pigmentation in posterior pole is termed chronic or diffuse retinal pigment epitheliopathy (DRPE).

DRPE is associated with older age, chronic (more than 6 months), detachment of posterior pole, poorly defined RPE leakage, multiple pigment epithelial detachment (PED’s) and poor visual prognosis owing to cystoid macular edema, foveolar atrophy, subretinal fibrosis and less commonly CNV.3

CSC usually occurs between ages 25 and 50 years and affects men more often than women (6:1). Patients above 50 have bilateral disease with male: female 2:1. CSC is common in Asians, Hispanics and Latinos than in Caucasians. Blacks appear to be least affected.4 It is seen in hyperopics and not in myopes due to pre-existing chorioretinal atrophy.

Stress has been implicated as a causative factor as also certain personality types like type A personality (competitive drive, sense of urgency, aggressive nature and hostile temperament), hypochondria, hysteria and conversional neurosis.5 Steroids whether systemic, topical or inhaled are associated with CSC.6 It may also be seen in pregnant women, especially in the last trimester.7 Other systemic associations include organ transplantation, endogenous hypercortilism (Cushing’s disease), systemic hypertension, systemic lupus erythematosus (SLE), gastrointestinal reflux disease and use of sildenafil citrate.

High endorphin levels or opiate levels are also associated with CSC . CSC may get worse even during a cold or sore throat. Tooth decay is another form of infection and should be treated as soon as possible. Any form of infection will push up the cortisol levels, leading to CSC.

Pathophysiology

It is not yet clearly understood. The previous hypothesis of abnormal ion transport across RPE and focal choroidal vasculopathy has given way to the concept of multifocal choroidal hyperpermeability subsequently leading to secondary dysfunction of the RPE as shown by ICGA. Abnormal pumping functions at the level of RPE have been implicated, although the primary pathology may involve the choriocapillaris.

In other words, the intrachoroidal edema or leakage exerts pressure on pigment epithelium causing it to blister up into focal or multifocal elevations known as serous detachments. The blister disrupts or develops a mechanical opening usually at junction between its elevated and attached areas, permitting fluid leakage through pigment epithelium beneath the neurosensory retina which

294 is avascular although neovascularization, may evolve as a secondary complication.

Central Serous Chorioretinopathy

A study by Michael Tittl et al showed that there was increased foveal pulsatile choroidal blood flow (CBF) and an abnormal distribution of fundus pulsation amplitude in areas close to the leak in patients with active CSC. 8 CBF in eyes with CSC was 45% lower than in fellow eyes. The decreased CBF might be correlated with small localised hypofluorescent areas which may indicate non-perfused areas of choriocapillaries that are frequently seen during ICGA.

Yet another study has shown that there is an abnormal subfoveal CBF regulation in patients with relapsing CSC compared with age matched non smoking healthy volunteers during isometric exercise. At 85% increase in ocular perfusion pressure, subfoveal CBF was approximately twice as high in patients with CSC compared with healthy control group.9

Multifocal electroretinogram (mfERG) has demonstrated bilateral diffuse retinal dysfunction even when CSC involves only one eye. Multifocal ERG may prove useful as a clinical marker for susceptibility to serous detachment.10 As per a study by Aimee V , Chappelow et al,11 after recovery of CSC , mfERG A wave and B wave amplitudes increased markedly where the detachment resolved and moderately elsewhere in the posterior pole of both eyes. They remained either subnormal relative to controls. mfERG B wave latencies improved from prolonged to mid normal value in both eyes. These findings support the theory that subretinal fluid retention in CSC is secondary to diffuse pathologic changes in choroid and RPE.11

Elevated circulating cortisol and epinephrine which affect the autoregulation of choroidal circulation have been seen in persons with type A personality, systemic hypertension and obstructive sleep apnoea. The incidence of CSC in persons with Cushing’s syndrome is 5%.Tewari et al demonstrated that patients with CSC showed impaired autoresponse with significantly decreased parasympathetic activity and increased sympathetic activity. Carvalho-Recchia et al showed in a series that 52% of patients with CSC had used exogenous steroids within 1 month of presentation as compared with 18% of control subjects.6

Helicobacter pylori infection may represent a risk factor in CSC, though no studies apart from, Cotticelli et al have substantiated the same. The presence of bacteria is well co-related with visual acuity and other retinal findings following an attack.12,13

Keratoconus and CSC are two uncommon diseases, possibly due to dysfunction of epithelium and its basement membrane which can occur together in some individuals.14

Symptoms and Signs

Central Serous Chorioretinopathy

FIGURE 23-3:Showing characteristic RPE atrophic tracts in chronic CSC (Courtesy: Dr Nazimul Hussain, Al Zahra Pvt Hospital, UAE)

FIGURE 23-4: ICG angiography shows choroidal hyperpermeability in late phase in chronic CSC (Courtesy: Dr Nazimul Hussain, Al Zahra Pvt Hospital, UAE)

An experienced clinician can often diagnose idiopathic CSC based solely upon history and chief complaints in a young anxious patient who presents with unilateral metamorphopsia of recent onset. The classic fundus appearance is best seen with binocular indirect ophthalmoscopy, subtle cases requiring FFA for definitive diagnosis.

Imaging Studies

FLUORESCEIN ANGIOGRAPHY