J. Donald M. Gass

Reappraisal of biomicroscopic classification of stages of development of a macular hole

PURPOSE To update the biomicroscopic classification and anatomic interpretations of the stages of development of age-related macular hole and provide explanations for the remarkable recovery of visual acuity that occurs in some patients after vitreous surgery. METHODS Recent biomicroscopic observations of various stages of macular holes are used to postulate new anatomic explanations for these stages. RESULTS Biomicroscopic observations include the following: (1) the change from a yellow spot (stage 1-A) to a yellow ring (stage 1-B) during the early stages of foveal detachment is unique to patients at risk of macular hole; (2) the prehole opacity with a small stage 2 hole may be larger than the hole diameter; and (3) the opacity resembling an operculum that accompanies macular holes is indistinguishable from a pseudo-operculum found in otherwise normal fellow eyes. CONCLUSIONS The change from a yellow spot (stage 1-A) to a yellow ring (stage 1-B) is caused primarily by centrifugal displacement of retinal receptors after a dehiscence at the umbo. The hole may be hidden by semiopaque contracted prefoveolar vitreous cortex bridging the yellow ring (stage 1-B occult hole). Stage 1-B occult holes become manifest (stage 2 holes) either after early separation of the contracted prefoveolar vitreous cortex from the retina surrounding a small hole or as an eccentric can-opener-like tear in the contracted prefoveolar vitreous cortex, at the edge of larger stage 2 holes. Most prehole opacities probably contain no retinal receptors (pseudo-opercula). Surgical reattachment of the retina surrounding the hole and centripetal movement of the foveolar retina induced by gliosis may restore foveal anatomy and function to near normal.

Idiopathic Macular Holes: Observations, Stages of Formation, and Implications for Surgical Intervention

The authors have reviewed 158 eyes with evolving or completed idiopathic macular holes. Observations of these patients suggest that prefoveal vitreous cortex contraction is probably the cause of idiopathic macular holes. The earliest sign of an impending macular hole (stage 1) appears to be the development of a yellow spot or halo associated with loss of the normal anatomic foveal depression. No vitreous separation is present. This may resolve or progress to a small, early macular hole (stage 2). This hole gradually enlarged to a diameter of approximately 485 micron. The vitreous usually remained attached or a vitreofoveal separation developed (stage 3). Some eyes had complete posterior vitreous separation (stage 4). The implications for surgical intervention are discussed. A prospective study should be undertaken to confirm these findings and to investigate the feasibility of vitrectomy intervention to peel the prefoveal vitreous cortex in eyes with a stage 1 lesion.

FACTORS ASSOCIATED WITH REDUCED VISUAL ACUITY DURING LONG-TERM FOLLOW-UP OF PATIENTS WITH IDIOPATHIC CENTRAL SEROUS CHORIORETINOPATHY

Purpose To investigate factors associated with reduced visual acuity during long-term follow-up of patients with idiopathic central serous chorioretinopathy (ICSC). Methods Retrospective consecutive case series that included patients with ICSC who were younger than 50 years of age at the time of initial examination and were followed up for ≥3 years. Results The mean follow-up for 101 involved eyes of 61 patients was 9.8 years (median, 8.0 years). Eyes were stratified into two groups based on visual acuity at the final examination: Group 1, visual acuity of 20/40 or better; and Group 2, visual acuity of worse than 20/40. Findings identified as potential risk factors for reduced vision at the final follow-up examinations for Group 1 versus Group 2 included the following: macular retinal pigment epithelium atrophy (90.8% versus 96.0%, respectively;P = 0.68); persistent pigment epithelial detachment or persistent subretinal fluid (5.3% versus 28.0%, respectively;P = 0.004); recurrences (39.5% versus 68.0%, respectively;P = 0.020); laser treatment (28.9% versus 32.0%, respectively;P = 0.80); and submacular choroidal neovascularization (0.0 versus 8.0%, respectively;P = 0.059). Conclusions Factors associated with reduced visual acuity during long-term follow-up of patients with ICSC included persistent pigment epithelial detachment and/or subretinal fluid, recurrences, and submacular choroidal neovascularization.

The Acute Retinal Necrosis Syndrome: Part 2: Histopathology and Etiology

The acute retinal necrosis syndrome is manifested by diffuse uveitis, vitritis, retinal vasculitis, and acute necrotizing retinitis (see Part 1). We studied the histopathology and electron microscopic findings of an eye enucleated from a 67-year-old man with typical acute retinal necrosis. Histology showed profound acute necrosis of the retina, retinal arteritis, and eosinophilic intranuclear inclusions in retinal cells. Electron microscopy demonstrated a herpes group virus in all layers of affected retina. The implications of these findings for antiviral and other treatments are discussed.

Acute Syphilitic Posterior Placoid Chorioretinitis

Six patients with evidence of secondary syphilis presented with visual loss in both eyes caused by large, placoid, yellowish lesions with faded centers at the level of the pigment epithelium in the macula and juxtapapillary areas. All eyes had vitreitis. All of the lesions showed a similar fluorescein angiographic pattern of early hypofluorescence and late staining. Five patients had mucocutaneous lesions typical of secondary syphilis. All five patients treated with antibiotics had prompt improvement in visual function and resolution of the fundus lesions. The ophthalmoscopic and angiographic appearance of these posterior fundus lesions was sufficiently characteristic to suggest a diagnosis of secondary syphilis. Modification of the host response to syphilis by human immune deficiency virus (HIV) infection may be partly responsible for this peculiar fundus picture. Three of the four patients tested positive for HIV.

Evidence-based recommendations for the diagnosis and treatment of Neovascular glaucoma

PURPOSE To succinctly update information on the pathogenesis, etiology, diagnosis, and treatment of neovascular glaucoma based on a systematic review of available literature and to provide summary recommendations rated for their importance to clinical outcome. CLINICAL RELEVANCE Neovascular glaucoma is a devastating ocular disease that often results in loss of vision. The current standard of care includes retinal ablation and control of increased intraocular pressure with medical and surgical therapy. LITERATURE REVIEW METHODOLOGY: The authors conducted a MEDLINE literature search of articles published in English from 1966 to the present. Each article reviewed was rated as to the strength of evidence it provided, and summary ratings for the strength of evidence supporting clinical recommendations were generated. RESULTS Level A (most important to patient outcome) recommendations for the diagnosis of neovascular glaucoma include a high index of suspicion, a full ocular examination including undilated gonioscopy, and pupil examination. In regard to treatment, Level A recommendations include treatment of the underlying disease origin, complete panretinal photocoagulation (if retinal ischemia is a factor), and medical control of both elevated intraocular pressure and inflammation. Level B recommendations (moderately important to patient outcome) encompass glaucoma surgery to control intraocular pressure when medical therapy is unsuccessful, although the ideal surgical procedure is unknown. Currently, trabeculectomy with antimetabolite therapy, aqueous shunt implants, and diode laser cyclophotocoagulation are the preferred surgical treatment options. CONCLUSIONS The current literature on neovascular glaucoma has few articles that provide strong evidence in support of therapy recommendations (level I). Future research studies are needed to address areas in which the current evidence is moderately strong (level II) or weak, consisting only of a consensus of expert opinion (level III). Whenever practicable, these studies should be prospective, randomized clinical trials.

The Acute Retinal Necrosis Syndrome Part 1: Clinical Manifestations

The acute retinal necrosis syndrome is characterized by necrotizing retinitis, vitritis, and retinal vasculitis occurring in otherwise healthy patients. Experience with 11 cases and the review of 30 additional cases in the literature are presented. In this series, 50% of the affected eyes developed retinal detachments, and 64% had a final visual acuity of less than 20/200. The natural history, diagnosis, postulated etiology, and suggestions for management will be discussed.

Acute zonal occult outer retinopathy: a long-term follow-up study

PURPOSE To report the long-term follow-up of patients with acute zonal occult outer retinopathy (AZOOR). DESIGN Observational consecutive case series. METHODS Prospective and retrospective review of medical records of patients with AZOOR. RESULTS Fifty-one patients (37 women and 14 men) with a median age of 33 years (mean, age 36 years; range, 13-63 years) were followed for a median of 96 months (mean 100 months; range, 36-420 months). At presentation, AZOOR was present in one eye of 31 patients (61%) and both eyes in 20 patients (39%). All patients presented with an acute loss of one or more zones of visual field, and 45 (88%) patients presented with photopsia. Corrected visual acuity was 20/40 or better in 68 (76%) of 90 affected eyes. Funduscopic examination was normal in 82 eyes and revealed signs of AZOOR in 8 eyes. Electroretinographic amplitudes were depressed in all affected eyes. The median delay in diagnosis of AZOOR was 17 months. During follow-up, AZOOR developed in 19 fellow eyes. At final follow-up AZOOR was present in one eye of 12 (24%) patients and both eyes of 39 (76%) patients. Sixteen patients had 23 recurrences of AZOOR. Visual field loss stabilized within 6 months in 37 patients (72%), progressed stepwise in 2 patients (4%), and partly improved in 12 patients (24%). Fourteen patients (28%) had autoimmune diseases, including Hashimotos thyroiditis in 6 patients and relapsing transverse myelopathy in 4 patients. At last follow-up all patients had residual visual field defects. Final visual acuity was 20/40 or better in 61 (68%) affected eyes. Nine patients (18%) were legally blind. The fundi of 90 affected eyes revealed no changes of AZOOR in 47 eyes (52%) and changes in the pigment epithelium and retina caused by AZOOR in 43 eyes (48%). CONCLUSIONS Visual loss in AZOOR is characterized by one or more episodes of acute dysfunction, and in some cases, death of retinal receptor cells in one or more zones of one or both eyes. Central vision is often spared, but recovery of visual field occurs infrequently. The etiology of AZOOR is unknown. Electroretinography is essential for early diagnosis. The value of treatment is uncertain.

Bilateral bullous exudative retinal detachment complicating idiopathic central serous chorioretinopathy during systemic corticosteroid therapy

PURPOSE To present evidence that systemic corticosteroid therapy may cause bilateral bullous serofibrinous exudative retinal detachment in some patients with idiopathic central serous chorioretinopathy. BACKGROUND Idiopathic central serous chorioretinopathy usually causes mild, transient loss of central vision, usually in otherwise healthy men with a type A personality. A few patients have permanent visual loss because of chronic and recurrent retinal detachment. The clinical findings in these patients may lead to incorrect diagnoses and use of corticosteroid therapy. METHODS The clinical and photographic records of three patients in whom bilateral bullous serofibrinous exudative retinal detachment associated with idiopathic central serous chorioretinopathy developed after treatment with systemic corticosteroids were reviewed. RESULTS Systemic corticosteroid treatment was instituted (1) as a prophylaxis to prevent exacerbation of the disease while undergoing surgery in the fellow eye, and (2) as the result of misdiagnoses of multifocal choroiditis and retinal vasculitis (Eales disease). Two of the patients had a history of chronic recurrent retinal detachments before institution of corticosteroid treatment. In one of these patients, bilateral chronic inferior retinal detachment developed, causing peripheral retinal vascular nonperfusion, retinal neovascularization, and vitreous hemorrhage. All three patients had severe permanent visual loss in one or both eyes. CONCLUSION The findings in these patients provide further evidence that systemic corticosteroid treatment may cause severe exacerbation of retinal detachment and lasting visual loss in some patients with idiopathic central serous retinopathy. Recognition of the atypical presentations of this disorder is important to avoid incorrect diagnoses and treatment.

Focal inner retinal hemorrhages in patients with drusen : An early sign of occult choroidal neovascularization and chorioretinal anastomosis

Purpose To present evidence that superficial retinal hemorrhage in the macula of patients with age-related macular degeneration (ARMD) may be an early sign of occult chorioretinal anastomosis (OCRA) and type 1 occult choroidal neovascularization (OCNV). Methods Retrospective follow-up study of 16 patients presenting with a small focal area of superficial retinal hemorrhages and drusen in the juxtafoveolar area in 24 eyes. Results OCRA and OCNV occurred in an older subset of patients with ARMD (mean age, 75 years). Of 22 eyes with the early stages of chorioretinal anastomosis (CRA), 18 had evidence of a piggyback neovascular complex, with the smaller subsensory retinal type 2 complex lying anterior to the larger subretinal pigment epithelial type 1 complex. At initial presentation, three patients had OCRA and OCNV bilaterally, and three patients had large disciform cicatricial lesions with overt CRA in the fellow eye. Nine patients had one or more laser photocoagulation treatments for early stages of CRA. Only one patient maintained visual acuity of better than 20/200 for >1 year. At the last follow-up, 24 of 26 eyes with CRA had visual acuity of 20/200 or less. Conclusion Superficial retinal hemorrhage in the paracentral area of patients with drusen is the earliest sign of OCRA and OCNV. Fluorescein angiography and indocyanine green angiography are important in detecting the dual nature of the subretinal neovascular network. Photocoagulation and photodynamic treatment is usually unsuccessful in preserving central vision.