Retinal arteriolar macroaneurysms: long-term visual outcome.

Visual outcome was analysed in 16 consecutive eyes with symptomatic retinal arteriolar macroaneurysms treated by direct laser photocoagulation and 26 consecutive symptomatic eyes followed with no treatment. No difference existed between groups in presenting visual acuity, macular involvement, presence of macular subretinal fluid, or presence or location of associated haemorrhage. The mean follow up was 41 months. In the 26 untreated eyes, visual acuity was improved by 2 or more lines in 13 (50%), was unchanged in nine (35%), and decreased in four cases (15%). In the 16 treated cases, three improved (19%), seven were unchanged (43%), and six had decreased visual acuity (38%). The average minimum angle of resolution improved 0.53 log units in untreated cases and decreased 0.14 log units in treated cases (p = 0.02). Multivariable logistic regression modelling analysis revealed that laser treatment remained a significant risk factor for final visual acuity of less than 20/80 even when controlling for the effects of subretinal haemorrhage and foveal subretinal fluid (odds ratio 8.4, p = 0.01). Laser photocoagulation directly to the macroaneurysm did not improve the visual outcome in this series.

Surgical management of a long-standing subfoveal neovascular membrane secondary to ocular histoplasmosis.

Subretinal neovascular membranes located under the fovea typically lead to significant visual loss. Laser treatment has been limited due to the anatomic location of the neovascular process, but surgical removal has been reported with promising results. We surgically removed a long-standing, presumed ocular histoplasmosis syndrome (POHS) subfoveal neovascular membrane that had been present for 10 months in a patient's only functional eye, with unexpected good results. This case suggests that the good visual outcomes achieved by surgically excising smaller subfoveal neovascular membranes early in their natural history also can be achieved in selected patients with more chronic and severe subfoveal neovascular processes.

Retinal periphlebitis in patients with acquired immunodeficiency syndrome with cytomegalovirus retinitis mimics acute frosted retinal periphlebitis.

Acute frosted retinal periphlebitis is an inflammatory condition of unknown origin characterized by marked perivenular infiltration in otherwise healthy patients. We encountered seven patients with acquired immunodeficiency syndrome who exhibited visual loss associated with an unusual diffuse retinal periphlebitis very similar in appearance to acute frosted retinal periphlebitis. Each patient developed a thick inflammatory infiltrate surrounding the retinal venules, creating a frosted appearance. Two cases were bilateral. All patients had areas of more typical cytomegalovirus retinitis in their involved eye(s). Five of six patients treated with ganciclovir sodium showed improvement not only of the cytomegalovirus retinitis but also of the periphlebitis. Although we do not have histopathologic evidence that cytomegalovirus was the cause of these cases of periphlebitis, we believe that periphlebitis may be a previously unrecognized finding of cytomegalovirus retinitis in patients with acquired immunodeficiency syndrome. So far, there is no evidence implicating cytomegalovirus as the cause of acute frosted retinal periphlebitis in healthy patients.

Long-term visual outcome in Terson syndrome.

The presentation and long-term visual outcome in 30 eyes with Terson syndrome is evaluated. In 25 of 30 eyes (83%), visual acuity of 20/50 or better was attained. This occurred in 12 of 16 eyes (75%) managed by observation alone and 12 of 14 eyes (86%) treated by pars plana vitrectomy. The most common long-term sequelae in all eyes studied was the formation of an epiretinal membrane. These occurred in 14 of 18 eyes (78%) followed for 3 or more years but accounted for significant visual loss in only 2 eyes. There was no difference in final visual outcome between those patients undergoing vitrectomy and those managed conservatively. However, visual recovery was more rapid in eyes undergoing vitrectomy despite the fact that vitrectomy was reserved for eyes with more dense vitreous hemorrhage.

Pupillary constriction to darkness in a patient with blue-cone monochromatism.

A 17 year old male patient presented with bilateral photophobia, poor color vision, visual acuity 20/80-20/200, nystagmus, and showed normal fundi. Electroretinography revealed evidence of blue-cone monochromatism. This patient showed constriction of the pupils to darkness or the paradoxic pupillary phenomenon.

Ocular findings in Turcot syndrome (glioma-polyposis).

Turcot syndrome is a hereditary condition characterized by multiple, adenomatous gastrointestinal polyps associated with neuroepithelial tumors of the central nervous system. The authors examined a patient with Turcot syndrome who had multiple regions of congenital hypertrophy of the retinal pigment epithelium (CHRPE) with areas of surrounding hypopigmentation in the fundi of both eyes. Multiple, bilateral patches of CHRPE have been reported in patients with familial adenomatous polyposis and Gardner syndrome. This finding is thought to be a sensitive and specific clinical marker for these conditions and useful for predicting the presence and development of colorectal polyposis. Our findings provide further evidence that familial adenomatous polyposis, Gardner syndrome, and Turcot syndrome may be related conditions representing the variable phenotypic expression of a single, autosomal dominant genetic disorder. Children and young adults with multiple patches of CHRPE and a family history of adenomatous polyposis may be at increased risk for the development of central nervous system tumors as well as gastrointestinal polyps.

Long-term visual outcome in patients with optic nerve pit and serous retinal detachment of the macula.

Serous detachment of the macula is a well-known complication in patients with an optic nerve pit. Despite the many descriptions of this condition and possible treatment options, the long-term natural history is not well known. The authors identified 15 eyes of 15 consecutive patients seen over 21 years who were diagnosed with a serous detachment of the macula arising from an optic nerve pit. Average length of follow-up was 9 years. Twelve eyes lost three or more lines of vision, two eyes remained unchanged, and only one eye improved. All of the 12 eyes losing three or more lines of vision experienced this decrease within the first 6 months of follow-up. Although only two patients had a visual acuity of 20/200 or less initially, 12 of 15 patients had a visual acuity of 20/200 or less at the time of their last examination. The appearance of the macula at last examination included cystic changes of the neurosensory retina, full-thickness hole formation, retinal pigment epithelial mottling, and lamellar hole formation in the outer retinal layer. The long-term visual prognosis in patients with optic nerve pit and untreated serous retinal detachment of the macula is poor, and visual loss occurs within 6 months of the serous detachment.

Visual prognosis in macular retinoblastomas.

Since 1979, we have treated 11 patients who had macular retinoblastomas. Two patients eventually recovered 20/20 visual acuity despite the presence of subretinal fluid in the fovea at the time of diagnosis. The diagnosis was made at 11 and 14 months of age, and follow-up periods were ten and seven years, respectively. One case was sporadic and the other was hereditary. Both patients were treated with external radiation; one patient was also treated with chemotherapy. The lesions regressed markedly after treatment. These cases demonstrate that visual prognosis in macular retinoblastomas is not uniformly poor even when a foveal detachment is present. Visual acuity may be good in some cases, which supports the merits of medical treatment rather than enucleation in selected patients.

Ocular manifestations of patients with circulating antineutrophil cytoplasmic antibodies.

Antineutrophil cytoplasmic antibodies are seen in patients with systemic vasculitides, especially Wegener's granulomatosis. Antineutrophil cytoplasmic antibodies are helpful laboratory markers for these disease. We report on the ocular findings of six patients with systemic vasculitis who had antineutrophil cytoplasmic antibodies. Four patients had systemic Wegener's granulomatosis, one had microscopic polyarteritis, and in one a specific histopathologic diagnosis could not be made. Two patients were first evaluated for systemic vasculitis because of their ocular manifestations. Ocular findings included ptosis, bilateral lacrimal gland masses, proptosis, choroidal folds, episcleritis, phlebitis, retinal and vitreous hemorrhage, keratitis sicca, and bilateral central scotomas. It was difficult to make a systemic diagnosis in all cases. If systemic vasculitis is in the differential diagnosis of a patient with suggestive ocular findings, antineutrophil cytoplasmic antibody testing should be considered. A prospective study of antineutrophil cytoplasmic antibody testing should be considered in patients with ocular findings that suggest the possibility of vasculitis.

Rapid streptococcal antigen detection in experimental keratitis.

We assessed the role of commercially available immunodiagnostic procedures in comparison to Gram stain and culture in experimental bacterial keratitis. Rabbit corneas were inoculated with Streptococcus pneumoniae, S. pyogenes, S. faecalis, or Haemophilus influenzae. Corneal scrapings were processed before and during antibacterial therapy using a coagglutination assay to detect pneumococcal capsular antigen (Phadebact Pneumococcus test) and an enzyme immunoassay to detect group A streptococcal cell-wall antigen (TestPack Strep A test). In untreated infected eyes, both immunoassays were highly specific and as sensitive as Gram stain for detection of the respective microorganisms. For S. pneumoniae keratitis, the sensitivity of coagglutination was 82% and Gram stain, 73%. For S. pyogenes keratitis, the sensitivity of enzyme immunoassay was 100% and Gram stain, 62%. Immunoassays and Gram stain were less sensitive than culture during antibacterial therapy. Successful clinical application of the coagglutination assay in a patient with pneumococcal keratitis permitted early use of specific cephalosporin treatment.