Onchocerciasis: a potential risk factor for glaucoma.

BACKGROUND: Onchocerciasis is a microfilarial disease that causes ocular disease and blindness. Previous evidence of an association between onchocerciasis and glaucoma has been mixed. This study aims to further investigate the association between onchocerciasis and glaucoma. METHODS: All subjects were patients at the Bishop John Ackon Christian Eye Centre in Ghana, west Africa, undergoing either trabeculectomy for advanced glaucoma or extracapsular extraction for cataracts, who also had a skin snip biopsy for onchocerciasis. A cross sectional case-control study was performed to assess the difference in onchocerciasis prevalence between the two study groups. RESULTS: The prevalence of onchocerciasis was 10.6% in those with glaucoma compared with 2.6% in those with cataracts (OR, 4.45 (95% CI 1.48 to 13.43)). The mean age in the glaucoma group was significantly younger than in the cataract group (59 and 65, respectively). The groups were not significantly different with respect to sex or region of residence. In models adjusted for age, region, and sex, subjects with glaucoma had over three times the odds of testing positive for onchocerciasis (OR, 3.50 (95% CI 1.10 to 11.18)). CONCLUSIONS: This study has shown a positive association between subclinical onchocerciasis and glaucoma. This finding emphasises the importance of eradication of onchocerciasis from west Africa.

Oculocerebral malformations. A reappraisal of Walker's 'lissencephaly'.

We describe an infant with multiple ocular and cerebral malformations. The eye lesions included microphthalmia, detachment and dysplasia of the retina, hypoplasia of the optic nerve, persistence of the primary vitreous, cataracts, obliteration of the anterior chamber, and vascularization of the cornea. In the brain, the salient features were microencephaly, agyria of an unusual type, cerebellar hypoplasia and dysplasia, persistent corpus pontobulbare, obliteration of the subarachnoid space, and a midline arachnoid cyst in the posterior fossa. We suggest that these lesions were the end result of a fetal infection that operated during a protracted period starting not later than the fourth month of intrauterine life.

Radiotherapy of lymphoid diseases of the orbit.

Thirty-two patients with orbital pseudotumor (18), reactive lymphoid hyperplasia (2), atypical lymphoid infiltrate (4) or malignant lymphoma (8) were treated in the Division of Radiation Therapy at Stanford University between January 1973 and May 1983. Of the 20 patients with pseudotumor or reactive lymphoid hyperplasia, 10 had unilateral lesions and 10 had bilateral lesions. Biopsy samples were obtained in 15 patients; in five patients with bilateral disease the diagnosis was made on the basis of computed tomography (CT) and clinical findings. The majority of patients were referred because of disease refractory to treatment with corticosteroids. The patients were given a mean dose of 2360 rad using complex, individualized megavoltage techniques including lens shielding. Radiotherapy was well tolerated with no significant acute or late complications. Fifteen patients had complete resolution of symptoms after treatment; five had continued symptoms. Of the 12 patients with malignant lymphoma or atypical lymphoid infiltrate, four had systemic lymphoma with orbital involvement and eight had orbital involvement only. The diagnosis was made by biopsy in all patients and immunophenotyping was done in six cases, of which 5 were monoclonal. Patients were evaluated with a chest radiograph, lymphogram or abdominal CT, bone marrow biopsy and orbital CT. A mean dose of 3625 rad was delivered to the orbit only. Most of the patients received complex megavoltage treatment using bolus. All patients in this group had a complete response and local control. There were no relapses in those with localized disease. Two patients developed cataracts. Carefully planned orbital radiotherapy provides local control without symptomatic sequelae for orbital masses ranging from pseudotumor to malignant lymphoma.

Aggressive management of second primary tumors in survivors of hereditary retinoblastoma.

Survivors of hereditary retinoblastoma are at increased risk for the development of second primary tumors, most commonly osteosarcoma. Recent molecular genetic data demonstrate that a pleiotrophic effect of the retinoblastoma gene may be responsible for the development of these sarcomas. This report describes the incidence of second nonocular malignancies among 53 infants seen at Stanford University Medical Center who have been followed a median of 11.7 years. Of these, 42 initially had bilateral disease and eleven had unilateral disease. Of 53 infants, 50 received irradiation either as part of the initial therapy or as treatment for recurrent disease. The actuarial survival for the entire group is 67% at 30 year follow-up with a median survival of 79% at 11.7 years. Eight patients developed eleven second primary tumors. All occurred in the group having hereditary retinoblastoma. Eight were within the previously irradiated field and three were at distant sites. The second tumors included seven osteosarcomas, one angiosarcoma, one rhabdomyosarcoma, one malignant fibrous histiocytoma, and one unclassifiable round blue cell tumor. The actuarial incidence of the development of a second primary malignancy was 6% at 10 years, 19% at 20 years, and 38% at 30 years. The latent period from treatment of retinoblastoma to the diagnosis of malignancy ranged from 5.2 years to 36.2 years (median 16 years). An aggressive approach with combined modality therapy including radical resection, re-irradiation and/or chemotherapy was used to treat these second primary tumors in five of eight patients. In four of the five, there was no evidence of disease at 22-72 months following treatment. In the three patients who did not receive aggressive combined treatment, there were no survivors. These data confirm the previously reported risk of developing a second primary tumor among survivors with hereditary retinoblastoma. Careful long-term follow-up for this genetically susceptible group is essential for early detection and implementation of curative therapy.

Results of extracapsular cataract surgery and intraocular lens implantation in Ghana.

Neither outpatient surgery nor intraocular lenses have been widely used in developing countries. We performed extracapsular cataract extraction with posterior chamber intraocular lens implantation in a simple outpatient clinic in Ghana, West Africa. Forty-nine (64%) of 77 eligible patients with follow-up times of 12 to 29 months after surgery underwent an eye examination and an interview related to activities of daily life. Preoperative visual acuity was counting fingers or worse in all but one patient. Visual acuity improved in 44 patients (90%) after surgery. Twenty-six patients (53%) had a corrected visual acuity of 20/40 or better, 11 (22%) had a corrected visual acuity of 20/50 to 20/100, and 12 (25%) had a corrected visual acuity of 20/200 or worse. Subjectively, 94% of patients believed that their vision improved after surgery. No major late complications occurred following the immediate postoperative period. In addition, our population experienced no complications attributable to the outpatient format of this surgery. Every patient, all of whom lived within a 32-km radius of the clinic, reliably returned for postoperative appointments. Our results demonstrate that outpatient surgery may be a safe and practical alternative to routine hospitalization for eye surgery in developing countries.

Regression of iris neovascularization following panretinal photocoagulation.

A 57-year-old woman with proliferative diabetic retinopathy in both eyes also had iris surface neovascularization with early angle neovascularization in her right eye, which regressed completely following treatment with panretinal photocoagulation. Histopathologic examination confirmed the complete absence of abnormal vessels on the iris surface or in the iridocorneal angle of the right eye; an avascular fibrous membrane persisted on a segment of the posterior iris pigment epithelium near the pupil. There was advanced rubeosis in the untreated left eye.

Multiple ocular anomalies associated with maternal LSD ingestion.

Severe ocular malformations, including microphthalmos, intraocular cartilage, cataract, persistent hyperplastic primary vitreous, and retinal dysplasia, occurred in a premature baby girl. The mother had ingested LSD during the first trimester of pregnancy. To our knowledge, this is the third case reported of ocular teratogenesis associated with maternal LSD ingestion. Further cases must be documented to establish an actual cause and effect relationship between the drug and the induced malformations.

Nosematosis of the cornea. Case report, including electron microscopic studies.

A 26-year-old woman underwent enucleation of a blind, painful eye because of a perforated corneal ulcer. Histopathologic examination of the eye disclosed an acute, necrotizing keratitis surrounding myriad small (2.5 to 3 x 4.5 to 5 micrometer), oval, faintly visible structures. Under polarized light, they were partially birefringent and stained intensely positive with the acid-fast technique. The organisms were located mainly within the cytoplasm of histiocytes in the deep corneal lamellae. They were interpreted as a protozoa of the genus Nosema. The diagnosis was confirmed by electron microscopic studies. The distinguishing features between Nosema species and Encephalitozoon are discussed. To our knowledge, this is the second documented case of corneal nosematosis.

Visual results and ocular complications following radiotherapy for retinoblastoma.

Between 1956 and 1974, 28 children with retinoblastoma have been irradiated with the linear accelerator at the Stanford University Medical Center. Twenty-seven children (96%) have been cured of their tumor, with follow-up ranging from 2 1/2 to 21 years. In these survivors, 50 eyes were affected. Twelve were treated by primary enucleation and 38 were irradiated. Sixteen of the 38 irradiated eyes (42%) were ultimately enucleated for recurrent tumor, neovascular glaucoma, or inability to observe the tumor through opaque media. Thus, 22 (58%) irradiated eyes were saved. Of these, five eyes had visual acuity of 20/40 or better, five had 20/50 to 20/100, nine had 20/200 to hand motion, and three had light perception or no light perception. Radiation therapy can sterilize the tumor and maintain useful vision in many children with retinoblastoma.

Diode laser transscleral cyclophotocoagulation as a primary surgical treatment for primary open-angle glaucoma.

OBJECTIVES: To evaluate the feasibility of diode laser transscleral cyclophotocoagulation (TSCPC) as a primary surgical treatment for primary open-angle glaucoma and to compare 2 laser energy settings used for treatment. METHODS: In a prospective clinical trial in Cape Coast and Accra, Ghana, 1 eye of each of 92 patients with primary open-angle glaucoma was treated by diode laser TSCPC as a primary surgical treatment. Eyes were randomly assigned to receive treatment by 20 applications of either 1.5 W applied for 1.5 seconds or 1.25 W applied for 2.5 seconds. RESULTS: Seventy-nine (86%) of 92 patients completed 3 months of follow-up; follow-up was 13.2 +/- 6.0 months (mean +/- SD). Intraocular pressure decreased in 53 (67%) of the 79 eyes. The drop in intraocular pressure was 20% or more in 37 eyes (47%) and final intraocular pressure was 22 mm Hg or less in 38 eyes (48%). An atonic pupil was a previously unreported complication that arose in 27 (28%) of 92 eyes. There were no serious complications of hypotony, phthisis bulbi, or sympathetic ophthalmia. Visual acuity decreased in 18 (23%) of 79 eyes treated by TSCPC and in 10 (23%) of 47 fellow eyes treated only with glaucoma medications. There was no difference in outcomes between the 2 laser energy settings. CONCLUSIONS: Diode laser TSCPC is a practical, rapid, well-tolerated procedure that may provide a modest and variable lowering of intraocular pressure. The treatment, used with conservative energy levels applied to the eye, seems to have few serious complications, although a previously unrecognized complication of atonic pupil needs further evaluation. A moderate variation in laser energy settings does not influence the results of treatment.

Optic nerve head involvement with cytomegalovirus in an adult with lymphoma.

An optic nerve swelling that was thought clinically to represent a lymphomatous infiltrate of the nerve head developed in a 51-year-old man with histiocytic lymphoma. Histologic examination of the eyes showed the presence of cytomegalovirus (CMV) in the nerve head, but no lymphoma cells. Tru lymphomatous invasion of the nerve head in systemic lymphoma is probably very rare. This case points out an unusual manifestation of adult CMV infection, and a new concern in patients with compromised immune systems.

Candida endophthalmitis after intravenous drug abuse.

Patients with endogenous Candida endophthalmitis associated with intravenous (IV) drug abuse may manifest ocular and systemic signs different from those seen in other forms of endogenous Candida endophthalmitis. There may be a sparcity of evidence of systemic candidiasis, including negative serology and normal physical examination results. Anterior uveitis and extensive vitreous involvement are common and do not necessarily have associated typical retinal lesions, which are more commonly seen in the compromised host. This may occur either because of the more transitory nature of choroidal or retinal lesions or because these patients often seek treatment at later stages. Even with a typical clinical picture, it is difficult to get culture confirmation of the diagnosis. Material obtained by vitrectomy must be concentrated before inoculation of media because of the known difficulty of culturing Candida from the vitreous cavity.

Histologic study of healing after ab interno laser sclerostomy.

We examined the histologic characteristics of healing after ab interno laser sclerostomy in a human eye. A KTP 532 green laser coupled to a 300-microns quartz fiberoptic probe was used to create an ab interno sclerostomy in a terminally ill patient with pigmentary glaucoma. The intraocular pressure increased five days postoperatively and did not respond to medical treatment. The patient died six weeks postoperatively of metastatic lung cancer. Histopathologic analysis showed a patent 150-microns scleral lumen from the anterior chamber to the episclera, surrounded by a 300-microns zone of acellular thermal damage. There was no healing of the lumen. The subconjunctival end of the lumen was capped with a thick episcleral scar, which caused the failure of the operation.

A simple conjunctival biopsy.

Biopsy of conjunctival goblet cells may be carried out by pressing a piece of Millipore filter on the conjunctival surface. This can then be stained with PAS and hematoxylin and observed under the microscope after the filter has been cleared with oil. Either spots of mucous corresponding to the openings of the goblet cells are seen, or the filter adheres to the surface and pulls off one or more layers of epithelial cells and goblet cells. The procedure can be carried out on the unanesthetized eye and does not cause discomfort.

A prospective trial of intraoperative fluorouracil during trabeculectomy in a black population.

In a study population of black Africans with advanced glaucoma in Ghana we conducted a prospective study of intraoperative 5-fluorouracil alone. Eyes undergoing trabeculectomy were randomly selected either to receive or not receive a single intraoperative application of 5-fluorouracil (50 mg/ml for five minutes). Fifty-five eyes had a mean follow-up of 282 days (minimum, 92 days). Twenty of 24 eyes (83%) in the 5-fluorouracil group vs 12 of 31 eyes (39%) in the control group had postoperative intraocular pressure of 20 mm Hg or less with or without medical therapy (P = .01). Eleven of 24 eyes (46%) in the 5-fluorouracil group and five of 31 eyes (16%) in the control group had intraocular pressure of 15 mm Hg or less (P = .02). Without medical therapy, 17 of 24 eyes (71%) in the 5-fluorouracil group and ten of 31 eyes (32%) in the control group had intraocular pressure of 20 mm Hg or less (P = .02). The overall complications were similar in the two groups. In this population, intraoperative 5-fluorouracil markedly improved the ability of trabeculectomy to lower intraocular pressure. We recommend that intraoperative 5-fluorouracil be considered in glaucoma surgery with poor prognosis as an alternative to postoperative subconjunctival injections when multiple injections are not feasible.

Trabeculectomy with intraoperative 5-fluorouracil vs mitomycin C.

PURPOSE: To compare the effectiveness of intraoperative 5-fluorouracil (5-FU) and mitomycin C used adjunctively with trabeculectomy in a black West African population. METHODS: Eighty-five consecutive eyes of 85 black patients undergoing primary trabeculectomy for open-angle glaucoma were prospectively randomly assigned to receive either 5-FU (50 mg/ml for 5 minutes) or mitomycin C (0.5 mg/ml for 3 1/2 minutes) intraoperatively by soaked sponge. RESULTS: Of the 81 eyes with at least a 3-month postoperative follow-up, 41 of 44 (93.2%) in the mitomycin C group and 27 of 37 (73.0%) in the 5-FU group had a final intraocular pressure of less than 21 mm Hg (P = .01). Twenty-eight of 44 eyes (63.6%) in the mitomycin C group and 18 of 37 (51.4%) in the 5-FU group had a final intraocular pressure of less than 15 mm Hg (P = .26). Mean postoperative intraocular pressure was 13.7 mm Hg in the mitomycin C group and 16.3 mm Hg in the 5-FU group (P = .05). There were no differences between the two groups in mean age, preoperative intraocular pressure, postoperative visual acuity, and complications. Mean follow-up was 10.0 +/- 4.41 months (range, 4 to 19 months). CONCLUSIONS: The adjunctive use of mitomycin C with trabeculectomy is equally safe and more efficacious compared to 5-FU in this West African population. Use of mitomycin C in this study was not associated with a statistically significantly greater proportion of patients achieving low intraocular pressure (less than 15 mm Hg) compared to 5-FU.

Posterior ocular abnormalities after irradiation for retinoblastoma: a histopathological study.

Radiation-induced ocular lesions in the posterior eye and orbit were investigated in 33 surgical specimens of patients with retinoblastoma. The eyes were obtained from children 7 months to 6 years of age. Seventeen eyes were irradiated; 16 eyes had not received irradiation and served as controls. The majority of the irradiated eyes were treated with 6000 rads of external beam radiation. They were removed at a mean of 23 months after radiotherapy. All specimens were examined simultaneously by 2 observers without knowledge of treatment and analysed for the presence or absence of 15 lesions. The most consistent lesions in the irradiated eyes were abnormalities of the retinal vessels (11 of 17 eyes) and striking changes in the ciliary arteries (13 of 17 eyes). The retinal vessels showed thickening of the wall, often caused by deposition of fibrillary material, sometimes with fibrin deposits. The most consistent lesion was myointimal proliferation with narrowing of the ciliary arteries. Lesions of the central retinal artery were less common but occurred only in irradiated patients.

Intraocular involvement with subretinal pigment epithelium infiltrates by mycosis fungoides.

We report a case of intraocular mycosis fungoides in a 48-year-old man. The patient presented with decreased visual acuity, white subretinal lesions, and vitritis. Post-mortem histopathology revealed malignant T cell infiltrates consistent with mycosis fungoides in the retina, vitreous, and between the retinal pigment epithelium (RPE) and Bruch's membrane Focal atrophy of the RPE, along with the sub-RPE infiltrates, correlated with the clinically visible fundus lesions.

Risk of hypotony after primary trabeculectomy with antifibrotic agents in a black west African population.

PURPOSE: To evaluate the risk of hypotony and hypotony maculopathy following trabeculectomy with 5-fluorouracil (5-FU) and mitomycin C (MMC) in a black West African population. METHODS: One hundred and one eyes of black Ghanaian patients with advanced primary open-angle glaucoma received intraoperative antifibrotic therapy with trabeculectomy as part of two randomized clinical trials. Overall, 57 patients received 5-FU (50.0 mg/ml for 5 minutes) and 44 received MMC (0.5 mg/ml for 3.5 minutes). All cases were performed by one of five surgeons at a single outpatient surgery center. RESULTS: Two of 101 eyes had a final intraocular pressure (IOP) of less than 5 mm Hg and were thus classified as having hypotony. No patient in either group was noted to develop hypotony-related maculopathy. Overall mean pre- and postoperative IOPs were 30.1 and 15.9 mm Hg, respectively. The patients receiving intraoperative MMC had a lower mean postoperative IOP (14.7 mm Hg) than those receiving 5-FU (first study, 17.1 mm Hg; second study, 16.7 mm Hg; p = 0.05). Mean overall follow-up was 17.7 months and did not differ significantly between the MMC and combined 5-FU groups. CONCLUSION: Hypotony following trabeculectomy supplemented with antifibrotic agents is a rare complication in this population. No eyes in either clinical trial developed hypotony maculopathy, suggesting that the prevalence of this condition is substantially lower in black West Africans than in whites.