Bilateral atypical nodular posterior scleritis.

PURPOSE: To evaluate ocular features of nodular posterior scleritis simulating choroidal melanoma. METHODS: A 60-year old woman presented with blurred vision in her right eye of two weeks duration. On examination she had a mild right-globe proptosis with an episcleral nodular mass as well as a large elevated nonpigmented choroidal mass involving the nasal quadrant. RESULTS: A and B-scan ultrasonography showed a medium to high-reflective solid choroidal mass. MRI demonstrated a bi-convex mass in the medial aspect of the right globe with signal characteristics compatible with choroidal melanoma. Biopsy of the extraocular lesion demonstrated chronic inflammatory cell infiltrate suggestive of posterior scleritis. She responded to corticosteroid therapy. On evaluation 41 months later she was noted to have a similar choroidal mass in the left eye. CONCLUSIONS: The physician should be aware of the clinical manifestations and diagnostic hall marks of nodular posterior scleritis in order to differentiate this inflammatory process from choroidal melanoma.

Phacoemulsification surgery and foldable intraocular lens implantation in a child with regressed retinoblastoma.

PURPOSE: The management of retinoblastoma has been shifting toward conservative treatment. Visual preservation has become a concern as tumor regression is achieved. To report the surgical approach and outcome of a radiation-induced cataract in an only eye with regressed retinoblastoma. METHODS: Single case report and review of literature. RESULTS: Small incision sutureless phacoemulsification and implant of an acrylic foldable intraocular lens was performed. Best-corrected visual acuity 1 year after surgery was 20/20. No tumor recurrence has been noted after 27 months of follow-up. CONCLUSIONS: Phacoemulsification and foldable intraocular lens implant seemed to be an appropriate therapeutic option for this case.

Phacoemulsification surgery and foldable intraocular lens implantation in a child with regressed retinoblastoma.

PURPOSE: The management of retinoblastoma has been shifting toward conservative treatment. Visual preservation has become a concern as tumor re g ression is achieved. To report the surgical approach and outcome of a radiation-induced cataract in an only eye with regressed retinoblastoma. METHODS: Single case report and review of literature. RESULTS: Small incision sutureless phacoemulsification and implant of an acrylic foldable intraocular lens was performed. Best-corrected visual acuity 1 year after surgery was 20/20. No tumor recurrence has been noted after 27 months of follow-up. CONCLUSIONS: Phacoemulsification and foldable iintraocular lens implant seemed to be an appropriate therapeutic option for this case.

Bilateral ectopia lentis as a presenting feature of medulloepithelioma.

Ectopia lentis may be a feature of numerous systemic and ocular disorders. Kivela and Tarkkanen described an 8-year-old girl with medulloepithelioma who presented with ectopia lentis and a mass behind the temporal iris. Shields reported 2 children with medulloepithelioma who had ectopia lentis associated with neovascular glaucoma. To date, there has been no report of a child with ectopia lentis as the only presentation of an intraocular tumor. We present 2 children with malignant medulloepitheliomas who presented in this fashion.

The outcome of the functioning filter after subsequent cataract extraction.

OBJECTIVE: To evaluate and compare the outcome of functioning filtration surgery followed by cataract surgery with posterior intraocular lens implantation by both phacoemulsification and extracapsular cataract extraction (ECCE) techniques in glaucomatous eyes. PATIENTS AND METHODS: We retrospectively evaluated the clinical course of 77 eyes (68 patients) that after successful trabeculectomy, underwent cataract surgery by either phacoemulsification or ECCE techniques. We determined the frequency of partial and absolute failure following cataract surgery by either phacoemulsification or ECCE in eyes with functioning trabeculectomies. Partial failure of intraocular pressure (IOP), control after cataract extraction was defined as the need for an increased number of antiglaucoma medications or argon laser trabeculoplasty to maintain IOP < or =21mm Hg. Complete failure of IOP control after cataract surgery was defined as an IOP >21 mm Hg on at least two consecutive measurements one or more weeks apart or the performance of additional filtration surgery. Failure rates were calculated using the Kaplan-Meier actuarial method. Failure rates between phacoemulsification and ECCE subgroups were compared using the log rank test. RESULTS: The probability of partial failure by the third postoperative year after cataract surgery was 39.5% in the phacoemulsification subgroup and 37.3% in the ECCE subgroup. This small difference is not statistically significant (P = 0.48). The probability of complete failure by the fourth postoperative year after cataract surgery was 12.0% in the phacoemulsification subgroup and 12.5% in the ECCE subgroup. This difference is also not statistically significant (P = 0.77). At the 6-month follow-up visit, visual acuity of both groups improved one or more lines in 87.0% of patients, and worsened one or more lines in 3.9% of patients. Sixty-one percent achieved visual acuity of 20/40 or better. The most frequent complication was posterior capsular opacification requiring laser capsulotomy that occurred in 31.2% of patients. CONCLUSION: Cataract extraction by either phacoemulsification or ECCE following trabeculectomy surgery may be associated with a partial loss of the previously functioning filter and the need for more antiglaucoma medications to control IOP.

Outcome of trabeculectomy with mitomycin-C in the iridocorneal endothelial syndrome.

PURPOSE: Eyes with iridocorneal endothelial (ICE) syndrome have a high risk of failure in glaucoma filtering surgery failing. We investigated the efficacy of trabeculectomy with intraoperative mitomycin-C application in these patients. DESIGN: Retrospective nonrandomized comparative trial with historical controls. PARTICIPANTS AND CONTROLS: Ten patients with unilateral iridocorneal endothelial (ICE) syndrome were reviewed. Their intraocular pressures could not be controlled medically. In five eyes, this was the primary surgery performed. Five of the patients had undergone prior intraocular pressure-(IOP) lowering surgery that had failed at the time enrolled. Results were compared with previously published case series of similar patients treated with trabeculectomy alone or trabeculectomy and subconjunctival 5-fluorouracil injections. INTERVENTION: Intervention consisted of trabeculectomy with a limbus-based conjunctival flap and mitomycin-C application. The dosage of mitomycin-C was 0.4 mg/ml for 1 to 4 minutes (mean, 1.9 min). MAIN OUTCOME MEASURES: Adequate control of IOP (without medication lower than 21 mm Hg). RESULTS: In eight eyes the IOP remained well controlled (mean IOP, 12.1 mm Hg) over the entire length of available of follow-up (mean, 14.9 months). Two eyes required implantation of an aqueous tube shunt at 4 and 11 months, respectively, after trabeculectomy with mitomycin-C. One eye experienced visual loss of 3 Snellen lines because of hypotony maculopathy. CONCLUSIONS: Trabeculectomy with mitomycin-C application offers a reasonable intermediate-term success rate in ICE patients, who are otherwise at high risk for failure of filtering surgery.

A new approach to an old problem.

A patient with progressive visual loss was found to have an optic nerve sheath meningioma. The patient was treated with stereotactic radiotherapy, a computer-guided stereotactic technique that minimizes the risk of radiation-induced optic neuropathy. Six months after treatment, the patient was doing well and showed no signs of radiation-induced optic neuropathy.

Filtration procedures supplemented with mitomycin C in the management of childhood glaucoma.

AIMS: To evaluate the outcome of filtering procedures supplemented with mitomycin C in children with glaucoma. METHODS: All patients aged 17 or younger with glaucoma who underwent filtering surgery supplemented with mitomycin C at a tertiary care centre (n = 21) during a 5 year interval (1992 and 1996) were included. One eye for each patient was entered into the analysis. The postoperative intraocular pressure (IOP), use of antiglaucoma medications, clinical stability of glaucoma, complications, and visual acuity were retrospectively evaluated. Kaplan-Meier survival curves were used to estimate the probability of success. RESULTS: At the time of surgery mean age was 5.7 (SD 5.0) years. The most common diagnoses were trabeculodysgenesis (n = 6) and aphakic glaucoma (n = 8). Mean IOP before surgery was 35.7 (10.5) mm Hg. Average length of follow up was 18.6 (14.7) months. The probability of having IOP less than 21 mm Hg with no antiglaucoma medications and with clinically stable glaucoma 1 year after surgery was 76.9% in phakic eyes (n = 13) and 0% in aphakic eyes (n = 8). A phakic patient with Sturge-Weber's syndrome had choroidal effusion after surgery that resolved spontaneously. In the aphakic group one patient had retinal detachment and another developed an encapsulated bleb. Visual acuity deteriorated in one patient. CONCLUSION: A guarded filtration procedure with mitomycin C is relatively successful in phakic children with glaucoma, but unsuccessful in aphakic ones.

Survival following enucleation versus plaque radiotherapy in statistically matched subgroups of patients with choroidal melanomas: results in patients treated between 1980 and 1987.

BACKGROUND: The relative effectiveness of plaque radiotherapy and enucleation in patients with choroidal or ciliochoroidal melanoma remains controversial. Most published comparative survival studies have evaluated clearly biased groups, short-term follow-up, or both. METHODS: The authors performed a retrospective, non-randomized, comparative survival study on 734 patients with a choroidal or ciliochoroidal melanoma treated by enucleation (n=237) or plaque radiotherapy (n=497) between July 1980 and June 1985, and on a residual group of 448 patients, 103 treated by enucleation and 345 treated by plaque radiotherapy, identified by variable-by-variable range matching. Melanoma-specific mortality and all-cause mortality were evaluated by the life-table method. RESULTS: The melanoma-specific mortality rate was substantially worse in the original enucleation subgroup throughout available post-treatment follow-up; however, differences in baseline prognostic factors between the subgroups are likely to explain much of the survival differential. After eliminating patients with nonoverlapping values of individual clinical variables to adjust for recognized intergroup differences at baseline, there was no significant difference in the mortality curves of the residual subgroups. The cumulative 15-year survival based on deaths from metastatic melanoma in the enucleation subgroup was 57.1% (standard error=6.4%), while that in the plaque radiotherapy subgroup was 61.8% (standard error=3.3%). CONCLUSION: On the basis of these results, we conclude that a large difference in survival between equivalent groups of patients with primary choroidal or ciliary body melanoma treated by enucleation or by plaque radiotherapy appears unlikely.

Independent diagnostic value of fluorescein angiography in the evaluation of intraocular tumors.

BACKGROUND: Fluorescein angiography has been used in the clinical evaluation of suspected neoplastic lesions of the ocular fundus for over 30 years. Yet, the independent diagnostic value of this photographic technique in patients with suspected intraocular neoplasms has never been determined. METHODS: The authors evaluated color fundus photographs and fluorescein angiograms of 50 mass lesions of the ocular fundus. The cases were chosen to reflect a broad spectrum of lesions, including choroidal malignant melanoma, choroidal nevus, circumscribed choroidal hemangioma, metastatic carcinoma to the choroid, miscellaneous other neoplasms, hamartomas, or choristomas, and non-neoplastic lesions simulating neoplasms. Ten experienced retinal specialists independently reviewed the angiograms (presented in random order without the corresponding color fundus photographs) and rendered a diagnosis. Approximately 1 month later, each retinal specialist independently reviewed the color fundus slides (presented in a different random order without the corresponding fluorescein angiograms) and again rendered a diagnosis for each lesion. RESULTS: The accuracy of angiographic diagnosis by the different reviewers ranged from 16% to 56% (average 45.4%), while that based on review of the color fundus slides ranged from 32% to 78% (average 59.0%). This difference is statistically significant. CONCLUSION: Fluorescein angiography alone did not appear to be a reliable method for establishing the clinical diagnosis of neoplasms and simulating lesions of the ocular fundus.

Comparison between laser scanning tomography and computerised image analysis of the optic disc.

AIMS: To study the interchangeability of the measurements of the optic disc topography obtained by one computerised image analyser and one confocal laser tomographic scanner. METHODS: One eye of 28 patients with glaucoma or glaucoma suspects was studied. All cases had simultaneous stereoscopic disc photographs taken with the fundus camera Topcon TRC-SS and optic disc examination with the Heidelberg retina tomograph (HRT) during the same visit. The optic disc photographs were digitised and analysed with the Topcon ImageNet (TI) system. Three variables of the optic disc topography provided by the TI and the HRT were compared--cup volume (CV), rim area (RA), and cup area to disc area ratio (CA/DA). RESULTS: The mean values of CV and RA provided by the TI (0.52 (SD 0.32) mm3 and 1.58 (0.39) mm2, respectively) were greater (p < 0.01) than the mean values of CV and RA determined by the HRT (0.32 (0.25) mm3, and 1.33 (0.47) mm2, respectively). The mean value of CA/DA provided by the TI (0.42 (0.14)) and the HRT (0.42 (0.18)) was similar (p = 0.93). Correlation coefficients between measurements obtained by the two methods ranged from 0.53 to 0.73. CONCLUSION: There was a significant discrepancy in the measurements of rim area and cup volume of the optic disc obtained by a computerised image analyser and a laser scanning tomograph.

Long-term outcome of aqueous shunt surgery in ten patients with iridocorneal endothelial syndrome.

PURPOSE: To report the long-term outcome of ten patients with iridocorneal endothelial (ICE) syndrome who underwent aqueous shunt surgery for uncontrolled glaucoma. DESIGN: Noncomparative, retrospective case series. PARTICIPANTS: The authors reviewed charts of ten patients with ICE syndrome-related glaucoma who underwent aqueous shunt surgery at one institution between 1987 and 1996. MAIN OUTCOME MEASURES: Intraocular pressure (IOP), number of glaucoma medications, and further surgical interventions were measured. RESULTS: With a median follow-up of 55 months, four eyes had adequate IOP control (IOP <21 mm Hg) with one or two medications after the initial aqueous shunt surgery. An additional three eyes achieved adequate IOP control after one or more tube repositionings or revisions of the initial aqueous shunt. In this series, the aqueous shunt surgery most often failed because of blocking of the tube ostium by iris, ICE membrane, or membrane-induced tube migration. CONCLUSION: Aqueous shunt surgery appears to be an effective method for IOP lowering in some eyes with ICE syndrome-related glaucoma when medical treatment or conventional filtration surgeries fail, but additional glaucoma procedures and/or aqueous shunt revisions and tube repositionings are not uncommon.

Invasive giant cell astrocytoma of the retina in a patient with tuberous sclerosis.

OBJECTIVE: To report an unusual case of giant cell astrocytoma of the retina. DESIGN: Case report. INTERVENTION: A 10-month-old girl with tuberous sclerosis was found to have bilateral astrocytic hamartomas, the right eye being prominently involved by elevated and pedunculated lesions. At 7 years of age, she had posterior subcapsular cataract, retinal detachment, and subretinal exudation develop in the right eye. At 12 years of age, her blind, painful right eye had to be enucleated because of neovascular glaucoma and a spontaneous scleral perforation. RESULTS: Histopathologic examination showed that the entire vitreous cavity was filled with a mixture of tumor, granulation tissue, and necrotic debris. Part of the tumor was composed of spindle-shaped glial cells. The remainder was composed of large gemistocytic cells that contained large atypical nuclei and copious amounts of cytoplasm, which was intensely eosinophilic in some areas. The tumor contained foci of necrosis and rare mitotic figures. It had infiltrated the parenchyma of the retrolaminar nerve and extended to the surgical margin. Areas of unequivocal choroidal invasion were also identified. The tumor cells were intensely immunoreactive for neuron-specific enolase and S-100 protein. In contrast, glial fibrillary acidic protein was only minimally positive. CONCLUSIONS: The histologic and immunohistochemical features of this retinal tumor resemble those of subependymal giant cell astrocytoma, a characteristic lesion in tuberous sclerosis. Although this unusual giant cell astrocytoma of the retina had atypical histopathologic features and local aggressive behavior, the systemic prognosis was excellent.

Oral prednisone in guarded filtration procedures supplemented with antimetabolites.

BACKGROUND AND OBJECTIVE: To evaluate whether a three-day course of oral prednisone perioperatively improves the surgical outcome of guarded filtering procedures supplemented with antifibrosis agents. DESIGN, MATERIALS AND METHODS: A prospective, randomized, double-masked, placebo-controlled, clinical trial was designed. Adult patients with non-inflammatory glaucoma undergoing a guarded filtration procedure supplemented with antimetabolite were enrolled. Patients received a three-day course of prednisone (50 mg BID) or placebo perioperatively. The main outcome measures were intraocular pressure (IOP) and number of antiglaucoma medications. Surgical success was defined before data collection according to two different criteria: "success-I": IOP level < or = 15 mmHg with no more than one anti-glaucoma medication, and "success-II": IOP reduction of at least 20% of baseline level with no more than one antiglaucoma medication. RESULTS: Thirty-five subjects were enrolled. Seventeen patients were treated with prednisone and eighteen with placebo. Mean follow-up was 9.2 months +/-6.2 months. The probability of success-I at 9 months was 63.0% in the study group and 65.6% in the control groups (p>0.05). The probability of success-II at 9 months was 60.2% in the study group and 55.0% in the control groups, (p>0.05). The difference in frequency of postoperative complications between groups was not statistically significant. The most common complication was choroidal detachment (n=2) in the prednisone-treated group and bleb leak (n=2) in the control group. CONCLUSION: The perioperative use of oral prednisone did not alter the surgical outcome of filtering procedures associated with antifibrosis agents in this population of glaucoma patients.

Early-onset scleral necrosis after iodine I 125 plaque radiotherapy for ciliochoroidal melanoma.

A 62-year-old man with a large ciliochoroidal melanoma developed early-onset scleral necrosis with tumor extrusion within 1 month of epibulbar iodine I 125 plaque radiotherapy. The eye was enucleated. Pathologic study revealed nonmicrobial scleral necrosis with extrusion of histologically intact and necrotic uveal melanoma cells. The patient has been followed up for 15 months without clinical recurrence. We discuss possible mechanisms to explain the early development of scleral necrosis after plaque therapy in this patient.

Ultrasound biomicroscopy as a tool for detecting and localizing occult foreign bodies after ocular trauma.

OBJECTIVE: To show the utility of ultrasound biomicroscopy (UBM) in imaging small ocular foreign bodies of the anterior segment. DESIGN: Retrospective case series. PARTICIPANTS: Twelve eyes of 12 consecutive patients evaluated in the emergency department or referred to specialty services at 1 institution between August 1994 and November 1997 were examined. INTERVENTION: Ocular ultrasound biomicroscopy was performed. MAIN OUTCOME MEASURES: Detection and localization of an ocular foreign body were measured. RESULTS: An intraocular or superficial foreign body was detected by UBM in 9 (75%) of 12 eyes. The foreign body was classified as corneal in two eyes, subconjunctival in two, intrascleral in three, and intraocular in two eyes. The foreign body was not visible by ophthalmic physical examination in seven of the nine eyes with a confirmed ocular foreign body. In the remaining two eyes, UBM was used to determine the depth of a visible foreign body. In three of the eyes with a confirmed foreign body, computed tomography and/or contact B-scan ultrasonography was obtained and failed to show a foreign body. Six of the foreign bodies were nonmetallic. CONCLUSIONS: Clinical detection of ocular foreign bodies after trauma can be hindered by small size, haziness of the optical media, poor patient cooperation, or hidden location. Ultrasound biomicroscopy is a valuable adjunct in the evaluation of suspected ocular foreign bodies, especially in cases involving small, nonmetallic objects.

The role of ultrasound biomicroscopy in surgical planning for limbal dermoids.

PURPOSE: Limbal dermoids usually involve corneal stroma and more rarely might extend into the anterior chamber. Depending on the size and amount of penetration of the lesion, different therapeutic approaches are indicated. Depth information is difficult to obtain by slit-lamp examination because the structure is opaque. We investigated the use of ultrasound biomicroscopic (UBM) examination for surgical planning in limbal dermoids. METHODS: The UBM (Zeiss Humphrey) with its 50-MHz probe was used for preoperative evaluation of the depth of penetration in two cases of infantile limbal dermoid. Histology specimens of the lesion corresponding to the UBM images were obtained. RESULTS: UBM was able to distinguish normal cornea from the more sonolucent lesion. Presence or absence of Descemet's membrane could be identified. Depth of involvement of limbal dermoids could be assessed. CONCLUSION: UBM is able to assess depth involvement of opaque corneal lesions such as limbal dermoids. Because planning of the surgical approach in these cases is facilitated by preoperative knowledge about the depth of penetration of these opaque lesions, UBM can be regarded as a helpful tool in the clinical management.