Serous macular detachment in Waldenström macroglobulinemia: a report of four cases.

PURPOSE: To describe a series of 4 patients with Waldenström macroglobulinemia and serous macular detachment, and propose a mechanism for development of subretinal fluid based on optical coherence tomography (OCT) findings. DESIGN: Retrospective observational case series. METHODS: The records of patients with Waldenström macroglobulinemia and OCT documentation of serous macular detachment at Wills Eye Institute were reviewed. Data collection included clinical examination, as well as findings on fluorescein angiography (FA) and OCT. RESULTS: Four patients (8 eyes) with Waldenström macroglobulinemia and serous retinal detachment were identified. All eyes had varying degrees of venous stasis retinopathy and intraretinal edema overlying the macular detachment. Three patients had no FA leakage, while 1 patient had macular leakage in a petaloid pattern. Focal outer retinal defects within the detached retina were seen in 4 eyes on OCT imaging. In one eye, development of cystoid macular edema was observed before the outer retinal defect and serous macular detachment. All patients with serous macular detachment had some degree of outer retinal disruption. CONCLUSION: Discontinuity of the outer retina within the macular detachment may enable immunoglobulins along with accumulated intraretinal fluid to flow into the subretinal space, creating a serous retinal detachment. Even with systemic treatment of the underlying Waldenström macroglobulinemia, the visual prognosis was guarded.

Intravitreal bevacizumab for choroidal neovascularization associated with choroidal nevus.

PURPOSE: To report 10 cases of occult choroidal neovascularization (CNV) associated with choroidal nevus managed with intravitreal bevacizumab. METHODS: Interventional case series. Each nevus was examined and imaged with fluorescein angiography, B-scan ultrasonography, and optical coherence tomography. Data were retrospectively analyzed to evaluate outcomes of treatment response and visual acuity. RESULTS: Nine patients presented with CNV overlying a chronic choroidal nevus with a posterior margin within 1.5 mm of the foveola. In the 10th patient, the posterior margin of the nevus was located 10 mm from the foveola with extension of subretinal fluid into the macula. The CNV was subfoveolar in four cases, juxtafoveolar in two cases, and extrafoveolar in four cases. Initial visual acuity was 20/20 to 20/50 in 5, 20/60 to 20/100 in 2, and 20/200 or worse in 3 cases. Clinical features included subfoveolar fluid in nine, exudation in five, and hemorrhage in four cases. Intravitreal bevacizumab (1.25 mg/0.05 cc) was injected with regression of CNV in all 10 cases using 2 to 14 injections (median 3 injections). In 2 eyes, after therapeutic response to bevacizumab later consolidation with photodynamic therapy (juxtafoveolar CNV) (n = 1) or conventional laser (extrafoveolar CNV) (n = 1) was provided. In the remaining 8 eyes, after discontinuation of bevacizumab, there was no recurrence of CNV over mean 10.1 months. At overall mean follow-up of 22.5 months, final visual acuity decreased by 1 line in 4 cases and improved by mean of 3 lines (range, 1-8 lines) in 6 cases. There were no adverse effects from bevacizumab injections. All 10 choroidal nevi remained stable. CONCLUSION: Intravitreal bevacizumab appears to be an effective treatment option for CNV secondary to choroidal nevus. In some cases, depending on the proximity of the CNV to the foveola, photodynamic therapy or conventional laser may be useful adjunctive therapy.

23-Gauge transconjunctival pars plana vitrectomy for removal of retained lens fragments.

PURPOSE: To examine the usefulness of 23-gauge pars plana vitrectomy (PPV) in removing retained lens fragments and to determine which cases require 20-gauge ultrasonic fragmentation. DESIGN: Retrospective comparative case series. METHODS: Twenty eyes in 20 consecutive patients who had 23-gauge PPV for retained lens fragments after complicated cataract surgery at Wills Eye Institute were retrospectively reviewed. Retained lens fragments were graded based on percentage of total nuclear size. Every surgical case started with 23-gauge PPV, but 1 sclerotomy was enlarged to facilitate 20-gauge ultrasonic fragmentation if all lens material could not be adequately removed. RESULTS: Twelve patients (60%) were successfully managed with 23-gauge PPV alone while 8 patients (40%) required 20-gauge ultrasonic fragmentation. The 23-gauge cases had a lower percentage of retained lens fragments (median 10%, range 0% to 75%) compared to the 20-gauge cases (median 90%, range 10% to 100%). CONCLUSION: 23-Gauge PPV is a feasible approach in the surgical management of select cases of retained lens fragments.

Optical coherence tomography imaging of the fovea in retinopathy of prematurity.

BACKGROUND AND OBJECTIVE: To report foveal optical coherence tomography (OCT) findings in patients with a history of retinopathy of prematurity (ROP). PATIENTS AND METHODS: In a case-control study, OCT imaging of the fovea was performed on mostly adult patients with a history of ROP and no significant macular pathology. The same OCT imaging was performed on control subjects who were born full-term. RESULTS: Fourteen eyes of 12 patients with ROP were studied. Mean age was 39 years and mean gestational age at birth was 28 weeks. Of the 11 eyes with no cataract or pseudophakia, median best-corrected visual acuity was 20/40. Three eyes with cataracts had visual acuities of 20/50, 20/60, and 20/100. OCT imaging showed that eyes in the ROP group had a relative loss of the foveal depression, increased macular thickness, and continuation of inner retinal layers within the fovea. CONCLUSION: OCT imaging demonstrated abnormal foveal architecture in patients with a history of ROP, but most of these patients maintained good visual acuity.

Ranibizumab for exudative age-related macular degeneration in eyes previously treated with alternative vascular endothelial growth factor inhibitors.

BACKGROUND AND OBJECTIVE: To evaluate ranibizumab for exudative age-related macular degeneration previously treated with pegaptanib, bevacizumab, or both. PATIENTS AND METHODS: This was a retrospective, interventional case series of patients with exudative age-related macular degeneration who were treated with ranibizumab after being initially treated with pegaptanib, bevacizumab, or both. The primary outcome was change in visual acuity following the switch to ranibizumab. RESULTS: One hundred two eyes of 92 patients were identified. Following the switch to ranibizumab, there was an average gain of 0.7 lines in visual acuity. Ninety-four eyes (92%) lost 3 or fewer lines, 29 eyes (28%) gained more than 3 lines, and 3 eyes (3%) lost more than 6 lines after switching to ranibizumab. Lesion type and time between previous vascular endothelial growth factor inhibitor and ranibizumab did not affect the response. CONCLUSION: Ranibizumab maintained visual acuity in the majority of patients and appears to be an effective treatment regardless of previous anti-vascular endothelial growth factor therapy.

Orbital burkitt lymphoma in immunocompetent patients: a report of 3 cases and a review of the literature.

PURPOSE: To describe a series of 3 immunocompetent patients with the sporadic form of orbital Burkitt lymphoma and review the outcomes of such patients reported in the scientific literature. METHODS: Retrospective review of medical records and the literature. Cases of orbital Burkitt lymphoma in immunocompromised and African patients were excluded from the review. Measured parameters included gender, age, ocular signs, imaging results, the range of systemic involvement, and treatment. RESULTS: Current cases and review of the scientific literature resulted in 16 immunocompetent patients with sporadic orbital Burkitt lymphoma. The median age at presentation was 12 years. Most common ocular signs at presentation were proptosis (13/16), external ophthalmoplegia (9/16), and eyelid edema (7/16). Optic neuropathy was noted in 5 of 16 patients. Concomitant paranasal sinus involvement was present in 8 of 16 patients. Fourteen (88%) of the patients had systemic involvement, of which the most common locations were central nervous system (6/16), lymphatics (6/16), bone marrow (6/16), and liver (4/16). Survival data were available for 13 patients. Seven patients (54%) died within 12 months of presentation. CONCLUSIONS: Sporadic orbital Burkitt lymphoma occurs in immunocompetent individuals with a wide age range. Fifty percent presented with adjacent paranasal sinus involvement. Concurrent or eventual systemic involvement is common. The large number of patients with central nervous system involvement in our review of the literature supports the role of lumbar puncture and neuroimaging in the workup of all patients with orbital Burkitt lymphoma. Prognosis remains guarded, with significant mortality within 1 year of presentation.

Stem-cell therapy in retinal disease.

PURPOSE OF REVIEW: Stem-cell research is being investigated for the treatment of retina diseases. Cell replacement strategies have the potential to improve vision in patients who were previously considered to be untreatable. This review summarizes progress within the field and obstacles which must be overcome to make stem-cell therapy a viable treatment for select retinal disease. RECENT FINDINGS: Researchers have demonstrated that stem-cell transplants can survive, migrate, differentiate, and integrate within the retina. Stem cells from various developmental stages have been used in these experiments, including embryonic stem cells, neural stem cells, mesenchymal stem cells, retinal stem cells, and adult stem cells from the ciliary margin. Not only can these transplants adopt retina-like morphologies and phenotypes, but they have also shown evidence of synaptic reconnection and visual recovery in both animal and human studies. Still, work must be done to achieve higher yields of functioning retinal neurons and to promote better integration within the host retina. SUMMARY: Although many obstacles remain, stem-cell-based therapy is a promising treatment to restore vision in patients with retina disease.

Optical coherence tomography of west african crystalline maculopathy.

PURPOSE: To describe a case of West African crystalline maculopathy and the optical coherence tomography (OCT) findings. METHODS: A 47-year-old West African woman presented with bilateral yellow-green refractile crystals clustered in the fovea. She had proliferative diabetic retinopathy and a long history of chewing kola nuts. Given her history and clinical examination findings, a diagnosis of West African crystalline maculopathy was made. RESULTS: OCT revealed that the foveal crystals were located in the innermost layers of the retina. CONCLUSIONS: OCT findings, along with history and examination, can be helpful in confirming the diagnosis of West African crystalline maculopathy.

Myopia in adults with retinopathy of prematurity.

PURPOSE: To evaluate myopia in adults with retinopathy of prematurity (ROP). DESIGN: Observational case series. METHODS: Axial length (AL), corneal radius (CR), and AL/CR ratio were measured in myopic adults with ROP using ultrasound A-scan biometry and keratometry, respectively. RESULTS: Twenty-five eyes of 18 patients (mean age, 48 years) were studied. Mean gestational age at birth was 29 weeks. The average refraction was -5.23 diopters, AL 24.43 mm, CR 7.45 mm, and AL/CR ratio 3.28. On average, myopic adults with ROP had a shorter AL and decreased CR (increased corneal curvature), but a similar AL/CR ratio, relative to adults with the same degree of myopia who were born full-term. Myopic adults with ROP also had increased lens thickness (LT) and shallow anterior chamber depth (ACD). CONCLUSIONS: Myopic adults with ROP had a shorter AL and smaller CR than expected for their degree of myopia. However, AL/CR ratios for these patients were similar to the values of adults who were born full-term. These results suggest that the refractive element most responsible for myopia in adults with ROP is higher corneal curvature (decreased CR), whereas the predominant factor in adult myopic individuals who were born full-term is increased AL. We believe that increased LT and shallow ACD make a lesser contribution to myopia in adults with ROP.