Long-term outcomes of Group D eyes in bilateral retinoblastoma patients treated with chemoreduction and low-dose IMRT salvage.

BACKGROUND: To evaluate outcomes of Group D eyes of bilateral retinoblastoma patients treated with primary chemoreduction and external beam radiation as salvage. PROCEDURE: Retrospective chart review of patients diagnosed with bilateral retinoblastoma and designated Group D in at least one eye from January 1, 2000 to December 31, 2009. Overall, 62 Group D eyes of 49 patients were included; 13 had bilateral Group D disease. Primary chemoreduction with vincristine, etoposide, and carboplatin with local consolidation was administered, followed by external beam radiation in the form of intensity-modulated radiation therapy (IMRT) as salvage for recurrent tumor. Primary outcome measure was globe salvage. RESULTS: Of 62 Group D eyes, 7 were enucleated primarily; 55 were treated with systemic chemoreduction, and local therapy. Chemoreduction cured 26 of 55 eyes (47%). Recurrences were found in 29 eyes; 5 underwent enucleation and 24 were treated with IMRT at a dose of 24 Gy (2 eyes) or 36 Gy (22 eyes). Of the 24 irradiated eyes, 19 (79%) were salvaged and 5 required enucleation. Final visual acuity ranged from 20/20 to light perception with 10 eyes having 20/80 or better visual acuity. Average follow-up was 54.2 months. CONCLUSION: Kaplan-Meier estimates of eye survival of Group D eyes in bilateral patients at 12 months is 82% (95% confidence interval [CI] 70.1-89.7%); at 60 months eye survival is estimated to be 68% (95% confidence interval [CI] 55.4-82.8%). Systemic treatment for retinoblastoma demonstrated a high rate of globe preservation with acceptable complications and many eyes retaining functional vision.

Bilateral oedipism.

A 20-year-old woman presented with self-inflicted pencil wounds to her bilateral orbits. CT angiography revealed penetration of pencils through optic canals into temporal lobes. Examination revealed bilateral no light perception, fixed dilated pupils, complete ptosis, and complete ophthalmoplegia. The patient was admitted to the psychiatric ward and her vision and cranial nerve deficits did not improve.

Unmet need for corrective eyeglasses: results from a Los Angeles County Hospital survey.

To investigate the socioeconomic challenges encountered by patients from Los Angeles County Ophthalmology Clinic, California, United States, in obtaining prescription eyeglasses. A retrospective survey study. Patients sent for refraction in June 2009 were asked to rate their satisfaction with their eyeglasses. If they did not obtain eyeglasses, they were asked the reason e.g., cost, lost prescription, etc. Fifty-five percent (54/99) of patients who received a prescription did not obtain eyeglasses. The most common reason was cost (67 %). Eighty-eight percent of patients had some form of health insurance; however, 100 % were lacking eyeglasses coverage. The mean visual acuity pre-refraction was 20/80 versus 20/30 post-refraction (p < 0.001). It is well known that refractive error is a major cause of blindness worldwide, which negatively impacts the patient and their community. However, it is infrequently addressed that these conditions persist in parts of the develop world, including the United States. Los Angeles County Hospital is an example of an environment with many low-income patients in need of refractive correction who are limited mainly by cost in attaining eyeglasses. Furthermore, insurance organizations that cover low-income patients provide minimal or no coverage for prescription eyeglasses.

Wavefront-guided photorefractive keratectomy with the VISX platform for myopia.

PURPOSE: To evaluate the safety, efficacy, and clinical outcome of wavefront-guided photorefractive keratectomy (PRK) for the treatment of low to moderate and high myopia and compound myopic astigmatism. METHODS: This retrospective study included 174 eyes of 102 patients with low to moderate (-0.125 to <-6.50 diopters [D]) and high (≥-6.50 D) myopia treated with wavefront-guided PRK using the VISX CustomVue S4 IR (Abbott Medical Optics) excimer laser. Eyes were analyzed preoperatively and at 1, 3, 6, and 12 months postoperatively. At 3 months, 174 eyes of 102 patients were evaluated, and at 12 months, 146 eyes of 75 patients were evaluated. No eyes were retreated in the study. RESULTS: Mean patient age was 38.3±8.76 years (range: 20 to 66 years), and mean preoperative manifest refraction spherical equivalent (MRSE) was -5.60±2.58 D (range: -0.13 to -10.50 D). At 12 months postoperative, MRSE was -0.16±0.45 D (range: -1.50 to 1.38 D); 96.6% of eyes were within ±1.00 D of intended correction, whereas 81.5% were within ±0.50 D of intended correction. For all eyes, the safety and efficacy indexes were 1.01 and 1.02, respectively. CONCLUSIONS: These data support the safety and efficacy for the correction of low to moderate and high myopia and compound myopic astigmatism using wavefront-guided PRK.

Wnt-mediated self-renewal of neural stem/progenitor cells.

In this work we have uncovered a role for Wnt signaling as an important regulator of stem cell self-renewal in the developing brain. We identified Wnt-responsive cells in the subventricular zone of the developing E14.5 mouse brain. Responding cell populations were enriched for self-renewing stem cells in primary culture, suggesting that Wnt signaling is a hallmark of self-renewing activity in vivo. We also tested whether Wnt signals directly influence neural stem cells. Using inhibitors of the Wnt pathway, we found that Wnt signaling is required for the efficient cloning and expansion of single-cell derived populations that are able to generate new stem cells as well as neurons, astrocytes, and oligodendrocytes. The addition of exogenous Wnt3a protein enhances clonal outgrowth, demonstrating not only a critical role for the Wnt pathway for the regulation of neurogenesis but also its use for the expansion of neural stem cells in cell culture and in tissue engineering.

Isolated trochlear palsy secondary to Lyme neuroborreliosis.

The objective of the study is to report the first case of isolated trochlear palsy secondary to Lyme neuroborreliosis in an adult. A 22-year-old male presented with history of flu-like illness and headache, accompanied by vertical binocular diplopia, worse on downgaze and better in upgaze and right head tilt. Physical examination revealed trochlear palsy on the left side with a compensatory head tilt to the opposite side. A subsequent workup for trochlear palsy was conducted, including hematological and cerebral spinal fluid serum studies, and magnetic resonance imaging of the brain. Immunoglobulin (Ig)M and IgG antibodies against Borrelia burgdorferi were positive in serum and cerebral spinal fluid (CSF). Symptoms of double vision completely resolved after 3 weeks of antibiotic treatment with intravenous ceftriaxone. CSF studies, in addition to clinical symptoms of vertical double vision and a positive Parks 3-step test, are a good diagnostic tool for B. burgdorferi. Resolution of symptoms was noted after a complete course of intravenous ceftriaxone.

Two cases of uveitis associated with severe transaminitis during a Rickettsia typhi outbreak in Los Angeles County.

PURPOSE: To report the clinical presentation, multimodal imaging, and management of two patients with Rickettsia typhi infection who presented with transaminitis and bilateral uveitis. OBSERVATIONS: We report two cases of murine typhus-associated uveitis in the setting of a Rickettsia typhi outbreak in Los Angeles County. In case 1, a 29-year-old Hispanic female presented with scotoma of the right eye and bilateral floaters after 2 weeks of persistent fevers, maculopapular rash, and arthralgia. Clinical examination and optical coherence tomography (OCT) revealed vitreous cell and scattered white spots in both eyes at the level of the inner retina, and a cotton wool spot inferiorly in the left eye. Multiple hyperautofluorescent spots were seen on widefield fundus autofluorescence (FAF). Retinal vascular leakage and optic disc hyperfluorescence were visualized on widefield fluorescein angiography (FA). These findings were concerning for a white dot syndrome (WDS). The patient was started on oral prednisone 30 mg daily. Serologic testing during the convalescent phase returned positive for R. typhi infection and she was started on doxycycline. 3 weeks later, she reported complete resolution of scotoma and significant improvement of bilateral floaters.In the second case, a 42-year-old Hispanic male presented with sudden bilateral increased floaters and blurry vision after 12 days of persistent fever and headache. Clinical examination revealed trace flare with 1+ cell in the anterior chamber, 1+ vitreous cell, and multiple white dots in both eyes at the level of the inner retina. FAF showed scattered hyperautofluorescent spots in both eyes. FA demonstrated late retinal vascular leakage with bilateral hyperfluorescent optic discs. He was started on oral prednisone 40mg, prednisolone acetate 1% drops, and cyclopentolate 1% drops daily. 2 weeks later, serologic titers returned positive for murine typhus and he was started on doxycycline with gradual taper off of steroids. He subsequently had complete resolution of floaters, blurry vision, and the inner retinal white spots. CONCLUSIONS AND IMPORTANCE: Murine typhus-associated uveitis may present with scotoma and increased floaters in the setting of persistent fevers and transaminitis, with pre- or inner retinal white spots seen on fundus examination. Ophthalmologists may aid in prompt diagnosis and initiation of antibiotic therapy, which can shorten the course of the disease and in turn, reduce the risk of severe complications.

Rifampin and linezolid in the treatment of methicillin-resistant Staphylococcus aureus preseptal cellulitis.

Methicillin-resistant Staphylococcus aureus (MRSA) preseptal cellulitis is an aggressive ophthalmic infection of increasing frequency. Previously reported cases were successfully treated with first line drugs such as vancomycin or Bactrim (trimethoprim and sulfamethoxazole); however, such drugs have limited efficacy in treating cutaneous MRSA. The authors report the first 2 known cases of MRSA-mediated preseptal cellulitis that resolved with systemic linezolid and rifampin following failed intravenous vancomycin treatment after incision and drainage of the abscess. The authors conclude that in cases of cutaneous MRSA infections that respond poorly to vancomycin, linezolid and rifampin combination therapy may provide an alternative therapeutic option.

Hematopoietic stem cell-derived pericytic cells in brain tumor angio-architecture.

Bone marrow-derived cells are recruited into tumor vasculature in response to angiogenic signals, and some of the cells within the newly forming tumor vessels are hematopoietic stem cells (HSCs) in origin. Previous studies suggest that bone marrow-derived pericytes are associated with newly formed vessels in tumors. In this study, we used an orthotopic rat glioma model (RT-2/RAG) to examine the contribution of long-term hematopoietic stem cell (LT-HSC)-derived pericytic cells to brain tumor angiogenesis. Mice (RAG-2/KO5.2) were lethally irradiated, and their hematopoietic cells were repopulated by transplantation of double fluorescence-activated cell-sorted LT-HSCs that express green fluorescent protein (GFP+). RT-2/RAG cells were then injected into the striatum of the chimeric mice 6 weeks post-transplantation. The animals were sacrificed 9 days after tumor implantation, and the incorporation and lineage-specific marker expression profile of the GFP+ cells within the growing tumor and tumor periphery were analyzed. LT-HSC-derived GFP+ cells were noted to incorporate onto the surface of tumor vessels within the perivascular space. LT-HSC-derived GFP+ cells express the pericyte progenitor marker, platelet-derived growth factor receptor-beta (PDGFR beta), as well as mature perictyte markers such as nerve/glial antigen 2 proteoglycan (NG2), alpha-smooth muscle actin (alpha SMA), and desmin. These LT-HSC-derived cells may represent a population of progenitor or committed pericytes within the neovascular tree and may play a role in shaping the angio-architecture in the vascular niche of brain tumors.

Visual outcomes of wavefront-guided laser in situ keratomileusis in eyes with moderate or high myopia and compound myopic astigmatism.

PURPOSE: To evaluate the safety, efficacy, and clinical outcomes of wavefront-guided laser in situ keratomileusis (LASIK) surgery for the treatment of moderate to high myopia and compound myopic astigmatism. SETTING: Stanford University School of Medicine, Department of Ophthalmology, Stanford, California, USA. METHODS: This retrospective study included patients with moderate (-6.0 to -8.0 diopters [D]) and high (greater than -8.00) myopia treated with wavefront-guided LASIK using the WaveScan linked to the CustomVue system (AMO USA, Inc.). Eyes were analyzed preoperatively and 1, 3, and 12 months postoperatively. RESULTS: The mean patient age was 38.4 years +/- 7.14 (SD). Eighty-nine eyes of 45 patients were evaluated at 3 months and 50 eyes of 25 patients at 12 months. No eye was retreated during the study. The mean manifest refraction spherical equivalent was -8.10 +/- 0.98 D (range -6.00 to -10.63 D) preoperatively and -0.33 +/- 0.55 D (range -1.625 to 1.375 D) 12 months postoperatively. Ninety percent of the eyes were within +/-1.00 D of the intended correction and 64.0%, within +/-0.50 D. For all eyes, the safety index was 1.00 and the efficacy index, 1.18. CONCLUSION: The data support the safety and efficacy of correcting moderate to high myopia and compound myopic astigmatism using wavefront-guided LASIK.

Wavefront-guided laser in situ keratomileusis retreatment for consecutive hyperopia and compound hyperopic astigmatism.

PURPOSE: To evaluate the efficacy, predictability, and safety of wavefront-guided laser in situ keratomileusis (LASIK) using the Visx CustomVue excimer laser (Advanced Medical Optics) in eyes with consecutive hyperopia and compound hyperopic astigmatism after LASIK. SETTING: Stanford University School of Medicine, Department of Ophthalmology, Stanford, California, USA. METHODS: This retrospective analysis included 19 eyes of 16 patients who had wavefront-guided LASIK for consecutive hyperopia and compound hyperopic astigmatism after initial LASIK surgery. Primary outcome variables, including uncorrected visual acuity (UCVA), best spectacle-corrected visual acuity (BSCVA), higher-order aberration (HOA) analysis, and spherical equivalence, were evaluated at 1 and 3 months. Nine eyes of 7 patients were available for all visits. RESULTS: The mean patient age was 51.7 years +/- 3.77 (SD) (range 44 to 55 years). The mean preoperative manifest refractive spherical equivalent (MRSE) was 0.99 +/- 0.32 diopters (D) (range 0.50 to 1.50 D) and the mean 3-month postoperative MRSE, -0.04 +/- 0.66 D (range -1.50 to 0.75 D). At 1 month, 57.9% of eyes had a UCVA of 20/20 or better and 78.9% of 20/25 or better; 84.2% were within +/-1.00 D of emmetropia. At 3 months, 66.7% of eyes had a UCVA of 20/20 or better and 88.9% of 20/25 or better; 88.9% were within +/-1.00 D of emmetropia. No eye lost 2 or more lines of BSCVA at 1 or 3 months. CONCLUSION: Wavefront-guided LASIK was an effective, predictable, and safe procedure for consecutive hyperopia and compound hyperopic astigmatism after LASIK.

Congenital glioblastoma multiforme: case report and review of the literature.

Congenital glioblastoma multiforme is a rare primary brain tumor that has a unique biology distinct from pediatric and adult variants. In this report, we present a case of congenital glioblastoma with complicated management course. A literature review of previously reported cases is included to illustrate the epidemiology and natural history of this disease. A 9-month-old male infant developed acute lethargy, hemiparesis and unilaterally dilated pupil. Imaging studies revealed a large hemispheric tumor, resulting in significant midline shift suggestive of impending herniation. Emergent tumor cystic fluid drainage was performed at initial presentation. A frontotemporoparietal craniotomy was performed on the following day to attempt a gross total resection. Adjuvant chemotherapy consisting of oral temozolomide was administered. The patient eventually succumbed 4 months later due to aggressive tumor progression. Congenital glioblastoma should be included in the differential diagnosis of infants with large intracranial tumors. Although surgical intervention may increase survival, the overall outcome remains poor despite maximal multimodal treatment.

Cystoid macular edema and macular pigmentation associated with nab-Paclitaxel therapy.

PURPOSE: To report the first known case of retinal pigment epithelium hyperpigmentation changes and cystoid macular edema in a patient on nab-paclitaxel therapy. METHODS: Observational case report. RESULTS: A 72-year-old man on nab-paclitaxel therapy for non-small cell lung carcinoma developed cystoid macular edema with minimal capillary leakage with subsequent retinal pigment epithelium hyperpigmentation after resolution of cystoid macular edema. CONCLUSION: Nab-paclitaxel therapy may be associated with the development of retinal pigment epithelium hyperpigmentation in the setting of resolved minimal capillary leakage cystoid macular edema.

Angioarchitectural evolution of clival dural arteriovenous fistulas in two patients.

Dural arteriovenous fistulas (dAVFs) may present in a variety of ways, including as carotid-cavernous sinus fistulas. The ophthalmologic sequelae of carotid-cavernous sinus fistulas are known and recognizable, but less commonly seen is the rare clival fistula. Clival dAVFs may have a variety of potential anatomical configurations but are defined by the involvement of the venous plexus just overlying the bony clivus. Here we present two cases of clival dAVFs that most likely evolved from carotid-cavernous sinus fistulas.

The utility of routine tuberculosis screening in county hospital patients with uveitis.

BACKGROUND/AIM: To evaluate the utility of tuberculosis (TB) screening in diagnosing ocular TB in uveitis patients in a government-funded hospital. METHODS: The charts of 142 consecutive patients seen during August 2011-July 2012 at the Los Angeles County Hospital uveitis clinic were reviewed for manifestation/laterality of uveitis, purified protein derivative (PPD) test results, interferon γ release assay, chest x-ray, birthplace, treatment history and diagnosis. 'Presumed TB-uveitis' was diagnosed when patients had positive TB screening and favourable response to anti-TB therapy, and definite ocular TB when Mycobacterium tuberculosis' presence was demonstrated. Post-test probabilities were determined. RESULTS: TB screening was positive in 21.1%. Six patients were diagnosed with TB-related uveitis: one definite, four presumed and one systemic TB with uveitis. With regard to PPD positivity, being foreign-born was the only statistically significant factor with OR of 2.26 (95% CI 1.01 to 5.13; p<0.01) if born in Mexico and 4.90 (95% CI 1.74 to 13.83; p<0.01) if born in other foreign countries. The post-test probabilities of a positive PPD in a uveitis patient showed a 17.2% (overall) or 30.3% (foreign-born patients) chance of ocular TB. CONCLUSIONS: PPD skin test plays an important role in the diagnosis of TB-associated uveitis in high-risk groups, such as immigrants from TB endemic regions.

Functional and visual improvement with prosthetic replacement of the ocular surface ecosystem scleral lenses for irregular corneas.

PURPOSE: To evaluate the Doheny Eye Institute Experience with Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) scleral lenses for the management of irregular corneas with outcomes based on visual acuity (VA) and visual function. METHODS: A retrospective chart review of 58 subjects (90 eyes) with irregular corneal surfaces referred to the Doheny Eye Institute for PROSE treatment between July 2009 and December 2011 was performed. The best-corrected VA before and after PROSE fitting was recorded. A functional assessment before and after PROSE fitting was also performed using the Ocular Surface Disease Index, a 12-item questionnaire that grades the severity of ocular discomfort and vision-related function. RESULTS: Keratoconus (43%) represented the largest group, and post-PK astigmatism (31%) represented the second largest group of patients with irregular corneas who had completed the PROSE treatment. Patients with keratoconus had the greatest improvement in VA after PROSE fitting with an 88% improvement in the logarithm of the minimal angle of resolution vision. Patients with post-PK astigmatism had the greatest improvement in Ocular Surface Disease Index scores with a 79% improvement observed after PROSE fitting. CONCLUSIONS: PROSE scleral lenses offer improvements in the VA and function, and they could be an option for patients with irregular corneas who have failed conventional treatments before considering additional surgery.

Reconstruction of total lower eyelid defects with the temporoparietal fascial flap.

Purpose. To describe the use of the temporoparietal fascial flap (TPF) in the reconstruction of extensive lower eyelid defects in a functioning eye. Methods. We present a surgical case report of a 73-year-old female with melanoma of the left lower eyelid. The lower eyelid was resected, and a composite nasal cartilage-mucosa graft, a skin graft, and a TPF were used to reconstruct the lower eyelid. Results. This achieved reconstruction of the lower eyelid with the protection of the eye and preservation of the visual system function. Conclusion. The TPF serves as a good option for reconstruction of the lower eyelid in a viable functioning globe.

Isolated abducens nerve palsy secondary to Lemierre syndrome.

Lemierre syndrome is characterized by pharyngotonsillitis that is typically secondary to Fusobacterium necrophorum infection and causes adjacent septic thrombophlebitis and thrombosis with subsequent metastatic abscesses. A 3-year-old boy presented with fever, tonsillar enlargement, and bilateral otomastoiditis with purulent discharge. Physical examination revealed abducens nerve palsy on the left side, with a compensatory left head turn. Otomastoid discharge culture was positive for anaerobic F. necrophorum. Magnetic resonance venography of the head and neck revealed thromboses in left internal jugular vein, left cavernous sinus, left superior ophthalmic vein, and left sigmoid sinus. The patient was treated with anticoagulation and a 10-week course of intravenous antibiotics, including ceftriaxone and metronidazole.