Diana Shechtman

Review and update: Current treatment trends for patients with retinitis pigmentosa

BACKGROUND Retinitis pigmentosa (RP) is a heterogeneous group of inherited retinal disorders characterized by progressive photoreceptor apoptosis. It is the leading cause of inherited retinal degeneration-associated blindness. RP has a unique set of clinical characteristics that make it a complex disease associated with distinct inheritance patterns. An understanding of the pathogenesis is essential in the process of the differential diagnosis and the development of treatment options. Recent developments in research are likely to expand the various therapeutic modalities to include gene therapy, pharmacologic treatment, cell transplantation, and neuro-prosthetic devices. METHODS A literature search was performed to comprehensively review RP diagnosis, pathophysiology, and treatment. CONCLUSION Advances in the understanding of the pathophysiology of RP are creating new opportunities for the treatment of this often visually debilitating eye condition. Optometrists, as primary eye care practitioners, should be aware of the inheritance, pathophysiology, and current treatment options for RP as well as treatments in development so that they can best care for their patients with inherited retinal disorders.

The expanding spectrum of vitreomacular traction

BACKGROUND Vitreomacular traction (VMT) syndrome is characterized as a partial detachment of the posterior vitreous with persistent adherence to the macula. The dynamic process associated with macular traction may induce a variety of macular conditions including cystoid macular edema (CME), epiretinal membranes (ERM), and macular hole formation. METHODS Dilated fundus evaluations as well as Stratus and Cirrus optical coherence tomography (OCT) (Zeiss-Humphrey, Dublin, California) imaging were used to evaluate patients with various maculopathies associated with VMT. CONCLUSION The use of the OCT has enhanced the evaluation of the vitreal-retinal interface, leading to a better understanding of VMT. Once thought to be a rare distinct clinical entity, VMT is now considered a spectrum of macular diseases. The understanding of VMT and its role in the pathophysiology of various macular conditions may facilitate diagnosis and management of these conditions.

Maximum Angle of Ocular Duction During Visual Fixation as a Function of Age

Purpose: To measure and compare the maximum angle of ocular duction in healthy individuals as a function of age. Methods: A calibrated arc perimeter was modified to display one of six randomly presented targets (high contrast Snellen equivalent letters), in both vertical (supra/infraduction) and horizontal (ab/adduction) gaze to the dominant eye of 204 healthy volunteers with best-corrected visual acuity. A bite-bar and headrest were employed to prevent head movement. Using a modified method of limits for discrimination threshold, a maximum mean angle of ocular duction was determined by stepping a target out in 5° steps until an error was reported and thereafter bracketing around the limits of the target identification in 1° steps. A mean threshold value was determined as the angle at which a subject obtained a correct response 75% of the time in two and as many as three trials in each of four randomly presented directions of gaze (abduction, adduction, supraduction and infraduction). Results: A decrease in mean maximum duction angle was found over all age groups in all four directions (p < 0.001), with a steep decline beginning in the sixth decade and almost doubling in the oldest age group tested (80–95 year-olds). The percent of change in mean maximum angle of duction due to age from the 14–19 to the 80–95 year-olds was: abduction 21%, adduction 24%, supraduction 35%, infraduction 26%. Conclusion: Baseline data are useful to differentiate normal changes occurring with age from early signs of disease. Additionally, disease progression and effects of treatment can be monitored.

Hemorrhagic complications of optic disc drusen and available treatment options.

BACKGROUND Optic disc drusen typically are considered benign findings. However, optic disc drusen can manifest with hemorrhagic complications. CASE REPORT A dilated fundus examination on a 27-year-old visually asymptomatic optometry student found a juxtapapillary hemorrhage one quarter of a disc diameter in size in the right eye, immediately off the superior nasal aspect of the disc. Bilateral disc drusen, confirmed by hyperreflectivity on ultrasonography, were also noted. Fluorescein angiography and optical coherence tomography were diagnostic only for a deep isolated intra- and subretinal hemorrhage. Neither diagnostic modality could confirm the presence or absence of a choroidal neovascular membrane. Because of the asymptomatic nature, location of the hemorrhage, and lack of conclusive etiology, close monitoring was recommended. After 4 months, 75% of the hemorrhage had reabsorbed with no visual complications. CONCLUSIONS Optic disc drusen can cause ocular hemorrhages through several mechanisms. Although many are benign and self-limiting, it is important to understand available treatment modalities should vision be threatened.

Laser scanning confocal ophthalmoscopy and polarimetry of human immunodeficiency virus patients without retinopathy, under antiretroviral therapy.

Purpose. Confocal laser scanning ophthalmoscopy (HRT; Heidelberg retinal tomograph II) and scanning laser polarimetry (GDx-variable corneal compensator [VCC]) were used to investigate whether early indicators of retinal nerve fiber layer (RNFL) thickness loss could be observed in patients infected with the human immunodeficiency virus (HIV) that had no associated retinopathy or optic neuropathy and were concomitantly receiving antiretroviral medications. Methods. HRT and GDx-VCC parameters obtained from a group of 13 HIV-positive subjects (n = 26 eyes) on antiretroviral therapy examined with HRT, with a subgroup of six subjects (n = 12 eyes) examined with both HRT and GDx-VCC, were compared with those of a matched HIV-negative control cohort (13 subjects, n = 26 eyes) examined with HRT, with a subgroup of five subjects (n = 10 eyes) examined with both HRT and GDx-VCC. We employed generalized estimating equations for statistical analysis. Results. Reduced mean values for the HRT height variation contour (p < 0.045) and HRT mean RNFL thickness (p < 0.023) were observed in HIV-positive subjects controlling for age, sex, and race. A significantly reduced mean value corresponding to the GDx-VCC superior maximum (p < 0.014) and inferior maximum (p < 0.016) were also observed for the HIV-positive cohort analyzed controlling for age, sex, and race. Conclusion. HRT and GDx-VCC indicators of RNFL thickness appear to be significantly reduced in HIV-positive subjects without retinopathy or optic nerve disease using antiretroviral medication, suggesting RNFL loss occurs in this population of HIV-positive patients. The lack of correlation between CD4 counts, viral load, number of antiretroviral medications used, or years from diagnosis of HIV and RNFL thinning, suggests that possibly other factors associated with HIV infection may contribute to the apparent RNFL thickness loss.

Pupil sparing incomplete third nerve palsy secondary to a cavernous sinus meningioma: challenges in management

Background:  The diagnosis of incomplete third nerve palsy can be clinically challenging because the aetiologies, as well as presentations, can be variable and subtle. The optometric clinician should be familiar with the association of third nerve palsy with compressive lesions, including the clinical presentations and management of these patients.

Idiopathic polypoidal choroidal vasculopathy (IPCV) presenting with simultaneous choroidal neovascular membrane (CNM).

Idiopathic polypoidal choroidal vasculopathy (IPCV), a rare retinal condition initially described in 1982, is characterized by retinal pigment epithelial (RPE) detachments associated with choroidal polypoidals. Although it is recognized as a unique entity, many consider it a peculiar representation of choroidal neovascular membrane (CNM), commonly associated with age-related macular degeneration (ARMD). We report a case of IPCV with simultaneous presentation of CNM. Dilated examination and fluorescein angiography (FA) revealed RPE detachments associated with choroidal polypoidals. FA also revealed a lacy hyperfluorescent vascular lesion. Ocular manifestations, differential diagnoses, and treatment options are discussed, with emphasis on similarities and differences between IPCV and CNM. It is imperative to consider IPCV in the differential diagnosis of RPE detachments, including those associated with CNM. Careful funduscopic evaluation, FA, and/or indocyanine green videoangiography analysis helps confirm the diagnosis.

Effect of Osteopathic Cranial Manipulative Medicine on Visual Function

CONTEXT The effects of osteopathic cranial manipulative medicine (OCMM) on visual function have been poorly characterized in the literature. Based on a pilot study conducted by their research group, the authors conducted a study that examined whether OCMM produced a measurable change in visual function in adults with cranial asymmetry. STUDY DESIGN Randomized, controlled, double-blinded clinical trial. The intervention and control (sham therapy) were applied during 8 weekly visits, and participants in both groups received 8 weekly follow-up visits. PARTICIPANTS Adult volunteers aged between 18 and 35 years with unremarkable systemic or ocular history were recruited. Inclusion criteria were refractive error between 6 diopters of myopia and 5 diopters of hyperopia, regular astigmatism of any amount, and cranial somatic dysfunction. INTERVENTION All participants were evaluated for cranial asymmetry and randomly assigned to the treatment or sham therapy group. The treatment group received OCMM to correct cranial dysfunctions, and the sham therapy group received light pressure applied to the cranium. OUTCOME MEASURES Preintervention and postintervention ophthalmic examinations consisted of distance visual acuity testing, accommodative system testing, local stereoacuity testing, pupillary size measurements, and vergence system testing. A χ2 analysis was performed to determine participant masking. Analysis of variance was performed for all ophthalmic measures. RESULTS Eighty-nine participants completed the trial, with 47 in the treatment group and 42 in the sham therapy group. A hierarchical analysis of variance revealed statistically significant within-groups effects (P<.05) from before the intervention to visit 16 in distance visual acuity of both eyes, local stereoacuity, Donder pushup in both eyes, and near point of convergence break and recovery. For treatment group vs sham therapy group, a statistically significant effect (P<.05) was observed from before the intervention to visit 16 in pupillary size under bright light in the left eye and in near point of convergence break. CONCLUSION Osteopathic cranial manipulative medicine may affect visual function in adults with cranial asymmetry. Active motion testing of the cranium for somatic dysfunction may affect the cranial system to a measurable level and explain interrater reliability issues in cranial studies. (ClinicalTrials.gov number NCT02728713).

Achromatopsia: case presentation and literature review emphasising the value of spectral domain optical coherence tomography

A literature review and case presentation are used to discuss the diagnostic value of spectral domain optical coherence tomography (SD-OCT) in the assessment and management of congenital achromatopsia. A 24-year-old Hispanic man presented to the clinic with a longstanding history of decreased vision and associated possible recent progression. A comprehensive eye examination and a battery of tests including SD-OCT, fundus photography, electroretinogram (ERG) and Farnsworth D-15 were completed. SD-OCT and photopic ERG confirmed the clinical diagnosis of congenital achromatopsia. There was the classic subfoveal flattened hyporeflective ‘punched out’ zone, resulting from an absence of inner segment/outer segment junction. SD-OCT findings associated with congenital achromatopsia have been documented recently, helping in the diagnosis of the condition. The SD-OCT findings have further expanded our knowledge of congenital achromatopsia, while also aiding in the management of the disease.A literature review and case presentation are used to discuss the diagnostic value of spectral domain optical coherence tomography (SD‐OCT) in the assessment and management of congenital achromatopsia. A 24‐year‐old Hispanic man presented to the clinic with a longstanding history of decreased vision and associated possible recent progression. A comprehensive eye examination and a battery of tests including SD‐OCT, fundus photography, electroretinogram (ERG) and Farnsworth D‐15 were completed. SD‐OCT and photopic ERG confirmed the clinical diagnosis of congenital achromatopsia. There was the classic subfoveal flattened hyporeflective ‘punched out’ zone, resulting from an absence of inner segment/outer segment junction.

Pupillometry Study of Brimonidine Tartrate 0.2% and Apraclonidine 0.5%

This study investigated possible effects of brimonidine tartrate 0.2% and apraclonidine 0.5% on pupil diameter. Ten subjects between 20 and 40 years of age participated. A Colvard pupillometer (Oasis Medical) was used to measure pupil diameter. Baseline and serial measurements were obtained at 3 luminance levels (>6.4, <0.82–0.4, and <0.2–0.02 cd/m2) during a 4‐hour interval following instillation of 1 drop of brimonidine tartrate 0.2% or apraclonidine 0.5% in one eye versus a placebo in the contralateral eye. The measurements for each drug were obtained on different days. A nested random effects model controlling for subjects age, race, and sex was used for statistical analysis. A maximum reduction in pupil diameter was observed at 90 minutes from instillation (1.40 mm at >6.4 cd/m2, 1.69 mm at <0.82–0.4 cd/m2, and 1.55 mm at <0.2–0.02 cd/m2) for brimonidine tartrate 0.2%. At all time intervals and illumination levels, miosis (P < .01) occurred. Apraclonidine 0.5% did not produce a significant effect on pupil diameter. Brimonidine tartrate 0.2% produced a moderate miotic effect. No effect was observed for apraclonidine 0.5%. A predominant agonistic effect on α‐2 receptors of the iris dilator may explain this behavior.