Andrew S. Gurwood

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.

Intravenous and indocyanine green angiography

BACKGROUND Indocyanine green angiography (ICGA) is an adjunct procedure to intravenous sodium fluorescein angiography (IVFA) for evaluation of disorders of the retina. It has particular application when choroidal pathology is implicated or suspected. Indocyanine green (ICG) dye has been used in the medical field since 1956, when it was approved by the Federal Drug Administration for imaging cardiac and hepatic circulations. Indocyanine green is a well-tolerated, tricarbocyanine dye with a molecular weight of 775 daltons that absorbs light at 790 to 805 nm and has a peak emission at 835 nm. While the substance gives off 4% the fluorescence of sodium fluorescein, its spectral properties allow it to be visualized through ocular pigments, blood, and serous fluids. METHODS Using search engines and library resources, clinically relevant ICGA studies were reviewed for their purpose, description, outcome, clinical importance, and compared and contrasted to IVFA. RESULTS Information is presented that represents the current modalities of ICGA. CONCLUSION ICGA still remains an adjunct therapy to traditional IVFA. However, as the technology improves--allowing easier recording and visualization of ICG angiograms--and as more studies are conducted on the best use of the information obtained from ICGA, this technique may emerge as an important diagnostic tool in the fight to prevent the severe vision loss associated with the various retinal disorders mentioned.

Persistent hyperplastic primary vitreous

Persistent hyperplastic primary vitreous (PHPV) is a common congenital developmental anomaly of the eye that results following failure of the embryological, primary vitreous and hyaloid vasculature to regress. It typically presents unilaterally without associated systemic findings. Although the etiology is assumed to be identical in each of its three variants, PHPV is still subclassified into three presentations. The purely anterior presentation of PHPV is also known as persistent tunica vasculosa lentis and persistent posterior fetal fibrovascular sheath of the lens. It occurs in eyes with pathology of the anterior segment. This form typically involves cataract, glaucoma and a retrolenticular membrane. The purely posterior presentation of PHPV is termed falciform retinal septum and ablatio falcicormis congentia. It occurs in eyes with abnormalities confined to the posterior segment such as retinal folds, vitreal stalk, vitreal membranes, macular abnormalities and optic disc abnormalities. A combination of anterior and posterior presentations is the most commonly seen clinical presentation. Case report. We present a case in which an 11-year old male was referred to our office for reevaluation of a large angle esotropia, strabismic and deprivational amblyopia and previously diagnosed PHPV, OD. Conclusion. Without treatment, PHPV can produce recurrent intraocular hemorrhage, secondary glaucoma and eventually require enucleation. Early surgical intervention is necessary to prevent progressive pathologic changes and to obtain the best visual results. Finally, while PHPV is a documented source of leukocoria, clinicians should be aware of differential diagnoses which involve the white pupil (congenital cataract, retinoblastoma, Norries disease, retinopathy of prematurity, retinal detachment and Coats disease).

Idiosyncratic ocular symptoms associated with the estradiol transdermal estrogen replacement patch system.

The Estradiol Transdermal Estrogen Replacement Patch System (Estraderm) is designed to provide continuous estrogen replacement therapy through a rate-limiting membrane applied to the skin. The relative advantage of the patch over the more commonly prescribed oral medication, Premarin, is that the drug is absorbed directly into the bloodstream without extended metabolism by the digestive system and the liver. Ocular side effects associated with Estraderm, reported in the literature, include fluctuation in corneal curvature and variations of keratitis sicca. Here, a case is presented in which a 50-year-old Caucasian female, without previous ocular sequelae status after hysterectomy and oophorectomy, experienced what appears to be atypical ocular symptoms associated with the Estraderm. To the best of our knowledge, this information has not been reported in the literature. Clinicians should be aware of the unusual symptoms that could accompany the use of the Estradiol Estrogen Replacement Patch and include them among the differential diagnoses. The condition, its associated symptoms, and summary of the literature are discussed.

Giant cell arteritis

Giant cell arteritis has been considered an enigmatic disease. It is characterised by chronic granulomatous inflammation of the walls of large and medium‐sized arteries. The process has a predilection for the extradural cranial arteries, which include the ophthalmic and the posterior ciliary arteries. It is a multi‐symptom disease of older individuals and patients often present with challenging issues and diagnostic dilemmas. We review the literature and latest protocols for the diagnosis and management of giant cell arteritis.

Leber's hereditary optic neuropathy: a case report

BACKGROUND Lebers hereditary optic neuropathy (LHON) is a bilateral optic neuropathy of mitochondrial inheritance that produces significant painless, central vision loss and dyschromatopsia. LHON usually occurs in young males between the ages of 15 and 30 years and manifests an episode of subacute or acute vision loss in one eye, with the opposite eye becoming involved weeks to months later. Approximately 80% to 90% of all LHON patients are male. While the disease usually presents itself around the third decade of life, its onset ranges anywhere from 5 to 80 years. CASE REPORT We report a case of an uncooperative 12-year-old Hispanic boy who was brought to our group practice following referral from an outside optometrist for amblyopia therapy. Following the workup by the binocular vision clinician, a neuro-ophthalmic consultation was obtained, eventually leading to the diagnosis and confirmation of LHON. CONCLUSION Lebers hereditary optic neuropathy may manifest signs and symptoms that mimic common ophthalmic entities. Teenage males often are reluctant to report its subtle clinical findings, making its discovery even more challenging. LHON should be kept in mind as a possibility for anyone who manifests unexplained visual loss.

Literature review of hepatitis B : should eye care practitioners receive a hepatitis B vaccine ?

The incidence of Hepatitis B infection has been steadily increasing over the last 5 years. The current literature has established that eye care practitioners are in the high risk category for contracting and transmitting this serious viral infection. Hepatitis B Virus (HBV) is present in all bodily fluids including tears and is considered to be more easily transmitted with a higher degree of infectiousness than Human Immunodeficiency Virus (HIV). Fortunately, unlike Acquired Immune Deficiency Syndrome (AIDS), hepatitis can be prevented with a vaccine. Recent improvements in the hepatitis vaccine have made it safer and more effective. As optometrists expand their scope of practice, it becomes essential to increase the knowledge base and awareness of this clinical entity so informed decisions can be made regarding vaccination by eye care practitioners, teaching faculty, and students.

Acute zonal occult outer retinopathy: a case report.

BACKGROUND Acute zonal occult outer retinopathy (AZOOR) is a disorder of unknown etiology with a predilection for young women. AZOOR is characterized by an acute loss of one or more zones of outer retinal function with a corresponding loss of visual field in one or both eyes. Patients present with photopsia, variable funduscopic changes, and abnormal electroretinogram (ERG) findings. There are no proven treatments. Diagnosed cases have a reasonable prognosis because central vision is often spared. CASE REPORT A 24-year-old white woman presented with acute photopsia with clouded temporal vision in the right eye (O.D.). Initial evaluation found a slightly swollen nerve fiber layer with no other outstanding lesions O.D. Over a 7-week follow-up period, there was significant progression into the deep chorioretinal tissues O.D. with an anterior chamber response. At 3 months, an afferent pupillary defect (APD) developed with significant retinal pigment epithelium (RPE) changes. After extensive testing, the consulting retinal specialist identified AZOOR as the etiology. At 21 and 31 months, the process remained in remission. Although primary symptoms subsided, the dense inferior-temporal field defect remained O.D. CONCLUSION AZOOR is a rare condition with subtle and often vague signs and symptoms making diagnosis difficult. The course of AZOOR, its clinical presentations, hypothesis of etiology, differential diagnosis, workup, management, and prognosis are reviewed.

Using systemic analgesics for managing ocular pain

Abstract Pain is often the primary reason for patients to present to health care providers. The role of the optometrist as a primary and post-operative care provider requires an understanding of the current concepts of pain and its therapeusis. This paper will review the pathways and pathophysiolocial theories of pain and the medications prescribed to manage ocular pain.