Iatrogenically induced Stevens-Johnson syndrome after a car accident.

BACKGROUND: Stevens-Johnson syndrome (SJS) is a hypersensitivity reaction that targets the skin and mucosal membranes. Ophthalmic manifestations may include conjunctival and corneal keratinization, shortening of the fornices, symblepharon, entropion, trichiasis, and adnexal cutaneous blisters. The syndrome is often misdiagnosed, and patients are treated incompletely with topical agents only leading to a worsening of the condition and graduated symptoms. CASE: A 61-year-old black man was admitted to the hospital for management and rehabilitation of multiple lower limb fractures after an automobile accident. The ophthalmic service was consulted regarding a persistent bilateral conjunctivitis that was worsening and recalcitrant to standard topical antimicrobial therapies. Best-corrected visual acuities were 20/70 in the right eye (O.D.) and the left eye (O.S.) secondary to grade III punctate corneal epitheliopathy resulting from evolving cicatricial ectropion. Anterior segment examination uncovered maderosis, blepharoconjunctivitis and dense corneal subepithelial infiltration from 3 to 5 o'clock. Externally there was bilateral juxtaorbital and transfacial skin blistering. After a review of history, the diagnosis of SJS was made, presumably secondary to recent oral antibiotic administration. Topical and oral steroids were initiated while concurrently seeking a dermatologic consult. The dermatologist agreed with our findings and management. The disease responded to the oral and topical regimen with significant reduction of signs and symptoms. An immediate physical improvement was evident within 10 days. CONCLUSIONS: Stevens-Johnson syndrome is a multifactorial hypersensitivity reaction requiring prompt systemic and local anti-inflammatory therapy.

Neurosensory retinal detachment secondary to torpedo maculopathy.

BACKGROUND: Torpedo maculopathy has been characterized as a congenital retinal pigment epithelial (RPE) nevus. CASE: A 38-year-old black woman presented with a chief complaint of intermittent floating spots in the right eye of 7 months' duration. Dilated fundoscopy found a "torpedo-shaped" lesion, with the tip of the lesion pointing toward the temporal macula. Visual field testing found a paracentral nasal defect, and optical coherence tomography (OCT) found a sensory retinal detachment. CONCLUSION: This lesion was unique because of the visibly detectable alterations seen in the photoreceptor layer of the retina and retinal pigment epithelium (RPE) in the setting of what was confirmed as a neurosensory retinal detachment. Torpedo maculopathy, although benign, may be visually devastating if its neurosensory etiopatholgy involves the macula. Even so, it rarely requires intervention.

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.

Ocular metastasis of cutaneous malignant melanoma.

BACKGROUND: Metastatic melanoma to the eye and orbit are rare. Patients at greatest risk often have disseminated metastases in the setting of advanced disease. Because the prognosis for orbital metastatic disease is poor, emphasis must be placed on early detection and prevention. Although cutaneous malignancies include basal cell carcinoma, squamous cell carcinoma, sebaceous cell carcinoma, and malignant melanoma, the majority of cases that result in metastasis, ocular morbidity, and mortality are from sebaceous cell carcinoma and malignant melanoma. CASE REPORT: A 72-year-old white woman presented emergently with a chief complaint of sudden, painless vision loss in her left eye of 2 weeks' duration. Her best-corrected visual acuities measured 20/30 in the right eye and 20/400 in the left eye with no improvement on refraction. Her systemic medical history was significant for the diagnosis of a cutaneous malignant melanoma. Examination of the left eye found grade II disc edema with all other posterior pole and peripheral structures intact and unremarkable. Magnetic resonance imaging confirmed the diagnosis of metastatic lesions involving structures of the left orbit ultimately causing reduced visual ability. The patient opted to avoid aggressive care and "hope for the best." Denying significant medical intervention, the woman lived in hospice where she died 6 months later. CONCLUSION: The primary care optometric setting, by the way a patient's visual symptoms present or the appearance of fundus abnormalities, can offer the capability of first detection and referral for the discovery of metastatic lesions. Although orbital metastasis is considered a terminal finding in these cases, timely diagnosis enables, while limited, the best options for management.

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.

Bilateral familial Duane's retraction syndrome.

BACKGROUND: Duane's retraction syndrome (DRS) is a congenital oculomotor anomaly that is accompany by globe retraction with simultaneous narrowing of the palpebral fissure on attempted adduction. Hereditary factors have been postulated to be associated with the development of DRS since 1879. However, the modern literature fails to provide substantial scientific evidence to support this premise. CASE REPORT: A 7-year-old patient presented with bilateral abduction deficit and retraction of the globe with narrowing of the palpebral fissure on attempted adduction. CONCLUSION: We present a case of Duane's retraction syndrome in which an investigation of the family history uncovered 4 generations of affected family members, lending support to the theory that some cases of DRS are inherited.

Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute, demyelinating polyneuropathy involving the spinal roots, peripheral nerves, and often the cranial nerves. Although its exact mechanism remains unclear, an autoimmune etiopathology is theorized. It is characterized by rapidly progressing, symmetrical muscular weakness starting in the legs and ascending to the trunk and arms. Additionally, deep tendon reflexes are lost. Approximately half of the patients with GBS have cranial nerve palsies, with unilateral or bilateral facial nerve (CN VII) palsy being the most common. Paralysis of the muscles of the tongue, lips, palate, larynx, and pharynx from lesions involving cranial nerves IX, X, and XI and weakness of the muscles of mastication (CN V) are the next most common cranial nerve abnormalities. Ocular muscle palsy is not common, only occurring in approximately 10% of patients. The Miller Fisher variant of GBS is a distinct syndrome in which the only neurologic deficits are oculomotor palsies, areflexia, and ataxia. We present the rare case of a 45-year-old woman with GBS whose ocular muscles were affected. The oculomotor disturbance and limb weakness occurred within a few days of one another. Basic anatomic considerations are reviewed along with the pathophysiology and clinical features of GBS.

Acute onset of halos and glare: bilateral corneal epithelial edema with cystic eruptions--atypical presentation of amiodarone keratopathy.

BACKGROUND: Amiodarone (Cordarone; Wyeth, Ayerst, New York) is a potassium channel blocking antiarrythmal medication indicated for recurrent ventricular fibrillation and recurrent hemodynamically unstable ventricular tachycardia. Chemically, it is classified as an iodinated benzofuran derivate antiarrythmal drug not chemically related to any other available antiarrhythmic drug. Documented side effects of amiodarone include neurologic, gastrointestinal, dermatologic, cardiovascular, and ophthalmic. The ophthalmic abnormalities include optic neuropathy, optic neuritis, papilledema, corneal deposits, photosensitivity, lens opacities, and macular degeneration. Corneal microdeposits are seen in virtually all patients who receive amiodarone for more than 6 months. Corneal microdeposits result secondary to the secretion of amiodarone by the lacrimal gland with accumulation on, and absorption by, the corneal epithelium. Approximately 10% of these patients become symptomatic with glare and halos; however, that alone is usually not enough to precipitate intervention. CASE REPORT: A 69-year-old woman presented to our office with a 2-week history of halos and glare in both eyes (OU). Her ocular history was significant for occasional contact lens wear. Her systemic history was significant for atrial fibrillation, for which she was taking amiodarone daily for the last 6 years. Six weeks before the onset of her chief complaint, her daily amiodarone dosage was increased from 100 mg to 300 mg. Ophthalmic examination found decreased visual acuities OU, amiodarone keratopathy (subepithelial verticillata), and diffuse corneal epithelial edema with diffuse sodium fluorescein staining OU. The corneal compromise was treated in 2 ways: the source medication was discontinued, and the ocular signs were medicated with a prophylactic topical antibiotic along with supportive preparations (tears/lubricants) and monitored over 2 weeks until full elimination of the pathologic signs and their symptoms. Even after complete resolution of the acute keratitis, the infiltrative keratopathy persisted along with the initial complaints of halos and glare. The supportive treatments were maintained over the course of 2 months until full recovery. CONCLUSION: By exclusion, it was determined that the subepithelial depositions and cystic formations were secondary to an acute amiodarone dosage increase by a new practitioner. Prompt communication resulted in the physician discontinuing that medicine.

Leber's hereditary optic neuropathy: a case report.

BACKGROUND: Leber's 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: Leber's 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.

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.

The National Eye Institute of The National Institutes of Health: its history and impact on clinical eye care.

BACKGROUND: Research supported by the National Eye Institute (NEI) of the National Institutes of Health (NIH) has contributed to the visual well being of the world. Pertinent citations from the literature were compiled to produce a clinical database reviewing major studies of the NEI and their outcomes. METHODS: Using Internet search engines and library resources, clinical NEI studies were reviewed. Their purpose, description, outcome and clinical importance are summarized. RESULTS: The results of major clinical NEI research are presented and included are the current modalities of treatment for each disease studied. CONCLUSION: Each NEI study yielded results upon which current scientific guidelines for the treatment or prevention of ocular disease are based.

Idiopathic intracranial hypertension: pseudotumor cerebri.

BACKGROUND: Idiopathic intracranial hypertension (IIH), as defined by the modified Dandy criteria, is a diagnosis of exclusion. It is important to rule out other sight- and life-threatening causes of optic disk edema. IIH primarily affects obese women of childbearing age. CONCLUSIONS: Permanent visual loss is the primary morbidity associated with IIH. Quantitative perimetry is key in management decision-making to prevent blindness. Treatment consists of weight loss, medication, and surgery.

Herpes zoster ophthalmicus.

BACKGROUND: We examined the literature for the latest information on diagnosis and management of herpes zoster, and compiled a representative database. METHODS: Using search engines and library resources, we reviewed pathology, epidemiology, pathophysiology, differential diagnosis, and management. RESULTS: The varicella zoster virus is a member of the herpes virus family that produces an infection through direct contact with active skin lesions or airborne droplets. The infection resides latent in the trigeminal ganglion until reactivated, often affecting the sensory nerve, skin, eye, and adnexa. CONCLUSION: The varicella zoster virus has the potential to severely disrupt the structures of the eye. Patients less than 50 years of age should be referred for systemic workup to rule out an immunocompromised state. In general, management is often palliative and/or geared toward specific sequelae.

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.