Tricia L. Newman

Occlusive retinal vasculitis in a patient with West Nile virus.

Background:u2002 West Nile virus (WNV) was first identified in the United States in 1999. In addition to a spectrum of systemic manifestations, several ocular conditions secondary to the virus have been reported, including chorioretinitis, uveitis and optic neuritis. Age and diabetes mellitus (DM) have been reported to be associated risk factors for the more severe forms of the systemic disease. Only seven cases of occlusive retinal vasculitis have been reported in patients with WNV infection.

Neurosensory detachment arising from a fractured inner-limiting membrane secondary to chronically elevated intraocular pressure.

Diffuse optic nerve excavation and focal rim loss mimicking an optic pit have never been reported to predispose patients to serous detachments despite their relative frequency among patients with glaucoma. Recent reports of idiopathic macular schisis detachments occurring in the setting of elevated intraocular pressure without evidence of a contributing comorbidity have caused some to speculate that alternative mechanisms exist with the capacity to engender these retinal complications. Experimental simian research has unveiled the capacity of chronically elevated intraocular pressure to yield conduction portals between the posterior hyaloid face and the subretinal space by inducing microscopic fractures in the inner-limiting membrane. To our knowledge, this is the first case report providing objective evidence of an idiopathic neurosensory detachment resulting from a fractured inner-limiting membrane arising in the setting of chronically elevated intraocular pressures. Case Report. A 34-year old black man presented with transient eye pain and fluctuating vision in his left eye with his current spectacle prescription. A 2-year history of right eye blindness from glaucoma was uncovered. Funduscopic evaluation revealed a broad neurosensory detachment in the setting of an excavated optic nerve in the patient’s right eye. Optical coherence tomography confirmed the hydrodynamic separation of the sensory retina from the retinal pigmented epithelium and permitted visualization of a fractured inner-limiting membrane with a contiguous communication between the posterior hyaloid face and the subretinal space at the nasal limit of the detachment. Fluorescein angiography studies identified the absence of chorioretinal vascular compromise contributing to the minimal expansion of the dye into serous cavity late into the study. No optic pit was discernible using optical coherence tomography imaging or fluorescein angiography. Conclusions. Although glaucomatous damage of the optic nerve has rarely been shown to predispose an individual to serous complications within the macula, recent reports attest to its pathogenic capacity and propose a theory to explain their infrequent clinical coexistence. This case provides additional support for the mounting evidence to support the role of a compromised inner-limiting membrane in inducing a macular detachment in the setting of chronically elevated intraocular pressure without evidence of preexisting optic pits.

Bilateral abducens palsies and facial weakness as initial manifestations of a Chiari 1 malformation.

Purpose. Chiari I malformations are rare, congenital anomalies involving the caudal herniation of the cerebellar tonsils into the upper cervical spinal canal. Osseous abnormalities of the skull permit the extension of the hindbrain below the foramen magnum and engender syringohydromyelia formation. Neuroophthalmic manifestations are not uncommon; nevertheless, this is the first report of concomitant bilateral abducens palsies and facial nerve impairment as presenting manifestations of a Chiari I malformation. Case Report. A 30-year-old black female presented with a recent history of blurred vision and binocular diplopia in both left and right gazes. Headaches, dizziness, and orofacial sensorimotor impairments were noted in conjunction with the onset of her diplopia. No history of surgery, trauma, or systemic illness was elicited. Extraocular motility testing confirmed a complete bilateral abduction deficit. Additional cranial nerve testing revealed bilateral facial nerve weakness and tactile hypesthesia in the perioral region. No taste disturbances were reported. Nuclear magnetic resonance imaging studies revealed a Chiari I malformation with a syringohydromyelia formation in the vicinity of the patient’s sixth cervical vertebrae. Foramen magnum decompression surgery was performed. Two months after surgery, abduction capacity was restored along with resolution of symptoms of perioral numbness and signs of facial weakness. Conclusion. Bilateral sixth nerve palsies are rare clinical commodities especially when they do not occur in isolation. Intracranial neoplams, trauma, subarachnoid hemorrhaging, demyelinating disease, and meningeal infarcts have all been shown to induce mixed cranial neuropathies. The concomitant bilateral abducens palsy and concomitant facial nerve weakness reported in this case represents an atypical and previously unreported presentation of a Chiari I malformation.

Quantification of long anterior lens zonules and their resulting zonule-free zone sizes.

1. Alvi AP, Cantor LB. Aqueous misdirection syndrome. Ophthalmology 2013; 10: 1092–3. 2. Epstein DL. The malignant glaucoma syndromes. Glaucoma 1997; 4: 285–7. 3. Ramulu PY, Gedde SJ. Aqueous misdirection. Glaucoma 2014; 84: 836–46. 4. Fanous S, Brouillette G. Ciliary block glaucoma: malignant glaucoma in the absence of a history of surgery and of miotic therapy. Can J Ophthalmol 1983; 18: 302–3. 5. Schwartz AL, Anderson DR. Malignant glaucoma in an eye with no antecedent operation or miotics. Arch Ophthalmol 1975; 93: 379–81. Quantification of long anterior lens zonules and their resulting zonule-free zone sizes

Necrotising herpetic retinopathies: a review and progressive outer retinal necrosis case report

Necrotising retinopathies can be visually devastating. Most often associated with the viral family Herpesviridae and seen in both immune‐competent and immunocompromised hosts, possible complications of necrotising retinopathies include progressive retinal necrosis with or without macular involvement, optic neuropathy and ultimately, secondary retinal detachment. Examples include progressive outer retinal necrosis, acute retinal necrosis and cytomegaloviral retinitis. If diagnosed early and treated aggressively, visual complications can be prevented; however, there is no current consensus on the most appropriate antiviral regimen for each of the different varieties of necrotising herpetic retinopathy. This paper reviews aspects of varieties of necrotising herpetic retinopathy, including pathophysiology, treatment and diagnostic testing.

Remnants of the anterior tunica vasculosa lentis and long anterior lens zonules.

Purpose:To investigate presence of remnants of the tunica vasculosa lentis, a possible indication of anterior segment dysgenesis, in subjects with the long anterior zonule (LAZ) trait. Methods:Retroillumination photographs of the pupil region had been collected in earlier study of the LAZ trait in African Americans. Secondary image analysis was performed to assess the frequency of intact persistent pupillary membrane iris strands (PPMIS). Results:The analysis included 148 subjects, comprised of 74 LAZ subjects (median age=70 y; range, 50 to 91 y; 64 females) and 74 controls (68 y; 50 to 83 y; 64 females). While controlling for age and sex, analysis showed that LAZ subjects were 3.1 times more likely than controls (odds ratio=3.1; 95% confidence interval, 1.4-6.7; P=0.004) to exhibit PPMIS in at least one of their eyes. Conclusion:The LAZ trait, which is being studied as a potential risk factor for glaucoma, was associated with presence of PPMIS in our study population.

Cilioretinal artery occlusion with central retinal vein occlusion

BACKGROUNDnCombined cilioretinal artery and retinal vein occlusions are infrequently documented retinal vascular disorders of speculative origin. Occlusion of the cilioretinal artery is believed to result from either mechanical compression of the artery as a result of an increase in venous pressure or from a reduction in perfusion pressure in both the cilioretinal and retinal arteries. The ophthalmoscopic and angiographic features of this condition are reviewed.nnnCASE REPORTSnTwo cases of cilioretinal artery occlusion after central retinal vein occlusion are presented, one of which evolved to the development of iris neovascularization.nnnDISCUSSIONnThe incidence of cilioretinal artery occlusions due to central retinal vein occlusions is infrequently reported in the literature. Excluding those with chronic cystoid macular edema, most patients have a favorable visual outcome. It is possible that the incidence of combined cilioretinal artery and central retinal vein occlusions is grossly underestimated.

Cytomegaloviral retinitis from chronic immunosuppression following solid organ transplant surgery

Human cytomegaloviral (CMV) infections are a frequent cause of morbidity and mortality in the immunocompromised population. CMV is a double-stranded DNA virus belonging to the human herpes virus family and is transmitted systemically through infected blood products, bodily secretions or across the placenta. In developing countries, most infections are acquired during childhood. Adult onset CMV disease is usually due to a primary infection, activation of a latent infection, or reinfection. Of the general population, 40 to 100 per cent harbour antibodies against CMV. In the United States, between one and two per cent of the population acquires CMV infection annually. In immunocompromised individuals such as human immunodeficiency virus (HIV) infected patients, bone marrow or solid organ transplant recipients, or newborns, clinically significant disease can result. Though there is the presence of an antibody response in transplant patients, it may not be adequate enough to eradicate the virus and can result in transmission of disease from a donor positive organ (seropositive for CMV antibodies). We present a case of CMV retinitis that developed two years following a renal transplant due to chronic post-operative immunosuppression.

Morphologic Patterns Formed by the Anomalous Fibers Occurring Along the Anterior Capsule of the Crystalline Lens in People With the Long Anterior Zonule Trait: LONG ANTERIOR LENS ZONULES

People with the long anterior zonule (LAZ) trait, which may have prevalence near 2%, have zonular fibers that extend more central than usual along the anterior capsule of the crystalline lens. The anomalous fibers can be observed in vivo with clinical slit lamp biomicroscopy after pharmacologic pupil dilation, and although minimally studied, the LAZ trait may have importance to glaucoma, retinal degeneration, and cataract surgery. To further characterize LAZ morphology, a custom computer program was used to trace LAZ fibers seen on retro‐illumination photos acquired during previous study at an academic, urban eye care facility in Chicago, IL. There were 59 African‐Americans (54 female; median ageu2009=u200970 years, 53–91 years) included in the analysis. After initial review of the zonule tracings, we identified three basic LAZ patterns. We called one pattern (47% of right eyes) a “non‐segmental LAZ pattern,” which was predominated by fibers that could be visually traced to the dilated pupil border where they became obscured by the iris. Another pattern (35% of right eyes), the “segmental LAZ pattern,” was predominated by fibers that appeared to terminate abruptly without detectable extension to the pupil border. The third pattern (18% of right eyes), the “mixed LAZ pattern,” had a more equivalent mixture of the other two fiber morphologies. Compared to the “non‐segmental” group, the “segmental” LAZ eyes had smaller central zonule‐free zones (Pu2009<u20090.0001), and they tended to exhibit fewer LAZ fibers (Pu2009=u20090.07). These data improve understanding of LAZ clinical anatomy and may be helpful to future investigation. Anat Rec, 300:1336–1347, 2017.

Selective inner retinal layer involvement in early syphilitic retinitis as evidenced by spectral domain OCT

Retinitis as a feature of syphilitic uveitis in immunocompromised individuals is a common finding. We present spectral domain OCT images of early syphilitic retinitis pre and post treatment with penicillin. This case suggests that the inner retinal layers may be selectively involved with early syphilitic retinitis. Early treatment is important to avoid outer layer retinal involvement and to decrease ocular morbidity.