Diabetic papillopathy: two case reports in individuals with adult onset diabetes mellitus

BACKGROUND: Diabetic papillopathy is a benign unilateral or bilateral optic neuropathy with transient optic disk edema and minimal reduction in visual function. The optic disk edema typically resolves in a few months with no resulting optic atrophy and minimal or no decrease in acuity. The exact etiology of the disk edema is unknown, but theories include retinal vascular leakage into and surrounding the optic nerve and disruption of axoplasmic flow resulting from microvascular disease of the optic nerve head vasculature. CASE REPORTS: Two adult patients receiving insulin for type II diabetes mellitus manifested bilateral disk edema and minimal visual dysfunction. Both patients showed funduscopic evidence of mild-to-moderate nonproliferative diabetic retinopathy O.D. and O.S., and one patient had clinically significant macular edema in both eyes. The diagnosis in both cases was diabetic papillopathy. Both patients had significant resolution of their disk edema in 3 to 6 months, with stable acuities and no signs of optic atrophy. CONCLUSIONS: Although diabetic papillopathy is a well-known clinical entity in patients with type I diabetes, the clinical profile can be expanded to include individuals with type II diabetes.     

Bilateral disc edema in retinitis pigmentosa

Retinitis pigmentosa (RP), one of the most common forms of hereditary retinal degeneration, is characterized by night blindness and constricted visual fields. In addition to bone spicule pigmentation, other ocular findings may include posterior subcapsular cataracts, cystoid macular edema, and hyaline bodies or drusen of the optic nerve. Rarely, optic nerve head (ONH) edema has been reported to be associated with RP. A 44-year-old white male with RP and neurosensory hearing loss (Usher's syndrome type II) presented to our clinic for routine examination. A dilated fundus examination revealed bone spicule pigmentation, vessel attenuation, several flame hemorrhages on or adjacent to the nerves, and ONH edema in the right eye. B-scan ultrasonography revealed drusen of the right ONH but not of the left. Late stage fluorescein angiography showed hyperfluorescence and dye leakage from both optic discs which was more pronounced in the right eye than the left. Computed tomography (CT) of the head and orbits and cerebrospinal fluid (CSF) examination by lumbar puncture were normal. The differential diagnosis of bilateral ONH edema in this case included ONH drusen or papilledema secondary to increased intracranial pressure. This patient was found to have RP with asymmetric, bilateral ONH edema of unknown cause. One theory regarding the cause of the ONH edema is disc vessel leakage secondary to an inflammatory reaction caused by rapid photoreceptor and retinal pigment epithelium (RPE) degeneration.     

HTLV-I associated myelopathy with macular degeneration

The authors report a case of macular involvement in a patient with HTLV-I associated myelopathy (HAM). The patient was a 10-year-old girl who noticed sudden decreased vision in her right eye in November 1987. The corrected visual acuity was 0.01 in the right eye and 1.0 in the left eye. Fundus examination of the right eye disclosed mild optic disc pallor. The macula appeared to have pigmentary mottling with superficial irregular retinal reflex that was three disc diameters in size. Fluorescein angiography revealed a discoid hypofluorescent area in the macula, surrounded by mottled hyperfluorescent areas. She had no contributory family history of ocular disease, but had a history of blood transfusion during an operation for patent ductus arteriosus and ventricular septal defect at the age of 8 months. In November 1990, she developed gait disturbance due to spastic paraparesis and was admitted to our hospital. Antibodies to HTLV-I were markedly elevated in serum (titer, 1:8192) and in cerebrospinal fluid (titer, 1: 1024). She was diagnosed as HAM. Two months later, she developed encephalopathy and bilateral optic disc atrophy.     

Serial magnetic resonance imaging shows separate medial and lateral medullary infarctions resulting in the hemimedullary syndrome

PURPOSE: To report two cases in which cystoid macular edema developed after initiation of topical latanoprost for glaucoma. METHODS: Case reports. One pseudophakic eye in each of two patients treated with latanoprost for glaucoma developed decreased vision and cystoid macular edema. Latanoprost was discontinued, and the cystoid macular edema was treated with topical corticosteroids and ketorolac. RESULTS: After discontinuing latanoprost and starting corticosteroids and ketorolac, visual acuity improved from counting fingers to 20/60 in one patient's left eye and from 20/100 to 20/25 in the other patient's right eye. The macular edema resolved in both eyes. CONCLUSIONS: Topical latanoprost may be associated with cystoid macular edema; this may be related to a prostaglandin-like action.     

Rapid growth of an intracranial aneurysm causing apparent retrobulbar optic neuritis

We describe a 31-year-old man who developed sudden painful loss of vision in the right eye and was found to have a retrobulbar optic neuropathy. Magnetic resonance (MR) imaging gave normal results, and a diagnosis of retrobulbar optic neuritis was made. The patient was treated with oral prednisone, but he continued to lose vision in the right eye and then began to lose vision in the left eye. Repeat MR imaging performed eight weeks after the initial study showed a giant intracranial aneurysm compressing the right and left optic nerves. Cerebral angiography revealed that the aneurysm arose from the origin of the right ophthalmic artery. Treatment of the aneurysm by a trapping procedure resulted in improvement in vision in the left eye but no change in vision in the right eye. This report emphasizes the difficulty in imaging intracranial aneurysms of various sizes, the rapidity with which intracranial aneurysms can enlarge, and the importance of continued follow-up examinations in patients thought to have idiopathic optic neuritis.     

Profile of some risk factors for coronary heart disease in a developing country: Nigeria

We examined visual evoked potentials and pattern electroretinograms in a patient with Tolosa-Hunt syndrome associated with optic nerve involvement. The 82-year-old woman developed unilateral painful ophthalmoplegia and visual loss in the right eye. Magnetic resonance imaging showed an abnormal soft-tissue area in the right cavernous sinus and the right orbital apex. Symptoms responded rapidly to treatment with corticosteroid. Visual evoked potentials to flash and pattern stimuli were both remarkably reduced and delayed in the right eye in the acute stage; however they improved to almost normal after steroid therapy. The pattern electroretinogram recorded in the acute stage was normal bilaterally. These results indicate that optic nerve involvement in Tolosa-Hunt syndrome can be mild and reversible.     

Bacillary angiomatosis associated with myositis in a patient infected with human immunodeficiency virus

PURPOSE: To report a patient with optic nerve avulsion caused by forceful rotation of the globe that occurred when his thumb penetrated the orbit while he was diving. METHODS: A 17-year-old boy was initially examined for sudden loss of vision after jumping feet first from a bridge 50 feet above a river. Upon hitting the water, he felt his right thumb push into his right globe. The patient underwent ophthalmologic and imaging examination. RESULT: Examination disclosed a tear of the optic nerve head from the sclera temporally in the right eye. CONCLUSION: Optic nerve avulsion occurs secondary to forceful rotation of the eye with tearing of the optic nerve as it exits the globe.     

Glaucomatous cupping of the optic disk by ultrasonography

B-scan ultrasonography was used to evaluate advance glaucomatous cupping of the optic disk (0.7 cup/disk ratio or greater) in one normal subject and six patients with glaucoma. The normal eye emitted a continuous echo from the posterior pole, which conformed to its mild degree of concavity. The glaucomatous eyes emitted echos from the posterior pole, demonstrating the more extreme concavity of the optic cup. Ultrasonographic evaluation of an acrylic plate containing holes of known diameter demonstrated the limits of resolution and artifacts that resulted from this examination.     

Impaired increase of prostaglandin E2 in gastric juice during steroid therapy in children

BACKGROUND: Lightning can cause a number of ocular complications. A case involving a patient who developed a cataract and reversible maculopathy in both eyes after being struck by lightning is reported. METHODS: The patient was evaluated for cataract and macular edema by ophthalmoscopic examination, fluorescein angiography, and potential acuity meter. RESULTS: Maculopathy developed that was characterized initially by a retinal cyst with surrounding edema. Later, the lesions evolved to simulate a full-thickness hole. These lesions subsequently resolved, and the patient's visual acuity improved to 20/20 in each eye after cataract extraction. CONCLUSION: Because the visual prognosis for lightning-induced maculopathy is potentially different than that for full-thickness macular holes, careful retinal examination is essential in the preoperative workup.     

Photocoagulation of well-defined choroidal neovascularization in age-related macular degeneration: clinicopathologic correlation

PURPOSE: To present the clinicopathologic features of the eyes of a patient with age-related macular degeneration (ARMD): the right eye was treated for well-defined extrafoveal choroidal neovascularization (CNV), and the left eye had an untreated disciform scar. METHODS: The patient was studied ophthalmoscopically and by fluorescein angiography at the time of presentation and on follow-up examinations up to 54 days after laser treatment, when he died. The posterior portions of both eyes (obtained postmortem), including the macula and optic nerve head, were sectioned serially for light microscopy. Tissue sections from both eyes were removed from glass slides and studied by transmission electron microscopy. RESULTS: Histopathologic study of the right eye disclosed a thin layer of basal laminar deposit throughout the posterior pole. Two defects in Bruch's membrane without CNV were present within the area of laser photocoagulation located superior to the fovea. No CNV was present in the scar. Eleven areas of early CNV were present in the posterior pole. Histopathologic study of the left eye showed a prominent basal laminar deposit throughout the posterior pole. A 2.6 x 2.7 mm disciform scar was present that was located mostly in the subretinal space. Four sources of CNV were present. CONCLUSIONS: The clinicopathologic features of a treated eye with well-defined extrafoveal CNV, and the fellow eye with a disciform scar, both associated with ARMD, are presented. Although laser treatment obliterated a choroidal neovascular membrane, 11 additional areas of early, subclinical CNV were present.     

Orbital compression syndrome in sickle cell disease

BACKGROUND: Orbital complications are an uncommonly reported finding in sickle cell disease. METHODS: The authors review the reported orbital manifestations of sickle cell disease and discuss a patient with hemoglobin sickle beta(0) thalassemia in whom rapidly progressive bilateral orbital compression developed. RESULTS: Computed tomography of the orbits in a patient with fever, headache, orbital swelling, and optic nerve dysfunction displayed bilateral superior subperiosteal cystic masses. Surgical exploration showed bilateral liquefied hematomas, which were evacuated. Recovery was complete 13 days after surgery. A mild recurrence 14 months later resolved with conservative treatment. The literature contains 11 reports of 16 young patients with sickle cell disease (15 sickle cell disease [Hb SS] and 1 hemoglobin sickle cell disease [Hb SC]) with rapidly developing findings ranging from frontal headache, fever, and eyelid edema to bilateral complete orbital compression syndrome. Including our patient, 60% had orbital hemorrhage on computed tomography. Ten of 12 patients tested were found to have orbital bone marrow infarctions. Sixteen of 17 patients had complete recovery; 13 were treated conservatively and 4 surgically. Only 2 of 17 had recurrence. CONCLUSIONS: Orbital complications in sickle cell disease are unusual manifestations in which a vaso-occlusive process in the marrow space around the orbit results in frontal headache, fever, eyelid edema, and often orbital compression syndrome. Subperiosteal hematomas are common and appear to result from bone marrow infarctions. Appropriate management requires a thorough evaluation to exclude other hemorrhagic, infectious or neoplastic processes, as well as vigilant ophthalmic monitoring. Supportive care is effective, unless optic nerve dysfunction or large hematomas are present, which would indicate that surgical evacuation is warranted to prevent loss of vision and to speed recovery.     

Location of the fovea at the fundus in relation to the optic nerve head

BACKGROUND: Exact determination of the location of the fovea may be difficult, especially in patients suffering from macular pathology. The purpose of this study was to evaluate whether the distance between the optic nerve head and fovea in healthy eyes may be helpful in such patients. PATIENTS AND METHODS: We measured the distance between the center of the optic nerve head and the fovea in 165 healthy eyes in a horizontal and vertical direction. Results were correlated to the refractive status. RESULTS: There was a relatively high deviation between all eyes for the distance between the optic disc and the fovea (15.5 +/- 1.2 degrees [12.8 to 21.3 degrees]). There was a significant relationship between this distance and the refraction (r = 0.353, P < 0.001). Intraindividual deviation between the right and left eyes showed a markedly lower variance (0.67 +/- 0.49 degree [0.05 to 2.3 degrees]). CONCLUSION: The distance between the optic nerve head and the fovea does not allow for meaningful determination of the location of the fovea in eyes in which morphological changes have occurred. However, knowledge of this distance from the other eye of the same patient is helpful.     

Macular edema caused by contraction of the posterior hyaloid in diabetic retinopathy. Surgical treatment of a series of 22 cases

We have observed some patients with diabetic macular edema who did not respond to grid laser treatment and who improved with spontaneous posterior vitreous detachment or vitrectomy. These cases have a taut and glistening vitreo-macular interface. Three such cases are presented in detail. Pars plana vitrectomy with separation of the posterior hyaloid was performed in 22 cases. All of them had proliferative diabetic retinopathy, previously treated by panretinal photocoagulation. Fourteen cases had an ineffective macular grid laser treatment. Postoperative visual acuity was improved in 19 eyes and was unchanged in three eyes. The macular edema disappeared in 12 eyes and decreased in 10. Complications included a vitreous hemorrhage in 6 eyes, a paramacular tear in 1 eye, a reghmatogenous retinal detachment in 1 eye and cataract formation in 2 eyes. Vitreous surgery can improve the visual prognosis in cases of diabetic macular edema associated with a pathological vitreo-macular interface.     

Complex anterior chamber dysgenesis with bilateral corneal ectasia

BACKGROUND: Sclerocornea is a congenital, uni- or bilateral, non-progressive, non-inflammatory malformation characterized by histological changes of the cornea resembling scleral tissue. CASE REPORT: We report the case of a 2-month-old child with bilateral dysplasia of the anterior segment including: 1. extreme corneal ectasia; 2. vascularized, opacified microcornea; 3. presence of rudimental limbus; 4. anterior scleral dysplasia. The axial length measured by means of ultrasonography (A-scan) was about 24 mm in both eyes. A penetrating keratoplasty was first performed in the right eye and one week later in the left eye. Intraoperatively an advanced iris atrophy with extensive anterior synechiae, as well as a localized anterior subcapsular cataract were seen bilaterally. A pale optic disk with a maximally enlarged optic cup could be seen only in the left eye. CONCLUSION: Although the histologic examination was compatible with the diagnosis of sclerocornea, the presence of atypical clinical findings previously unreported does not allow a definite classification of the case reported herein.     

A case of nonarteritic anterior ischemic optic neuropathy with cilioretinal artery occlusion

We report a case of nonarteritic anterior ischemic optic neuropathy (AION) with cilioretinal artery occlusion. The patient was a 61-year-old man with sudden visual loss in his right eye. Funduscopy showed pale swelling of the entire optic disc with retinal ischemic edema of the upper half of the retina, and fluorescein angiography revealed faint filling of the dye in the optic disc in the retinal arterial phase, and dye staining of the optic disc in the late phase. We initially diagnosed the disease as AION with branch retinal artery occlusion, but systemic administration of a corticosteroid and urokinase were ineffective and the optic disc became atrophic. As the optic disc swelling decreased and the course of arteries in the optic disc became clear, we repeated fluorescein angiography which proved that the involved upper retinal artery was a cilioretinal artery having earlier dye appearance than the lower retinal artery. Thus, we finally diagnosed the disease as AION with cilioretinal artery occlusion. We believe that Hayreh's view that AION may result from occlusion of the posterior ciliary artery is supported by the intraocular findings in this case.     

Bilateral idiopathic macular pucker: a case report

BACKGROUND: Idiopathic epiretinal membrane or macular pucker is a common disorder which typically affects older individuals. Contraction of epiretinal tissue causes changes to occur in the underlying retina stimulating the appearance of a macular hole. This paper presents a case of bilateral macular pucker with accompanying pseudohole. The diagnosis, pathophysiology and management are discussed. METHODS: A 68-year-old male was examined for decreased vision in both eyes. Relevant ophthalmoscopic findings included macular changes compatible with surface wrinkling maculopathy. RESULTS: The patient was diagnosed with bilateral epiretinal membranes with accompanying pseudoholes. The diagnosis was confirmed by fluorescein angiography. Since the patient's vision loss was minimal, the need for surgery was precluded. The patient was to be monitored routinely. CONCLUSIONS: Macular pucker, the most common of acquired macular disorders, has become an important clinical entity. Recognizing distinguishing features, especially in cases associated with pseudohole, will allow more precise diagnostic and prognostic patient counseling. Regular follow-up examinations of patients with mild visual loss become important, because they permit optimal time for surgical intervention.     

Retinal ganglion cell layer and visual function in a patient with optic disc drusen

BACKGROUND: To correlate the retinal ganglion cell pattern to visual acuity and visual field data in a patient with bilateral optic disc drusen, a quantitative clinicopathological study was carried out. METHODS: Both retinae of a patient with optic drusen were whole-mounted. Retinal ganglion cell counts were made using a sampling scheme covering the whole retina and compared to the findings in 10 normal retinae. Relative ganglion cell reduction in the drusen retinae was correlated to clinical data. RESULTS: The total retinal ganglion cell count was reduced from 1244858+/-98736 in normal retinae to 305319 on the right and 527571 on the left eye with optic disc drusen. Large ganglion cells had a better chance of survival. Parafoveal ganglion cell loss was 57% for the right and 36% for the left eye, while visual acuity was 0.8 and 1.0 respectively. The mean light sensitivity loss increased from the centre (6.2 dB) to paracentral (9.9 dB), mid-peripheral (13.7 dB) and outer peripheral (15.0 dB) retina, while ganglion cell losses were smallest in outer peripheral retina (21.9%), followed by central (53.0%), mid-peripheral (70.9%) and paracentral retina (87.7%). CONCLUSION: These data validate Frisén's theory on central retinal resolution and provide the structural basis for the clinical rule that low visual acuity should not be attributed to disc drusen. Automated light sense perimetry gives an inadequate picture of retinal damage caused by optic disc drusen.     

A histopathologic study of macular cysts and holes

Forty-four eyes from 39 patients with either lamellar macular holes (17 eyes), full-thickness macular holes (18 eyes), and/or macular cysts (9 eyes) were studied histopathologically. Lamellar and full-thickness holes were frequently found in eyes with a history of trauma or ocular surgery. Diabetes mellitus was the most common condition associated with macular cysts. Four lamellar holes and four full-thickness holes occurred in eyes with senile macular degeneration. Six lamellar holes, eight full-thickness holes, and one macular cyst developed on an idiopathic basis. Residual cystoid macular edema was the most prevalent accompanying pathologic feature. Cystoid macular edema was also noted in the opposite eye in seven cases of lamellar and full-thickness holes. A preretinal glial membrane, thought to be a secondary change, was found at or near the edges of the lamellar or full-thickness holes or over the macular cyst in the majority of cases. Wrinkling of the internal limiting membrane (ILM) was present in five cases. Vitreous traction, with or without an operculum, was infrequently associated with these entities.