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.     

Met 235 Thr polymorphism of angiotensinogen in Indonesians

PURPOSE: To report a previously undescribed cause of bilateral macular hole formation. METHOD: Case report. RESULTS: Septic emboli were noted at the center of the macula in both eyes of a 32-year-old man with acute bacterial endocarditis. Bilateral full-thickness macular holes later developed at the site of these retinal lesions. CONCLUSION: This case represents the first report of fundus lesions in septicemia resulting in full-thickness macular hole formation.     

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.     

Arterial vascular anomalies of the retina

The incidence of nonaneurysmal congenital anomalies of the retinal arteries was determined by ophthalmoscopic examination of the eyes of 2,100 consecutive healthy individuals whose ages ranged from 6 to 68 years. Unusual anomalies were triple branching, anomalous course, arteriolar-arterial crossing, unusual tortuosity, prepapillary loops, aberrant macular arteries, unusual supply of the optic disc, presumed total ciliary arterial supply of the retina, anomalous relationship with the central retinal vein at the optic disc, and pseudoaneurysm of a major retinal artery. Cilioretinal arteries are the commonest of the congenital vascular anomalies of the retina.     

Retropatellar contact stress in simulated patella infera

Because mutations in the peripherin/RDS gene have been found in retinal dystrophies involving the macula, we examined various types of macular dystrophies from southern France to characterize sequence variations that may be associated with these conditions. DNA sequence analysis of the full coding and flanking regions of the peripherin/RDS gene was performed in fifteen unrelated patients with different types of macular dystrophy, including nine with retinitis pigmentosa (RP). Of the 15 probands with macular disease, two (13.3%) were found to carry a mutation in the peripherin/RDS gene. The recurrent mutation P216S was identified in a pedigree with autosomal dominant RP. A previously unreported complex allele (1064delTC associated with IVS2 + 22ins7) that is predicted to result in the premature termination of peripherin/RDS synthesis was identified in a sporadic case of macular atrophy with RP. We also report eight novel neutral sequence variations in the peripherin/RDS gene, most of them found in the 3' untranslated part of the gene.     

Surface ultrastructure of human dermis and wounds

BACKGROUND: Intermediate uveitis is characterized by vitreal inflammation with associated inflammation of the vitreous base and peripheral anterior retina and choroid. It may be found as an isolated and idiopathic condition or in association with systemic disorders such as multiple sclerosis and sarcoidosis. OBJECTIVE: To identify the clinical features of intermediate uveitis and assess its association with systemic diseases. METHODS: Retrospective study of 83 patients presenting with intermediate uveitis between 1970 and 1991. RESULTS: Evidence of systemic disorders was found in 26 of 83 patients (31.3%). Of these 26 patients, 10 had presumed sarcoidosis, 6 had multiple sclerosis, 2 had isolated optic neuritis, 2 had inflammatory bowel disease, 4 had isolated thyroid abnormalities, and 2 had histories suggestive of Epstein-Barr virus infection. Associated ocular findings included cystoid macular edema, peripheral retinal perivascular sheathing, cataracts, posterior vitreous detachment, fine keratic precipitates, preretinal macular fibrosis, retinal tears, retinal detachment, and optic disc edema. CONCLUSIONS: Patients with intermediate uveitis may have associated systemic diseases and should have careful follow-up with regular systemic evaluation.     

The use of confocal scanning laser tomography in the evaluation of retinal elevation in age-related macular degeneration

OBJECTIVE: To evaluate the feasibility of using confocal scanning laser tomography in the analysis of macular topography in patients with subfoveal choroidal neovascularization associated with age-related macular degeneration (AMD) and to analyze quantitatively the changes in topography after local strontium-plaque radiation therapy. DESIGN: Prospective case series. PARTICIPANTS: A total of 16 eyes with subfoveal choroidal neovascular membranes (CNVM) treated with strontium-90 (90Sr)-plaque radiation therapy and 16 fellow eyes of 16 patients were examined. INTERVENTION: Confocal scanning laser analysis of macular surface topography before and after irradiation of the macula was performed. MAIN OUTCOME MEASURES: Parameters describing the height and volume of the retinal elevation in the macula were measured. RESULTS: The maximum height of the macular lesion at baseline was 0.25 mm (standard deviation [SD], 0.12 mm) in eyes showing regression of the CNVM during follow-up and 0.34 mm (SD, 0.19 mm) in eyes showing continued growth of the CNVM. During follow-up, a mean decrease in the maximum height of the macular lesion ranging from 0.03 to 0.10 mm occurred in eyes with regression of the CNVM, whereas the mean maximum height increased by 0.07 to 0.15 mm during follow-up visits in eyes with continued growth of the CNVM. All parameters describing the mean height and volume of the lesion also decreased significantly in patients showing angiographic regression, whereas they increased or remained unchanged in patients with continuous growth of the CNVM despite irradiation. The corresponding parameters also were higher in fellow eyes with untreated CNVM than in eyes without exudative AMD. CONCLUSIONS: Confocal scanning laser tomography can be used to monitor the amount of the change in neurosensory detachment in AMD. The parameters obtained by confocal scanning laser tomography correlate with CNVM perfusion after 90Sr-plaque radiation therapy. This technology is a useful tool for objective evaluation of morphologic change after institution of new therapeutic methods for the treatment of AMD.     

The effect of immunotoxins directed against CD80 and CD86 on primary T-cell alloresponses

A 13-year-old boy presented with acute loss of vision in his right eye of 2 weeks' duration. He had a high fever and was ill for several days, then improved but suffered recurrent episodes of sweating and a high fever. Ophthalmoscopy of the right eye showed optic disk edema, mild vitreous cells, and minimal exudates in the macula. Bartonella henselae titers were positive. A diagnosis of optic disk edema with a macular star secondary to cat-scratch disease was made. The patient was treated with doxycycline and made a dramatic improvement to visual acuity of 20/30 with a minimal residual relative central scotoma. The optic disk edema and macular star resolved, and the patient was left with mild optic atrophy in the right eye.     

Diabetic retinopathy

Nonproliferative diabetic retinopathy may cause visual loss when associated with macular edema or macular ischemia (secondary to retinal capillary nonperfusion). Proliferative diabetic retinopathy may cause severe visual loss if complicated by vitreous hemorrhage or traction detachment of the macula. Patients with diabetes benefit from collaboration between the internist and ophthalmologist. Tighter control of blood glucose levels and lower blood pressure reduce the risk of progression of diabetic retinopathy. Regular dilated eye examinations and appropriate intervention with laser or vitrectomy surgery help to preserve vision in patients with established macular edema or proliferative diabetic retinopathy.     

Vitrectomy for traction macular detachment in diabetic retinopathy

BACKGROUND: A small number of eyes with proliferative diabetic retinopathy develop massive central fibrovascular membranes characterized by vitreoretinal tractions along the arcades and optic disk and retinal traction lines extending through the macula. The aim of our study was first to present the results of vitrectomy for removal of these central membranes and second to determine the correlation between preoperative parameters and postoperative visual outcome. SUBJECTS AND METHODS: We treated 28 eyes with severe central fibrovascular diabetic membranes by a modified bi-manual en bloc excision technique during vitrectomy. Preoperative examination included general status, visual acuity, slit-lamp investigation, binocular funduscopy, ultrasound investigation and visual evoked potentials (VEP). Further, we analyzed intraoperative complications and postoperative anatomic and functional outcomes. RESULTS: The retinas of 27 eyes with central traction retinal detachments were reattached by surgery. With a minimum of 6 months' follow-up, the macula remained attached in 24 eyes, while the retinas were completely attached in 22 eyes. Preoperative visual acuity was defective light perception to 0.1; an increase in visual acuity to maximal 0.1 was seen in 50% of the patients postoperatively. Preoperative visual acuity of light perception was associated with no functional improvement. Preoperative ultrasound investigation gave information about the real anatomic situation of the retina, especially if funduscopy was not possible. The other preoperative parameters could not predict correctly the functional outcome of vitrectomy in diabetics with severe central fibrovascular membranes because of the damage of the optic nerve and the retina. CONCLUSIONS: The high rate of anatomical reattachment after vitrectomy in diabetic eyes with severe central fibrovascular membranes is associated with a slight improvement of function; only preoperative visual acuity of hand motions or better was associated with an improvement of function.     

Cytomegalovirus retinitis in a pediatric AIDS patient

INTRODUCTION: Cytomegalovirus retinitis (CMV) is the most frequently found opportunistic eye infection in adults with AIDS, with mean incidence of 20%-50%. However, only 5% of children with AIDS have this infection. CLINICAL CASE: We present the case of a six year old girl with stage C3 AIDS diagnosed at the age of 20 months, who developed unilateral diffuse retinitis due to CMV. The infection involved the posterior pole of the right eye, with retinal atrophy along the temporal vascular arcodes, and an active advance front in the temporal macula. The optic nerve was not found to be involved although the peripheral areas of the retina were involved leading to rhegmatogenous detachment of the superotemporal retina. In view of the systemic deterioration of the patient, no specific anti-CMV treatment was given. The patient died of respiratory insufficiency a few weeks later. CONCLUSIONS: CMV retinitis in paediatric AIDS patients is usually associated with more severe illness and a poorer general health than the adult population. In view of the absence of symptoms in these patients, periodic ophthalmoscopic examinations should be done in those who have severe immunological deterioration.     

Isolation and characterization of lectins and lectin-alliinase complexes from bulbs of garlic (Allium sativum) and ramsons (Allium ursinum)

PURPOSE: To evaluate the ophthalmic phenotype in families with three or more individuals who have age-related maculopathy. METHODS: Eight families were identified at academic centers in Massachusetts and North Carolina. Macular findings were graded based on a modification of the grading system used in the Age-Related Eye Disease Study (AREDS). RESULTS: All families had at least three members with stage 3 (extensive drusen change) or higher maculopathy in at least one eye, and six families had at least two members with advanced maculopathy (stage 4, geographic atrophy of the retinal pigment epithelium, or stage 5, exudative maculopathy). Both stages 4 and 5 maculopathy were observed among different individuals in four families. Individuals with stage 3 maculopathy were members of families with more advanced maculopathy (six families) and were of similar age as more severely affected family members but tended to be older than those with stage 2. CONCLUSION: The phenotypic appearance of the macula in families with multiple affected individuals is heterogeneous and representative of the spectrum of macular findings typically associated with age-related maculopathy.     

Pharmaceutical Benefits Advisory Committee: some questions answered

BACKGROUND: The butterfly-shaped dystrophy of the macula is an extremely rare autosomal dominant retinal disorder first described by Deutman in 1970. Two cases are presented. CASE REPORTS: The first patient presented to the clinic with slight deterioration of vision OD. Corrected visual acuity was OD 0.63 and OS 0.8. Examination of anterior segments was normal bilaterally. Funduscopically there was a mild disturbance of the foveolar retinal pigment epithelium. Fluorescein angiography, however, disclosed a large pattern-like hypofluorescence with hyperfluorescent areas in the macular region. Visual fields and electroretinography were normal. The second patient complained of decreased visual acuity OU. Funduscopic and angiographic findings were similar to the first patient. CONCLUSION: Different forms of pattern dystrophies of the macula have been described in the literature with moderate visual impairment. Differentiation is sometimes difficult as some forms have been reported to occur in one family. They may represent different clinical entities of the same disease. Diagnosis is usually made by fluorescein angiography.     

Loss of glucose transport in developing avian red cells

OBJECTIVE: This study aimed to examine the characteristics of intraretinal changes associated with macular holes and epiretinal membranes by scanning retinal thickness analysis. STUDY DESIGN: The study design was a nonconsecutive case series. PATIENTS: Fifty-six eyes of patients who had either a suspected or clinically diagnosed macular hole or epiretinal membrane were recruited. INTERVENTIONS: A commercial prototype of the scanning retinal thickness analyzer (RTA) was used. It projected a laser slit beam onto the retina and scanned it, in 200 or 400 msec, across a 2- x 2-mm area, yielding multiple optical cross sections that were recorded digitally. RESULTS: Epiretinal membranes were detected, and sites of attachment could be identified. Full-thickness holes corresponded to intraretinal cavities in which the inner retinal surface was broken, usually at the center. The majority of eyes with full-thickness macular holes showed increased retinal thickness surrounding the hole. The so-called "cuff of subretinal fluid," however, often was not present by retinal thickness analysis, despite clinical diagnosis to the contrary, even though retinal thickness analysis is capable of detecting such fluid. In 20 (42%) of 47 eyes diagnosed or suspected of having macular holes, scanning retinal thickness analysis showed findings different from those reported by retinal specialists. CONCLUSIONS: Examination of macular holes with the scanning RTA provides useful information in the diagnosis of macular holes in addition to that obtained through conventional techniques. The findings support the idea that many macular holes develop in association with intraretinal cystic changes. The precise chronology of the events remains to be determined.     

Intrachoroidal hemorrhage mistaken for malignant melanoma

An 84-year-old man had a choroidal mass in the temporal aspect of his left globe associated with a nonrhegmatogenous retinal detachment of the macula. Because of suspected malignant melanoma of the choroid the eye was enucleated. On histopathologic examination we found that an intrachoroidal hemorrhage, an extremely unusual type of intraocular hemorrhage, accounted for the mass.     

Longer-term visual outcome of eyes with retinopathy of prematurity treated with cryotherapy or diode laser

AIMS: Visual outcome of 66 eyes in 37 patients who had undergone treatment with either cryotherapy or diode laser for threshold retinopathy of prematurity was assessed. METHODS: 17 patients, representing 30 eyes treated with cryotherapy, were examined at between 56 and 98 months corrected age (median 68 months). 20 patients representing 36 eyes treated with diode laser, were examined at between 30 and 66 months corrected age (median 51 months). Structural outcome was categorised as: optimal--flat posterior pole; suboptimal--macular ectopia, optic nerve hypoplasia, retinal fold involving the macula, and retinal detachment involving the macula. RESULTS: Optimal structural outcome was, in the absence of amblyopia, associated with optimal visual acuity (of 6/12 or better) in all cases, with most eyes achieving a visual acuity of 6/9 or 6/6. Suboptimal structural outcome was invariably associated with suboptimal visual acuity. Amblyopia was present in eight out of 20 cryotherapy treated eyes and in five out of 26 laser treated eyes with an optimal structural outcome. Refractive errors were significantly less in laser treated eyes as was the incidence of anisometropic amblyopia. CONCLUSION: Eyes treated with either cryotherapy or diode laser for threshold retinopathy of prematurity with optimal structural outcome are associated with development of optimal visual acuity--that is, 6/12 or better. Treatment with either cryotherapy or laser does not in itself reduce the visual potential of these eyes.     

Molecular cloning and regional distribution of a human proton receptor subunit with biphasic functional properties

BACKGROUND: At present no satisfying treatment for subfoveal choroidal neovascularization (CNV) secondary to age-related macular degeneration (AMD) is available. Visual results after successful surgical removal of subfoveal CNV are disappointing. This has been explained by a primary dysfunction of the retinal pigment epithelium (RPE) in the macular region and the surgical trauma to the RPE in patients with AMD. Therefore, Machemer and Steinhorst developed a technique for macular translocation after surgical removal of subfoveal CNV. We report our first experiences with this technique in patients with subfoveal CNV secondary to AMD. METHODS: Seven patients aged between 71 and 83 years with subfoveal CNV were included in the study. Visual acuity of the fellow eyes was below 20/400. All patients underwent pars plana vitrectomy. Retinal detachment was produced by subretinal infusion of balanced salt solution and a 360 degrees retinotomy at the base of the vitreous was performed. After removal of the CNV, retinal rotation and reattachment, the retina bordering the retinotomy was coagulated with endolaser photocoagulation. Silicone oil was used as temporary tamponade. RESULTS: In all patients the subfoveal CNV was removed and the macula was translocated by a 15 degrees-45 degrees rotation onto functional RPE. The mean duration of follow-up was 11 +/- 3 months. Initial visual acuity ranged from 20/80 to hand movements. Final visual acuity was 20/100 to 20/400. Initially all patients complained of tilted vision. During follow-up the rotation of the image regressed and was well tolerated by all patients. Complications included the development of retinal detachment in three patients after silicone oil removal, development of a macula pucker, and a significant increase of lens opacity in the phakic eyes. CONCLUSION: In our series rapid improvement of visual function was observed in one patient only, even if the macula appeared ophthalmoscopically and angiographically normal. Vitreoretinal complications occurred frequently during follow-up.     

Vitrectomy-laser-gas for treating optic disk pits complicated by serous macular detachment

PURPOSE: Optic nerve pit is a rare congenital anomaly. In two third of the patients, the severity of the disease is increased by the apparition of a serous macular detachment, which may compromise the visual prognosis. The aim of this study is to propose a therapy appropriate to such complication. METHODS: A prospective study was performed including 10 patients with a serous macular detachment caused by optic nerve pit. All patients underwent intraocular surgery including vitrectomy, peripapillary photocoagulation and intravitreal injection of gas. The mean postoperative follow-up period was 9.5 months. RESULTS: Therapy success was based on anatomical and functional results. Serous macular detachment reattached in 7 patients out of 10 and a recurrence was observed in one case. Visual acuity improved from 0 to 18 lines of the EDTRS chart (mean increase: 6.7 lines). CONCLUSION: These results confirm that intraocular surgery including vitrectomy-photocoagulation-gas injection is a valuable treatment for serous macular detachment associated with optic nerve pit.     

Fluorescein angiography of retinal changes in patients with neurofibromatosis 2

BACKGROUND: Neurofibromatosis 2 (NF2) is a rare autosomal dominant disorder (prevalence 1:37.000) associated with changes in tumor suppressor genes. Intraocular manifestations have been reported as subcapsular cataract, retinal hamartomas, epiretinal membranes, keratopathy in facial palsy, and tumors of the nerves I-V are typical ocular manifestations of NF2. PATIENTS AND MATERIALS: We prospectively examined 7 patients who met the diagnostic criteria of NF2 with regard to retinal changes using fluorescence angiography and red-free fundus photography. RESULTS: In four patients, we found window defects of the pigment epithelium in the macular or paramacular areas. In one patient we observed a combined pigment epithelial and astrocytic hamartoma. A choked papilla was found in two patients. CONCLUSIONS: Presumably, retinal changes in NF2 occur more frequently than has been assumed previously. Our results confirm that in NF2 patients more attention should be paid to abnormalities of the optic disk, the retina and the choroid.