[Lyme disease from an ophthalmological point of view]. / La maladie de Lyme du point de vue de l'ophtalmologue.

The first case of Lyme disease was described more than 25 years ago in the city of Lyme, Connecticut (USA) and although the responsible pathogenic organism is known (Borrelia Burgdorferi) as well as its vector (a tick from the Ixode genus), it is still underdiagnosed and often poorly known by practitioners. The symptomatology is classically divided into three phases depending on the time between the tick bite and the first symptoms. However, in a number of cases this clinical division of the disease is not easily applied. We describe three cases of atypical ocular manifestations to illustrate this problem. Recently developed laboratory tests help confirm the diagnosis but should not be used without a good knowledge of the disease. This study aims to emphasize the difficulty of diagnosis and the importance of appropriate treatment and to avoid potentially permanent complications.

Successful photodynamic therapy combined with laser photocoagulation in three eyes with classic subfoveal choroidal neovascularisation affecting two patients with multifocal choroiditis: case reports.

Multifocal choroiditis (MC) is an idiopathic choroidal inflammatory disease affecting young subjects. Secondary choroidal--and often central--neovascularisation is a frequent complication leading to a poor visual prognosis. Photodynamic therapy (PDT) has now proven to be successful to treat classic subfoveal choroidal neovascularisation in age-related macular degeneration and in pathologic myopia. We describe the treatment applied to classic choroidal neovascularisation in two young women with MC, two eyes with subfoveal neovascular membrane and one eye in which new vessels encroach the foveal avascular zone. PDT has been useful in the three reported eyes, with stable or improved visual acuity. In two of them, it even made the membrane retract and become extrafoveal, allowing a secondary treatment using conventional laser.

[Ocular Behcet's disease: procedural aspects]. / Behçet oculaire: aspects pratiques.

The diagnosis of ocular Behçet is clinical. This affection is characterised by a bilateral anterior and/or posterior recurrent non granulomatous intraocular inflammation. The treatment consists in the use of one or several associated immunomodulators and immunosuppressors. In order to decrease the drug toxicity we prefer to increase the number of associated drugs than to increase the doses in severe resistant cases. The prognosis can be significantly improved by a very strict control of chronic inflammation and of each exacerbation. Interferon alpha could be a good new treatment in the future.

White spots syndromes.

The different types of white spots occurring in the fundus are analysed. A. Acute white spots, vanishing later on. 1. Multiple Evanescent White Dot Syndrome. 2. Cat scratch disease. 3. AIDS microangiopathy. 4. Cotton-wool spots. 5. Acute vitelliform maculopathy. B. Acute white spots with coalescence and diffuse scarring. 1. Acute posterior multifocal placoid pigment epitheliopathy. 2. Serpiginous choroïditis (geographic choroïditis). 3. Herpes retinitis. C. Acute white spots becoming white scars with variable pigmentation. 1. Multifocal choroiditis--classical form. 1a. Punctate inner choroidopathy. 1b. Diffuse subretinal fibrosis. 2. Toxoplasmic retinochoroiditis. 3. Tuberculous chorioretinitis. 4. Syphilitic chorioretinitis. 5. Lyme disease. 6. Sarcoidosis. 7. Sympathetic ophthalmia. 8. Vogt-Koyanagi-Harada disease. 9. Bacterial retinochoroiditis. 10. Fungal retinochoroiditis--Candida. 11. Pneumocystis carinii choroiditis. D. Late white spots with or without initial white-orange spots. Birdshot chorioretinitis.

Multiple arterial ectasias in patients with sarcoidosis and uveitis.

PURPOSE: To describe and evaluate the cause of a clinical entity characterized by bilateral intraocular inflammation, multiple arterial ectasias including beading, macroaneurysms, comma-like ectasias and kinking, with vasculitis, staining of the optic disk and multiple peripheral round punched-out hypopigmented chorioretinal scars in elderly patients. The formation and the course of the arterial ectasias is analyzed. METHODS: Seven patients with this syndrome were evaluated by clinical examination, fluorescein angiography, and systemic investigations. RESULTS: Three of the seven patients had a biopsy characteristic of sarcoidosis, two others showed positive bronchoalveolar lavage, as well as other analyses and tests suggesting sarcoidosis, and two showed other findings suggestive of sarcoidosis. The patients were all over 60 years of age and had arterial hypertension. In two patients, an arterial ectasia developed at the site of previous focal inflammation. The macroaneurysms either remained unchanged, became comma-like ectasias, arterial kinks, or completely vanished. CONCLUSION: Elderly patients with multiple arterial ectasias, uveitis, disk staining, and peripheral chorioretinitis should be thoroughly investigated for sarcoidosis. We suggest that sarcoidosis may cause some degree of arteritis, which may weaken the arterial wall, with resulting ectasia. Arterial hypertension may play a role in the formation of the ectasias by increasing the pressure on the arterial wall weakened by inflammation.

Familial primary pulmonary hypertension and associated ocular findings.

BACKGROUND: Familial primary pulmonary hypertension (PPH) is a rare, fatal, autosomal dominant disease that results in right heart failure from idiopathic obliteration of the pulmonary arteries. Patients develop stagnation of venous blood flow and elevated venous pressure. METHODS: The authors retrospectively reviewed the clinical records of three patients diagnosed with PPH that was confirmed on the basis of physical examination, chest X-ray, electrocardiogram, and echocardiogram. Cardiac catheterization excluded cardiac shunt and other secondary causes of pulmonary hypertension. RESULTS: Two patients presented with a clinical picture resembling venous stasis retinopathy, and one with bilateral choroidal detachments. Two patients had delayed choroidal filling on fluorescein angiography, which was confirmed in one patient with indocyanine green videoangiography. CONCLUSIONS: Elevated venous pressure found in PPH is responsible for the delayed choroidal perfusion and the reduced venous blood outflow. This explains the clinical findings of venous stasis retinopathy and choroidal detachments seen in these patients.

Familial spastic paraplegia and maculopathy with juxtafoveolar retinal telangiectasis and subretinal neovascularization.

PURPOSE: To describe a previously unreported condition involving familial spastic paraplegia and a peculiar type of maculopathy. METHODS: Two pairs of siblings were studied. All four cases underwent serial clinical examinations, fundus photography, and fluorescein angiography. Two siblings had extensive investigations. RESULTS: Characteristics of the four cases include spastic paraplegia diagnosed in the first decade of life and visual loss at about age 9 due to a maculopathy with an abnormal vascular complex. In the early stages, parafoveal dilatation of the capillary network was noted. The later stages were characterized by cystic macular degeneration, and seven of eight eyes developed fibrovascular scars with retinochoroidal anastomoses, pigment migration, and atrophic changes. In two siblings, electro-oculographic findings were subnormal, whereas results of electroretinography, magnetic resonance imaging of the brain and spinal cord, and metabolic and karyotype studies were normal. These siblings were an Indonesian girl and boy; the other siblings were white males. There was no consanguinity of the parents and family history was unremarkable. CONCLUSIONS: This study suggests that the two pairs of siblings have an identical familial and probably recessive disorder with neurodegenerative changes that have caused paraplegia and a peculiar maculopathy associated with anomalous retinal vascular complexes, retinochoroidal anastomoses, and subretinal neovascularization.

[Evaluation and utilization of home visual aids prescribed to the satisfaction of the patients for age-related macular degeneration]. / Evaluation de l'utilisation à domicile des aides visuelles prescrites et de la satisfaction du patient, en cas de dégénérescence maculaire liée à l'âge.

We evaluated the subjective advantage given by optical visual aids to patients affected by Age Related Macular Degeneration (ARMD). A total of 51 patients with ARMD having magnifying visual aids were retrospectively analysed. The 51 patients have been splitted up in 2 groups according to the final macular aspect and 7 subgroups according to their treatment, and have been questioned about the subjective advantage of their optical aids. Out of the 51 patients, 71% were satisfied, 5% were moderately satisfied and 24% were unsatisfied. The group with the lowest visual acuity at 5 meters has a satisfaction percentage only slightly lower (71%) than that with a higher visual acuity (75%). From the patients with a visual acuity superior to 3/10, 58% were affected with a persistence of macular edema. Seventy six percent of the patients with edema and 65% of the patients with a dry macular scar use their low vision aid every day. In summary, an important percentage of the patients are satisfied with their visual aids, independently of the fact that the retina is exsudative or dry, or that the visual acuity is good or bad. It seems useful to propose these optical aids to any patient suffering from ARMD who complains about reading-difficulties.

Late side-effects of radiotherapy for subfoveal choroidal neovascularization.

Radiation therapy for subfoveal neovascularization has not yet proved its efficiency, but, to our knowledge, no exudative complication of this treatment has been reported. We describe a late side effect observed in 16 eyes after a mean follow up of 33 months, characterized by major extension and exudation of the choroidal new vessels. In several cases, development of elongated, club like new vessels was observed at the border of the neovascular membrane. At the end of the follow-up, 4 eyes had an exudative retinal detachment affecting half of the retina or more and visual acuity was < or = 1/50 in 94% of the cases (including a case with no light perception and 2 cases with only light perception).

Subfoveal choroidal neovascularization: radiotherapy or perifoveal photocoagulation? A fellow eye study.

Perifoveolar photocoagulation is advised for subfoveal choroidal neovascularization responding to the MPS eligibility criteria. Recently, radiation therapy has been proposed but has not yet proved its efficacy. We compared these two treatments. We retrospectively reviewed 27 patients with age related macular degeneration and subfoveal choroidal neovascularization in both eyes. The first eye was treated with perifoveolar photocoagulation and the second eye with radiotherapy. In case of simultaneous diagnosis, radiotherapy was performed in the eye with the best visual acuity. Although the follow up is two times longer and the initial visual acuity is lower in the group of the photocoagulated eyes, both groups have similar results at the end of their respective follow up. However, in the eyes treated with radiotherapy the lesions are still active and edematous while in the eyes treated with perifoveolar photocoagulation, the lesions are stable and dry. The percentage of patients who subjectively prefer the photocoagulated eye has increased progressively from 0% to 71.5% along the 20 months of follow-up post radiotherapy. The perifoveolar photocoagulation of the subfoveal choroidal neovascularization seems, at median term, to give better objective and subjective results than the radiation therapy.

[Laser treatment of detachments of the retinal pigment epithelium associated with sub-retinal neovascularization in the context of age-related macular degeneration: retrospective study]. / Traitement au laser des décollements de l'épithélium pigmentaire rétinien associes à des néovaisseaux sous-rétiniens dans le cadre de la degenerescence maculaire liée à l'âgè: étude rétrospective.

Pigment Epithelial Detachment (PED) associated with subretinal new vessels (SRNV) is a particular aspect of Age Related Macular Degeneration (ARMD). We retrospectively analysed the results of dye laser photocoagulation in 63 eyes of 56 patients with vascularised PED. We photocoagulated in a confluent manner the presumed zones of SRNV, detected by fluorescein angiography and three-mirrors-lens examination. In most cases, the SRNV were of the occult type. In 89% of the treated eyes we obtained a flattening of the PED and the visual acuity was stabilized or ameliorated in 66% of the cases after a mean follow up of 29 months. This final visual acuity was better or equal to 1/10 in 64% of the cases and superior or equal to 5/10 in 46% of the cases. Subfoveal SRNV, initial visual acuity of less than 1/10, and persistence or recurrence of the PED after treatment were of bad prognosis. However, recurrence of the SRNV was not necessarily of bad prognosis if it could be retreated. Treatment of interpapillomacular SRNV had the best prognosis. Laser photocoagulation can be beneficial in well selected patients with vascularised PED.

[Radiotherapy treatment of subfoveal retinal neovascularization: results after two years]. / Traitement par radiothérapie des néovaisseaux sous-rétiniens rétrofovéolaires: résultats à 2 ans.

The treatment by radiotherapy (10 Grays) of subfoveal choroidal neovascular membranes is disappointing. Although after one year, 22% of cases had a positive result, after a mean follow up of 2.5 years only 14% of cases had a favourable outcome. The final visual acuity 30 months after treatment was the same as the one observed after 21 months of natural evolution. The well-defined nature of the subretinal new vessels, the presence of a pigment epithelial detachment and the occurrence of a vast hemorrhage were of bad prognosis.

[Intravenous corticosteroid megadose treatment in ocular Behçet disease]. / Traitement par mégadoses intraveineuses de corticoides dans l'atteinte oculaire de la maladie de Behçet.

Ocular Behçet's disease is characterized by a very poor visual prognosis. Twelve patients which had a maintenance treatment with immunosuppressors received 30 intravenous i.v. bolus of corticoids for uncontrolled severe retinal vasculitis (macular edema 58% of cases) and papillitis (100% of cases) (1 bolus treatment = 0.5-2 g of i.v. methylprednisolone once a day, for 3 days). In addition, oral immunosuppression was moderately increased in order to prevent further recurrences. Tolerance was good in all cases. Visual acuity (VA) remained stable or improved after one month in 87% of cases following the bolus treatment. In 92%, the VA remained stable or improved for a mean follow-up of 6 years. These good results suggest that intravenous bolus of steroids is a safe and efficient therapy which may prevent further decrease of the visual acuity and may reduce the side effects of the immunosuppressive treatment needed to control severe posterior uveitis in Behçet's disease.

[Diabetic retinopathy]. / La rétinopathie diabétique.

Diabetic retinopathy remains the most frequent cause of blindness in the active population of industrialized countries. However, decreased vision and blindness can efficiently be reduced by good metabolic control, yearly retinal examination since onset of diabetes from puberty onwards, and timely laser photocoagulation. Increased frequency of retinal controls, in case of retinal or general risk factors, enables to detect and to treat threatening lesions early, leading to reduction of decreased vision and blindness.

Hyperlipofuscinosis and subretinal fibrosis in Stargardt's disease.

BACKGROUND: The fundus aspect of Stargardt's disease is characterized by progressive atrophic macular changes often associated with fundus flavimaculatus flecks. Other fundus changes have been described such as pallor of the optic disc, attenuated retinal vessels, cicatricial chorioretinitis, retinal pigment hyperplasia, subretinal neovascularization, or subretinal fibrosis. Three cases of Stargardt's disease and presumed subretinal hyperlipofuscinosis or subretinal fibrosis are presented. METHODS: The clinical history, and the ophthalmoscopic and fluoroangiographic characteristics of these three patients are discussed, as well as the clinical course in the two first patients. RESULTS: Two young patients with the ophthalmoscopic and fluoroangiographic aspects of Stargardt's disease in one eye showed the presence of dense orange subretinal material in the posterior pole of the fellow eye. During the follow-up period, the abnormal material regressed partially in one patient and completely in the other, leaving subretinal fibrosis and localized retinal pigment epithelial hyperplasia. The left fundus in a third patient was characterized by an area of subretinal fibrosis in the midperiphery. CONCLUSION: It is presumed that the abnormal material in the two first patients is lipofuscin and that this represents an exaggerated response of the diseased retinal pigment epithelium, possibly to a minor trauma. That material will eventually be replaced by subretinal fibrosis. The fundus appearance in the third patient is possibly the result of a similar process.