[Recovery of sickle cell disease ischemic maculopathy after erythropheresis: a clinical case study]. / Maculopathie ischémique drépanocytaire résolutive après échange érythrocytaire.

The authors report a case of a young patient with a recent decrease in unilateral vision. He had homozygote sickle cell disease with multiple general complications. Fundus examination was normal apart from a mild alteration of the macular reflect in the left eye, but fluorescein angiography showed multiple arteriolar macular occlusions, explaining the decrease in vision in the left eye. After erythropheresis, vision acuity improved and fluorescein angiography showed reperfusion. This case suggests that transfusional exchange may improve acute macular ischemia secondary to sickle cell disease.

[Circumstances of choroid and ciliary body melanoma detection]. / Circonstances de découvertes des mélanomes de la choroïde et du corps ciliaire.

PURPOSE: To report and analyze the circumstances of uveal melanoma detection. METHODS: The records of 143 consecutive patients diagnosed in the Ophthalmology Department of Gustave Roussy Institute between September 1994 and September 2001 were analyzed. The study included 66 females and 77 males, aged from 21 to 91 years (mean, 62.75 years). RESULTS: The first symptom was decreased visual acuity in 37% of cases. In 34.9%, there was no functional sign and a systematic fundus exam provided the diagnosis. Of the 143 patients, 18.8% presented alteration of the visual field or scotoma, 9.9% complained of phosphenes, 9% complained of metamorphopsia, and 6.5% complained of floaters. In 5.5% of cases, there was documented tumor growth. In 2%, the presence of extrascleral exteriorization was the first sign. At the time of diagnosis, anterior tumors tended to be significantly larger than posterior tumors (p<0.007). Smaller lesions were significantly associated with a systematic detection of the tumor (p<0.005). Liver metastasis occurred more frequently with ciliary body melanomas (p<0.001), which were more frequently the largest lesions. CONCLUSION: These results emphasize the importance of early detection of uveal melanoma. We recommended frequent fundus examination after pupil dilatation.

[Characteristics of uveitis presenting de novo in the elderly]. / Caractéristiques des uvéites de novo chez les patients de plus de 60 ans.

INTRODUCTION: This study aimed to describe the clinical characteristics of uveitis presenting de novo in the elderly. The study design was a description of a retrospectively identified case series. PATIENTS: The records of 193 patients with uveitis referred to Bicêtre Hospital's department of ophthalmology between January 1995 and January 2000 were reviewed. Among these patients, the records of 57 patients with uveitis de novo beginning after age 60 were analyzed. RESULTS: Idiopathic uveitis accounted for the majority of cases. Whereas herpes viruses were the most frequent specific diagnosis, presumed sarcoidosis and birdshot choroidopathy were also identified as diagnostic entities of uveitis presenting for the first time in the elderly. Only three cases of masquerade syndrome were identified, two cases of intraocular lymphoma, and one metastasis of a visceral melanoma. CONCLUSION: Masquerade syndromes are not the leading cause of uveitis in the elderly. Idiopathic uveitis and herpes viruses are the most common etiology found.

[Therapeutic strategy in delayed postoperative endophtalmitis: a report on 15 cases]. / Stratégie thérapeutique dans les endophtalmies chroniques survenues après chirurgie de la cataracte.

PURPOSE: To report the treatment strategies and visual acuity outcomes of chronic postoperative endophthalmitis. MATERIAL: and methods: The authors reviewed the records of 15 patients presenting 3 or more weeks after cataract surgery with intraocular inflammation and treated at Bicêtre Hospital from 1992 to 1998. Group I included 6 consecutive patients treated with vitrectomy and intravitreal antibiotic injection (vancomycin and cefazolin). Group II included 9 consecutive patients treated with intravitreal antibiotic injection (vancomycin and ceftazidime) and irrigation of the capsular bag (vancomycin). The minimum follow-up period was 1 year. RESULTS: In group I, 2 patients had recurrent inflammation. In these patients, the capsular bag and the intraocular implant were removed. In 1 patient there was culture-proven Corynebacterium and in 1 patient a Staphylococcus epidermidis was found. Final visual acuity was 20/40 or better in 5 patients and 20/100 in 1 patient. Visual acuity improved in all cases. In group II no recurrence was seen in the 12-20 months of follow-up. In 2 patients there was proven Staphylococcus epidermidis and in one patient Propionibacterium acnes was found. Final visual acuity was 20/40 or more in 3 patients, 20/100 or more in 4 patients and less than 20/200 in 2 patients. Visual acuity improved in 8 cases. CONCLUSIONS: Intravitreal antibiotic injection with vitrectomy and intravitreal antibiotic injection with antibiotic irrigation of the capsular bag are both effective in the treatment of delayed chronic postoperative endophthalmitis; however, with the second approach, there is minimal surgical trauma and the intraocular implant is retained.

[Sturge-Weber syndrome: medical management of choroidal hemangiomas]. / Le syndrome de Sturge-Weber: prise en charge thérapeutique des hémangiomes choroïdiens.

PURPOSE: To investigate the outcome of irradiation of complicated choroidal hemangiomas in Sturge-Weber syndrome. PATIENTS AND METHODS: The charts of 6 patients (7 eyes) with Sturge-Weber syndrome and choroidal hemangiomas were reviewed. An exudative retinal detachment was the indication for treatment in all cases. The mean age of the 6 patients was 13 years (range, 4 to 20 years). The minimum follow-up time was 1 year. Patients were checked for initial and final best-corrected visual acuity, fundus examination, fluorescein angiography, and tumor thickness on B-scan ultrasonography. The patients were treated with radiotherapy. A total dose of 20 Grays was applied to 7 eyes: 2 with a circumscribed choroidal hemangioma underwent proton therapy and 5 with diffuse hemangioma were treated by external beam irradiation. RESULTS: Complete resolution of the subretinal fluid was achieved in all cases with the tumor height decreased. Visual acuity improved to 1 line or more in 5 eyes and remained stable in 2 eyes. Two cases that underwent proton therapy developed radiation retinopathy. CONCLUSION: External beam radiation is an effective and safe option in the management of choroidal hemangiomas complicated by retinal detachment. Based on our experience, proton therapy should be reserved for sporadic circumscribed choroidal hemangioma.

[Bilateral diffuse uveal melanocytic proliferation associated with systemic carcinoma: two case reports]. / Prolifération bilatérale mélanocytaire uvéale diffuse associée à un carcinome systémique: à propos de deux cas.

Diffuse uveal melanocytic proliferation is a rare paraneoplastic syndrome resulting in rapid bilateral visual loss in patients with systemic carcinoma, caused by proliferation of benign melanocytes within the choroid and the ciliary body. More often visual impairment is due to retinal detachment and cataract. The authors report two cases of presumed diffuse uveal melanocytic proliferation. The first patient was a 74-year-old man with a history of colic carcinoma and hemangioendothelioma of the liver who presented with bilateral multiple nevi of the choroid and extrascleral melanic nodule. The second patient was a 59-year-old woman who presented bilateral multiple nevi of the choroid and a history of carcinoma of the thyroid treated by thyroidectomy 2 years before. There was no evidence of systemic melanoma in either patient. Our two patients showed slow progression with no visual impairment and a longer survival than those described in the literature.

[Total anterior opacification on the anterior surface of hydrophilic acrylic implanted lens]. / Opacification antérieure totale en avant d'un implant acrylique hydrophile.

We report a case of cloudiness occurring on the anterior surface of a hydrophilic acrylic foldable intraocular lens 1 year following implantation. This was not a case of phimosis of the capsulorhexis; the dondiness seemed secondary to fibroepithelial proliferation. Successful reopening was obtained with YAG laser. No recurrence was observed during the six months of follow-up.

[Familial amyloid polyneuropathy type I complicated by chronic glaucoma: 3 cases]. / Amyloses familiales avec polyneuropathie de type I compliquées de glaucome chronique.A propos de trois cas.

PURPOSE: We report 3 cases of familial amyloid polyneuropathy type I (FAP) with amyloid infiltration of the vitreum and glaucoma. PATIENTS AND METHODS: We reviewed the records of three patients, 2 females and one male, aged 41, 47 and 83 years respectively. The 3 patients had familial amyloid polyneuropathy type I with vitreous infiltration and open angle glaucoma. The two women underwent a liver graft four years earlier. Vitrectomy allowed confirmation of the diagnosis in the 83-year-old patient. Two patients underwent trabeculectomy, histological analysis of the iris and the trabeculum was obtained for one patient. RESULTS: The tree patients presented a polyneuropathy, vitreous infiltration and open angle glaucoma. Results of light microscopy of the iris and the trabeculum showed amyloid deposits in the iris and the trabecular meshworks. CONCLUSION: Familial amyloid polyneuropathy is a hereditary disease which may have a wide range of ocular manifestations. Glaucoma is among the most serious complications of familial amyloid polyneuropathy.

[Current status of retinal detachment in AIDS patients]. / Aspect actuel des décollements de la rétine chez les patients atteints de SIDA.

Cytomegalovirus retinitis is the most common opportunistic ocular infection in patients with AIDS affecting 30 to 40% of the patients. It usually occurs in patients in the terminal stage of the disease presenting with low CD4+ count (<50/mm(3)). Retinal detachment (RD) is a frequent complication of this disease, with an incidence varying from 18% to 29%. Risk factors for development of rhegmatogenous RD in patients with CMV retinitis were peripheral involvement greater than 25%, the presence of active retinitis, greater patient age and lower CD4+ cell counts. Multiple or single holes, as well as micro holes, were observed in areas of retinal necrosis leading to complex retinal detachments. Strong vitreoretinal adherences in these young patients, associated with chronic inflammation, were important elements in the pathophysiology of retinal detachment in AIDS patients. For localized RD, demarcating laser photocoagulation may delayed or avoided vitreoretinal surgery. For RD with macula off, good anatomical results have been obtained by repairing CMV retinitis-related retinal detachments using primary vitrectomy and instillation of silicone oil. Despite good anatomical results, poor long term functional results are related to optic atrophy. Since the introduction of highly active antiretroviral therapy (HAART), retinal detachment incidence has nevertheless dramatically decreased. Under HAART, CMV retinitis remains quiescent for long periods of time with a reduction of retinal detachment incidence of approximately 77%. For some patients on HAART, retinal reattachment can be obtained using vitrectomy, posterior hyaloid removal, and intraocular tamponade with SF-6 gas.

[Hemangioma of the choroid with extra-scleral extension]. / Hémangiome de la choroïde avec extension extra-sclérale.

We report a case of atypical circumscribed choroidal hemangioma with retinal detachment in a 41-year-old man referred with the diagnosis of Harada disease. B. Scan ultrasonography showed an hyperechogenic area extended through the sclera. Careful examination of the fundus showed a small peripapillary orange mass and an inferior retinal detachment. Surgical exploration revealed an extrascleral hemangioma. Proton beam irradiation was followed by retinal reattachment within three months. No recurrence has been observed 18 months after treatment.

[Rupture of the hemato-ocular barrier, studied by fluorophotometry, in uveal melanoma patients. Preliminary results]. / Etude de la rupture de la barrière hémato-oculaire par fluorophotométrie du vitré chez des patients porteurs d'un mélanome de l'uvée. Résultats préliminaires.

PURPOSE: To evaluate the use of intravenous vitreous fluorophotometry in assessment of the blood-aqueous barrier in eyes with uveal melanoma. METHOD: Vitreous fluorophotometry was performed before treatment in 14 patients with uveal melanoma. Both eyes of patients were examined, and fifteen control healthy patients were examined between November 1996 and December 1996 at the department of ophthalmology of Bicêtre hospital. RESULTS: Tumors with height > 6 mm and serous retinal detachments were accompanied by marked alterations of the blood-aqueous barrier, vitreous fluorophotometry showed diffusion of dye in posterior, mid and anterior vitreous: 2.99 ng/ml in the posterior vitreous and 5.20 ng/ml in the anterior vitreous. The posterior vitreous fluorescence at 60 minutes in the control eyes was 1.43 ng/ml and 1.30 ng/ml in the anterior vitreous. Diffusion of dye was present in the posterior vitreous in patients with tumor height less than 6 mm: 2.38 ng/ml (1.43 ng/ml in control eyes) at 60 minutes. CONCLUSIONS: Fluorophotometry provides a method for the assessment of the blood-aqueous ocular barrier in eyes with choroidal melanoma.

[Toxoplasmic chorioretinitis complicated by retinal detachment]. / Choriorétinites toxoplasmiques compliquées de décollement de rétine.

PURPOSE: To describe and analyse relationship between chorioretinal toxoplasmosis and retinal detachment. PATIENTS AND METHODS: Seven immunocompetent patients examined and treated between November 1992 and March 1996, with ocular toxoplasmic retinochoroiditis and retinal detachment. RESULTS: Of the 7 patients examined, 5 had active retinochoroiditis and 2 had typical inactive scars. Of the patients with active focus 3 had giant retinal tears, one had a posterior retinal tear and one had a retinal tear located at the edge of an atrophic scar. Of the patients with inactive lesions, one had tractional retinal detachment and the other presented with a complete retinal detachment, multiples tears and PVR. Five patients were treated by corticosteroid without antitoxoplasmic drug before they were referred. The seven patients underwent endo-ocular surgery with silicon oil or long actic gas tamponade. Three patients developed PVR and redetachment of the retina and two patients underwent further surgery. Good anatomical result was obtained in 6 patients. CONCLUSION: Retinal detachment associated with toxoplasmic retinochoroiditis is rare. However it represents a serious complication. Steroid administrated to salvage vision may then worsen the clinical course, these may be justified to reduce hypersensitivity to toxoplasma antigen, but they should be combined with an antimicrobial agent.

[Uveitis with hypopyon in patients with acquired immunodeficiency syndrome, treated with Rifabutin]. / Uvéite à hypopion chez des patients présentant un syndrome d'immunodéficience acquise et traités par Rifabutine.

BACKGROUND: Iridocyclitis has been identified as a dosage-dependent side effect in patients with the acquired immunodeficiency syndrome who are treated for Mycobacterium avium complex infections with systemic Rifabutin. We reviewed cases of acute hypopyon uveitis occurring in patients with AIDS to establish whether there was an association or not. METHODS: All patients were referred by an infection disease specialty service for complete ophthamological evaluation and ancillary laboratory. Six patients with AIDS, aged from 29 to 65 years, presented with acute unilateral hypopion. RESULTS: At the time of presentation, all six patients were receiving treatment for MAC infection with Rifabutin (dosage range, 300-600 mg/d) and four received Fluconazol. Results of microbiological investigations were negative. Hypopyon developed in the second eye of two patients. Hypopion resolved rapidly with intensive antibiotherapy without corticosteroids. CONCLUSION: Concomitant use of Rifabutin and Fluconazol may precipitate hypopyon uveitis. The cause of the uveitis is less certain, but the possible role of a microbiological agent cannot be ruled out.

Ocular toxoplasmosis in human immunodeficiency virus-infected patients.

The files of 45 human immunodeficiency virus-infected patients with ocular toxoplasmosis were reviewed, with a median follow-up of eight months. The condition was unilateral in 37 of the 45 patients (82%) and was bilateral in eight (18%). Inflammation of the anterior chamber and the vitreous was present in 32 of 53 eyes (60%) and 38 of 53 eyes (72%), respectively. Cytomegalovirus retinitis developed during the follow-up period in nine patients (20%). Cerebral toxoplasmosis was concurrently diagnosed with the ocular toxoplasmosis in 13 patients (29%). The efficacy of the combination of pyrimethamine and sulfadiazine or clindamycin was assessed in 42 patients for the induction therapy and in 38 patients for the maintenance therapy. Induction therapy was always effective within a median period of six weeks. During maintenance treatment, the 24-month relapse rates were 0.20 and 0.18 for the 50-mg/day and 25-mg/day dosage of pyrimethamine, respectively. The overall 12-month survival rate was 0.72. Our results suggested that ocular toxoplasmosis has a better ocular prognosis than cytomegalovirus retinitis, but that it requires appropriate treatment because life-threatening cerebral involvement is often associated.

Efficacy and tolerance of intravitreal ganciclovir in cytomegalovirus retinitis in acquired immune deficiency syndrome.

Forty-four patients with acquired immune deficiency syndrome with cytomegalovirus (CMV) retinitis (64 eyes) intolerant of or refusing systemic antiviral therapy received 710 intravitreal injections of ganciclovir at the dosage of 400 micrograms per injection. The patients were followed for a mean period of 9 weeks. Induction therapy consisted of two injections a week until healing. Maintenance therapy consisted of one injection a week until relapse. All but 1 of 53 induction courses led to cicatrization, after a mean of 6.6 injections. In 54 maintenance courses, the 8-week relapse rate was 53%. During intravitreal therapy, involvement of the fellow eye occurred in 11% of the patients and CMV infection developed in a nonocular site in 16% of the patients. Five retinal detachments and two intravitreal hemorrhages occurred. No endophthalmitis or cataract was noted. Intravitreal ganciclovir appears to be a safe and effective alternative in patients intolerant of intravenous anti-CMV drugs.

[Syphilitic uveitis and human immunodeficiency virus infection]. / Uvéite syphilitique et infection par le virus de i'immunodéficience humaine.

Ocular syphilis is rare in human immunodeficiency virus infected individuals. We think that syphilis should be considered in evaluating such patients presenting with uveitis. Most often, ocular syphilis includes retinitis associated with anterior or posterior uveitis, sometimes with optic neuritis. Concurrent neurosyphilis is frequent and may be more aggressive; it may progress more rapidly and cause more atypical signs than in patients without human immunodeficiency virus infection. This suggests the need for lumbar puncture in the evaluation of coinfected patients. The standard serological tests for syphilis (in blood and cerebrospinal fluid) may be nonreactive in human immunodeficiency virus seropositive patients. It may be because of the alteration of immunologic response of such patients. All coinfected patients with human immunodeficiency virus and syphilis should be treated with high-dose intravenous penicillin G sodium as recommended for neurosyphilis. We describe two human immunodeficiency virus infected patients with ocular syphilis and neurosyphilis.