An atypical case of Foster Kennedy syndrome.

Foster-Kennedy syndrome was described in 1911 as an ophthalmologic manifestation of compression by a solid tumor in the frontal area with intracranial hypertension (ICHT). We describe a peculiar case of Foster-Kennedy syndrome associated with an arteriovenous malformation in which neither optic nerve compression nor ICHT was obvious. We discuss the different pathogenic mechanisms to explain this case, for which a chronic venous hypertension was the most probable etiology.

[Adenomatous hyperplasia of the conjunctiva]. / Hyperplasie adénomateuse de la conjonctive.

We report a case of adenomatous hyperplasia of the conjunctiva. A 42-year-old man was referred for a left conjunctival lesion involving the caruncle; the histopathological patterns revealed benign adenomatous hyperplasia, a disease that may originate in the conjunctiva. Adenomatous hyperplasia of the conjunctiva is a benign condition. The absence of cytonuclear anomalies and mitotic activity distinguishes adenomatous hyperplasia from adenocarcinoma and mucoepidermoid carcinoma.

Congenital dyserythropoietic anaemia, type I, in a Caucasian patient with retinal angioid streaks (homozygous Arg1042Trp mutation in codanin-1).

A congenital dyserythropoietic anaemia (CDA) was recognised in a French Caucasian male patient. Blood smears showed a pronounced aniso-poikilocytosis. Bone marrow light microscopy showed signs of dyserythropoesis, but no internuclear chromatin bridges. Electron microscopy disclosed erythroblast nuclei with the Swiss cheese aspect and the presence of cytoplasmic organelles, assessing the diagnosis of CDA I. The presence of internuclear chromatin bridges may thus be missing in CDA I. The patient proved to be homozygous for the Arg1042Trp mutation in codanin-1 (the 'Bedouin mutation'). By the age of 25, the patient's vision started to deteriorate as a result of retinal angioid streaks and macular abnormalities. Evolution was controlled and the patient, being nearly 50 yr old now, still has a partial use of his eyes. This second case of retinal angioid streaks reported in CDA I adds to the non-haematological features likely to be associated with this condition.

[Eyelid metastasis from a bladder urothelial carcinoma]. / Métastase conjonctivo-palpébrale d'un cancer de la vessie.

INTRODUCTION: Case report of an eyelid metastasis from a bladder urothelial carcinoma. OBSERVATION: A 71-year-old man presented with an eyelid metastasis. The patient had had a bladder carcinoma (pT1HG-2) resected 3 years before. Histologic patterns of bone and eyelid metastases were consistent with a high-grade urothelial carcinoma (pT1HG-2). In this case, eyelid metastasis was an incidental finding of end-stage disseminated metastatic spread of the tumor. CONCLUSION: Eyelid metastases of the bladder are infrequent and associated with disseminated metastatic spread of the tumor.

[Transitional carcinoma of the conjunctiva]. / Carcinome transitionnel de la conjonctive.

We report a case of transitional carcinoma originating from the conjunctiva. An 86-year-old woman was referred for a right conjunctival swelling lasting 6 months after a first surgical excision. The histopathological pattern revealed a transitional carcinoma, which usually originates from the airways and the lacrimal sac; recurrences are not uncommon. Transitional carcinoma may originate in the conjunctiva.

[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.

[Iris melanomas. A retrospective study of 11 patients treated by surgical excision]. / Les mélanomes de l'iris. Etude rétrospective de 11 cas traités par excision chirurgicale.

INTRODUCTION: Iris melanoma, even when a malignant tumor, has a slow progressive course. Surgical treatment is easy because of localization of the tumor but can be accompanied by a range of complications, from photophobia to cosmetic problems. MATERIALS AND METHODS: We reviewed the records of 11 patients with iris melanoma treated by surgical excision with posterior limbus incision. Conventional iridectomy was performed in nine cases and iridocyclectomy in two cases. RESULTS: There were seven females and four males ranging in age from 27 to 76 years. Histologically, 10 tumors were composed of B spindle cells and one was mixed. For all patients, followed up for 1-5 years, final visual acuity was more than 6/10. One patient complained of photophobia and cataract developed in one 76-year-old woman at the end of follow-up. CONCLUSION: Because of the good prognosis of iris melanoma, conservative treatment can be given in most of cases (without local complications). Surgical resection confirms diagnosis after histopathological examination, with good final functional result thanks to scleral tunnel incision, which induces less astigmatism than corneal incisions. In the future, these findings will have to be confirmed by a corneal topography study before and after surgery.

[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.

[Clinical course of ocular sarcoidosis in patients with histologically proven systemic sarcoidosis]. / Evolution clinique des sarcoïdoses oculaires au cours de la sarcoïdose systémique histologiquement prouvée.

PURPOSE: We reviewed the clinical features, natural history and visual prognosis of 9 patients with histologically confirmed ocular and systemic sarcoidosis. PATIENTS: Nine patients underwent a follow-up study between 1993 and 1998. The diagnosis of sarcoidosis was supported by histological evidence of non caseating epithelioid-cell granuloma in tissue biopsy. RESULTS: The mean age was 40.912 years. The mean follow-up was 22.7 months (range 6 - 54 months). The ophthalmic involvement was bilateral in 8 patients. Before treatment, 3 eyes (17.6%) had a visual acuity less than 1/10. Two eyes (11.7%) had a visual acuity less than 3/10 and 10 eyes (58.8%) more than 6/10. The anterior uveitis was granulomatous in 2 eyes (11.7%) and non granulomatous in 8 eyes (47%). Five eyes (29.4%) had a pars planitis. The posterior segment manifestations of sarcoidosis were retinal vasculitis in 7 eyes (41.1%), papillitis in 10 eyes (58.8%) and choroidal granulomas in 2 eyes (11.7%). Seven patients were treated with systemic corticosteroids. One patient was treated with steroid eye-drops. After treatment, one eye (5.8%) had a visual acuity less than 1/10 and 13 eyes more than 6/10. After treatment, the choroidal granulomas disappeared and the intra-ocular inflammation was controlled in all cases. CONCLUSION: Although the number of patients in our series is small, the prognosis for ocular sarcoidosis appears to be good.

Acyclovir-resistant bilateral keratitis associated with mutations in the HSV-1 thymidine kinase gene.

PURPOSE: To evaluate the contribution of molecular methods for the diagnosis of an acyclovir-resistant HSV-1 bilateral keratitis in an AIDS patient and to report a new point mutation in the nucleotide sequence of the thymidine kinase (tk) gene involved. METHODS: A 31 year old HIV-positive female presented with severe, active, bilateral and sight-threatening keratitis of 6 months duration, which was treated unsuccessfully with acyclovir. After corneal biopsy, samples were analysed by standard virological procedures, in situ hybridization, and PCR. The tk gene was cloned and subsequently sequenced. RESULTS: Conventional virological methods remained inconclusive. However, in situ hybridization and PCR rapidly confirmed the diagnosis of HSV-1 keratitis. The tk gene sequence revealed the presence of five variations previously described in two reference strains, but also a new point mutation at nucleotide position 431 which leads to an amino-acid change at position 144 that supported the hypothesis of a putatively altered functional form of the enzyme. Intravenous foscarnet treatment in an induction regimen was effective and cicatrization occurred within 3 weeks. CONCLUSIONS: PCR and in situ hybridization are effective and powerful techniques when other virological procedures are non-contributive, particularly in immunocompromised patients previously treated with antiviral drugs. The new point mutation identified in the tk gene may be associated with resistance to acyclovir.

[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.

[Lacrimal gland hypertrophy: revealing sarcoidosis]. / Hypertrophie des glandes lacrymales: mode de révélation d'une sarcoïdose.

We present a case of systemic sarcoidosis in a 34-year-old woman initially presenting with bilateral and symmetric proptosis caused by lacrimal gland enlargement. Based upon clinical, biological and radiological findings, sarcoidosis was suspected with lacrymal gland, parotid and pulmonary lesions. Biopsy of enlarged lacrimal gland for histological examination revealed a non caseating granuloma compatible with the diagnosis of sarcoidosis. Sarcoid lesions regressed with corticosteroid therapy.

[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.

Efficacy of proton therapy in circumscribed choroidal hemangiomas associated with serious retinal detachment.

OBJECTIVE: The purpose of the study is to evaluate the efficacy and safety of proton therapy in complicated circumscribed choroidal hemangiomas. DESIGN: The study design was a retrospective review. PARTICIPANTS: Studied were 13 patients (13 eyes) who had circumscribed choroidal hemangioma associated with serous retinal detachment. Of these, four eyes previously underwent laser unsuccessfully. INTERVENTION: Proton therapy including a total dose of 30 Cobalt-Gray-Equivalent was administered to each eye. MAIN OUTCOME MEASURES: Patients were controlled for initial and final best-corrected visual acuity, slit-lamp examination, intraocular pressure, fundus examination, fluorescein angiography, and tumor thickness on B-scan ultrasonography. RESULTS: The mean follow-up period was 26 months (range, 9-48 months). Retinal reattachment was obtained in all cases after a mean period of 52 days. The tumor height decreased in all cases. Visual acuity improved to two lines or more in eight eyes (62%) and reached 20/200 or more in nine eyes (69%). No radiation complication was detected during follow-up. CONCLUSIONS: Proton radiation seems to be effective and safe in the management of choroidal hemangioma associated with serous retinal detachment. It may be useful when photocoagulation can not be performed.