Pseudomembranous conjunctivitis following bone marrow transplantation: immunopathological and ultrastructural study of one case.

Pseudomembranous conjunctivitis (PMC) is a rare ophthalmic lesion usually caused by infectious agents such as streptococcus, Klebsiella, Chlamydia, or adenovirus. It can also be associated with skin lesions of toxic epidermal necrolysis (TEN) and graft-versus-host-disease (GVHD). We report the pathological study of a patient with PMC and GVHD who had a conjunctival biopsy before death. This allowed us to differentiate this form of PMC from PMC of infectious origin, and to find within the epithelium characteristic features of acute GVHD and mononuclear cells expressing natural-killer markers. This suggests that this form of PMC resulted from a very acute, cytotoxic form of GVHD. Because PMC in bone marrow recipients can be related to infections, TEN, or acute GVHD, a systematic pathological study is required to guide the therapy.

[Surgical treatment of congenital glaucoma. Long-term results]. / Traitement chirurgical du glaucome congénital. Résultats à long terme.

We studied 317 eyes, operated on for congenital glaucoma by Elliot, iridencleisis, goniotomy and trabeculotomy with a follow up of 5 to 35 years. Elliot and iridencleisis gave 30 to 50% intraocular pressure stabilisation and 60% bad visual results. Goniotomy gave without treatment 60% pressure stabilisation and 37% good visual results. Trabeculectomy gave 74% pressure stabilisation and 53% good visual results. Trabeculotomy gave the best results and can be considered as the most efficient operation in congenital glaucoma.

[Photocoagulation in sex-linked heredofamilial retinoschisis]. / La photocoagulation dans le rétinoschisis hérédofamilial lié au sexe.

In a series of 120 eyes of 60 patients in 24 affected families, 28 eyes were treated by photocoagulation in order to stabilize the extension of bullous peripheral retinoschisis to the posterior pole or to prevent retinal detachment. 3 eyes affected by severe retinal detachment in the months following this treatment. After analysis of spontaneous evolution of the untreated eyes and review of literature, the authors conclude that preventive photocoagulation in bullous retinoschisis is not advised, because of spontaneous regression with age and the possibility of complications. Prophylaxis treatment of retinal detachment by encircling the posterior pole by photo-coagulations is discussed.

[Syphilitic uveitis]. / Uvéites syphilitiques.

The incidence of syphilitic uveitis is reportedly increasing, up to 1% uveitis in general, with often polymorphic and atypical manifestations. Three cases of syphilitic uveitis are reported here, at different stages (secondary, latent, late). The bacteriologic sterilization is often unlikely in ocular syphilis, and recurrence is frequent; therefore a high-dose intravenous penicillin G treatment is then to recommend. HIV serology must be systematically associated with syphilitic tests.

[An atypical form of Recklinghausen's disease]. / A propos d'une forme fruste de maladie de Recklinghausen.

The authors report the case a young girl who presented ptosis of the left eye lid and in whom other ophthalmic findings (Lisch's nodules, larger wind sphenoid bone hypoplasia) led to the diagnosis of Von Recklinghausen's disease. They recall the importance of such manifestations for this diagnosis.

[Leber's optic neuropathy. Future prospects]. / Neuropathie optique de Leber. Perspectives d'avenir.

Leber's optic neuropathy is a maternally inherited disease. Its transmission does not correspond to Mendelian principles and two hypothesis about the role of cytoplasmic transmission are discussed. The role of a virus or a mutation mitochondrial DNA, maternally transmitted, are possible. However if not definite conclusion can be, actually, certified, there is a good hope to find a solution for this disease, in a near future.

[Results of peripheral retinoschisis treatment in sex-linked congenital retinoschisis]. / Résultats du traitement du rétinoschisis périphérique dans le rétinoschisis congénital lié au sexe.

Overall study of 20 years of curative and prophylactic peripheral retinoschisis treatment in 60 cases of sex linked congenital retinoschisis. Peripheral retinoschisis was present in 36 out of 120 eyes. Twelve eyes of children under 10 years severely affected, have undergone surgery (8 improvements and 4 aggravations) and 21 cases were photocoagulated (18 stabilizations, 3 aggravations which were subsequently treated by surgery). Six cases were not treated. Aggravations after surgery or photocoagulation have always been observed in early postoperative follow-up period. The above findings bring to light the problems of treatment. An epidemiological study of these 60 patients demonstrates that peripheral retinoschisis into bullous phase is only present under 5 years of age with tendency to regress with age (none reported after age of 50). This study corroborates ad absurdum the results of some authors who think that this type of retinoschisis has a tendency to improve spontaneously, and that indications for prophylactic and curative treatment must be thought of very carefully.

[Eye medulloepithelioma. Apropos of 2 cases]. / Les médullopithéliomes oculaires. A propos de deux cas.

The authors report two cases of medullo-epithelioma of ciliary body found in two young children. They describe the main clinical symptoms which are often non specific (cataract, glaucoma). Diagnosis is based on histological findings following enucleation: the tumor is developed from the ciliary body cells and has often a local malignancy. Metastasis are rare.

[Leber's hereditary optic neuropathy]. / La neuropathie optique héréditaire de Leber.

233 personal cases of leber's optic neuropathy have been analyzed by the authors in order to present clinical symptoms, evolution and genetic aspects. A group of 23 patients, in which 7 presented the disease and the others were asymptomatic members of families with the disease, was analyzed on evaluation of abnormalities of evoked visual responses; An other similar group, in repartition of subjects, was analyzed on frequency of cardiovascular abnormalities. The results have been analyzed and discussed about the role of predictive symptoms in diagnosis of the acute phase of the disease.

[Ophthalmologic manifestations revealing a forme fruste of Recklinghausen's disease]. / Manifestations ophtalmologiques révélatrices d'une forme fruste de maladie de Recklinghausen.

The authors report the case of a 14-year old European girl who presented with ptosis of the left eyelid and in whom other ophthalmic findings (Lisch's nodules, larger wing sphenoid bone hypoplasia at computed tomography) led to the diagnosis of von recklinghausen's disease. They recall and describe the main ophthalmic manifestations of this disease which may affect the eyelids (with a typical plexiform neuroma for example) the orbit (with bone dysplasia) and the eyeball (with very frequent Lisch's nodules). They discuss the frequency of such manifestations and their value in the diagnosis.

[Pheochromocytoma in Von Hippel Lindau disease. Apropos of a case of per-operative presentation]. / Le phéochromocytome dans la maladie de Von Hippel Lindau. A propos d'un cas de révélation per-opératoire.

A case of Von Hippel Lindau disease with bilateral pheochromocytoma is reported. The diagnosis of pheochromocytoma is evoked after complications during general anaesthesia for treatment of retinal hemangioblastoma.

[Hereditary pseudo-vitelliform macular degeneration. Apropos of 3 familial cases]. / Hérédo-dégénérescence maculaire pseudo-vitelliforme. A propos de 3 cas familiaux.

3 cases have been analysed, 2 sisters and one brother, 2 of which presented a macular lesion that could be related to best's disease. Certain clinical, EOG and angiographic characteristics rule out this diagnosis and statistic the diagnosis of pseudovitelliform degeneration.

[The course of fundus flavimaculatus in Stargardt disease]. / Mode évolutif du Fundus Flavimaculatus avec Stargardt.

From the study of twelve of Fundus Flavimaculatus and after recording the main characteristics of this disease the authors try to distinguish the "classical juvenile" form of Fundus Flavimaculatus form the "tardy" form of Fundus Flavimaculatus. This last one would affect the adult and have a better prognosis.

[Atypical familial hereditary degeneration of the macula]. / Les dégénérescences maculaires Hérédo-familiales atypiques.

Family case report presenting a central detachment initially associated to peripheral retinal disorders. Discussion on the uniqueness of this syndrome.

Analysis of results in the treatment of peripheral retinoschisis in sex-linked congenital retinoschisis.

Curative and prophylactic treatment of peripheral retinoschisis has been studied over a period of 20 years in 60 cases of sex-linked congenital retinoschisis. Peripheral retinoschisis occurred in 36 of 120 eyes. In all, 12 severely affected eyes in children under 10 years of age underwent surgery (8 improvements and 4 aggravations) and 21 cases were photocoagulated (18 stabilisations and 3 aggravations that were subsequently treated by surgery); 6 cases were not treated. Aggravations after surgery or photocoagulation were consistently observed in the early post-operative follow-up period. An epidemiological study of these 60 patients demonstrated that bullous-phase peripheral retinoschisis occurs only in patients under 10 years of age, with a tendency to regress with age (no cases were reported in patients over 50 years of age). This study closely corroborates the findings of authors who think that this type of retinoschisis tends to improve spontaneously and that indications for prophylactic or curative treatment should be very carefully considered.

[Apropos of 2 cases of oculo-orbital metastases of a prostatic epithelioma]. / A propos de deux cas de métastases oculo-orbitaires d'un épithélioma prostatique.

The authors report one case of choroid metastasis and one case of orbital metastasis from a prostatic carcinoma. A review of the literature confirms the exceptional nature of these metastatic sites (28 cases), which constitute a poor short-term prognostic factor. Palliative hormonal treatment allows regression of the ophthalmic signs without modifying the course of the disease.