Remote diagnosis in ophthalmology.

In cooperation with the C.H.U.-Lille (Eye Department) we set up an interregional and european project. We explain the possibilities and difficulties of remote diagnosis. The aim is to build up a network between different ophthalmological centers and so to give access to different centers of expertise to have a faster and more reliable diagnosis for the benefit of the patient.

Comparison between different retinoblastoma classifications.

We reanalysed the current staging systems for retinoblastoma, the classification of retinoblastoma for prognosis by Reese and Ellsworth and the new Essen prognosis classification for conservative sight-saving therapy by univariant analysis. We compared the value of those classifications with one another.

Risk factors for metastases in patients with retinoblastoma.

The study is based upon a review of data from 583 consecutive patients with retinoblastoma over the years 1956 to 1986. Mean follow-up was 8 years, and median was 5.5 years. In 41 patients, metastases developed within 5 years. The influence of clinical and histopathologic risk factors on the occurrence of metastases was first analyzed by univariate tests. Significant variables were then reevaluated using the Cox proportional hazards method. Four factors were found to be independently associated with the development of metastases: optic nerve invasion with and without involvement of the resection line, choroidal invasion and enucleation of an affected eye more than 120 days after initial diagnosis. The 5-year metastatic risks associated with these factors were 67%, 13%, 8%, and 4%, respectively. The relative risk estimate, calculated from the Cox model, was used for a score classification with groups of low, medium, and high metastatic risk. The 5-year incidence of metastases was 4%, 43%, and 68%, respectively.

[A numerical overview of various forms of retinoblastoma]. / Ein numerischer Uberblick über die verschiedenen Formen des Retinoblastoms.

The retinoblastoma is the most frequent intraocular tumour in early childhood occurring in a hereditary as well as non-hereditary form and showing an increasing incidence since the beginning of this century, which has nowadays stabilized near 1/16,000. Further differentiation is necessary in view of unilateral and bilateral as well as sporadic and familiar occurrence. Estimates of the relative frequencies of the resulting different groups are given und summarized in two tables in two different aspects.

The prognosis of retinoblastoma in terms of globe saving treatment. A computer assisted study. Part I.

At present there is no satisfactory classification or staging system. In order to compare the Reese classification (Reese & Ellsworth, 1963), the Essen classification (Höpping, 1983) and a new classification, a method of evaluating different parameters was created to get an optimal classification of globe saving treatment. A computer score table for the Essen eye clinic was established and this table could be valuable for all clinics using modern methods of treatment.

The prognosis of retinoblastoma in terms of survival. A computer assisted study. Part II.

At present there is no satisfactory classification or staging system. In order to compare the Reese classification (Reese, 1963), the Essen classification (Höpping, 1983) and a new classification, a method of evaluating different parameters was created in order to get an optimal classification of survival.

[Goniotomy in congenital glaucoma]. / La goniotomie dans le glaucome congénital.

Sixty eyes presenting with congenital glaucoma and operated on by goniotomy, have been followed-up for 1 to 30 years (mean 10.4 years). Intraocular pressure was normalized in 91 p. cent of cases, either by goniotomy alone with or without medical treatment, or by goniotomy with an associated fistulizing operation, if necessary. When preoperative intraocular pressure is less than 40 mmHg, 2 cases out of 3 are normalized, but when it is more than 40 mmHg, only 1 case out of 3 is normalized.

Retinal detachment and HLA typing.

105 patients with retinal detachment were HLA types and divided into hyperopic, emmetropic and myopic groups. None of these different groups showed a statistically significant correlation with any of the HLA antigens. For the patient group as a whole, there was a slightly significant correlation with HLA-B8 at the corrected p level of 0.05.

Aniridia-Wilms' tumor association and 11p interstitial deletion.

An 11p interstitial deletion is reported in a severely mentally retarded boy with aniridia, genito-urinary anomalies, and distinct craniofacial dysmorphism. At the age of 4 years a large Wilms' tumor of the right kidney was diagnosed.

Late extraocular tumours in retinoblastoma survivors.

Radiogenic or non-radiogenic osteosarcomas were found in approximately 15% of the observed bilateral retinoblastoma cases, and only exceptionally in the unilateral cases. Such osteosarcomas are also seen in subjects without retinoblastoma, but belonging to affected families. Late second malignant extraocular tumours were only observed when we were dealing with a germinal mutation, that is in hereditary retinoblastoma. They are due to a pleiotropic effect of a single gene situated on the chromosome D13 (q14), or to two different but very close genes.