[Optic disc drusen in children: Advantages of various imaging modalities]. / Druses de la papille de l'enfant : intérêt des différents examens d'imagerie.

In children, optic disc drusen pose a diagnostic problem with papilledema when they are buried. The goal of our study is to retrospectively compare the results of examinations performed in 34 children with optic disc drusen. In children who underwent ultrasonography and autofluorescence imaging, the results were conclusive in both examinations in 44.7% of cases, and in neither of the two examinations in 27.6% of cases. Ultrasonography alone demonstrated superiority in diagnosing 27.6% of eyes, whereas autofluorescence imaging was inconclusive. Since diagnostic confusion with papilledema persisted in seven children, angiography was performed. B-mode ultrasonography constitutes a relevant examination in the diagnosis of buried optic disc drusen in children and shows superiority in our study compared to autofluorescence imaging.

[Congenital achromatopsia: electroretinogram in early diagnosis]. / Achromatopsie congénitale: interêt de l'électrorétinogramme pour le diagnostic précoce.

PURPOSE: Achromatopsia is a hereditary disease responsible for congenital low vision. Patients present with nystagmus, abnormal visual behavior or photophobia. Only the electroretinogram (ERG) can confirm the diagnosis in infants. PATIENTS AND METHODS: Thirty children referred for nystagmus or low vision were included in this retrospective study. A complete ophthalmological examination, an ERG and when possible a color vision test (Ishihara, Farnsworth 15 Hue test) was done. A Ganzfeld ERG was performed in accordance with ISCEV standards in patients more than 6 years of age. In younger patients, a simplified method using electroluminescent diode stimulation was used and a comparative ERG in accordance with ISCEV standards was performed when the patients were old enough. RESULTS: The ERG response was identical in children and adults. It confirmed the diagnosis of achromatopsia: the scotopic components obtained in dark adapted conditions were normal, (scotopic a-wave, b2 wave). The photopic components, recorded in light-adapted conditions, in order to inhibit the scotopic response (photopic wave, b1 wave), were not recordable. The color vision tests confirmed color blindness; however, in some patients color denomination was correct. CONCLUSION: The simplified ERG procedures performed in our series were reliable in detecting achromatopsia. However, it may not be sufficient to discriminate complete from incomplete achromatopsia.

[Simplified electroretinography protocol and diagnosis of retinal dystrophies in children]. / Protocole simplifié d'électrorétinographie et diagnostic des dystrophies rétiniennes de l'enfant.

PURPOSE: To report results of a simplified electroretinogram in children. PATIENTS: 124 children under 6 years of age with nystagmus, blindness, neurological disease, cone rod dystrophy in the family, or abnormal fundus appearance were examined. METHOD: The electroretinogram was recorded by corneal electrodes in an awake state without sedation. A light-emitting orange diode stimulator was used. Stimulation was performed subsequently after 3 minutes of light adaptation and after 8 minutes of darkness. If the electroretinogram was abnormal, a second recording was done a few weeks later using the same method. In some cases, the children were re-examined, and a ganzfeld stimulation ERG was recorded. RESULTS AND DISCUSSION: This method allowed a reliable diagnosis of photoreceptor dystrophy: Leber's congenital amaurosis with or without rare metabolic diseases, X link pigmentary retinopathy or retinal dystrophy with general disease in children. It helped to differentiate isolated retinal pigment changes with normal electroretinogram from functional retinal impairment and rod-cone dystrophy from other retinal dystrophies.

[Jadassohn's sebaceous nevus]. / Naevus sébacé de Jadassohn.

We report the case of a child's Jadassohn sebaceous noevus, localised on the vertex cranii, the trunk and the limb, associated with the presence of ophthalmological and neurological abnormalities. The diagnosis of this syndrome has been difficult because all the signs were not present at birth. The surgical treatment of the vertex cranii lesions will be realised after expansion with sub cutaneous prothesis, allowing a large exeresis and an adequate reconstruction. Only the anatomo-pathological examination allows any malignant degeneration of the lesion.

[Clinical aspects of Coats]. / Aspects cliniques de Coats.

Coats' disease associates on exudative retinopathy to vascular telangiectasis. The authors analyse the different factors which take part to many clinical aspects of the Coats' exudation: the age of the patients and the vascular trouble which causes the exudative retinopathy.

[A protocol for contact lenses adaptation in aphakia and myopia in children; surveillance and follow-up]. / Protocole d'adaptation des lentilles de contact de l'aphakie et de la myopie chez l'enfant; surveillance et suivi.

The authors report their procedure of contact lens adaptation in child and particularly after traumatic cataract. Adaptation in the briefest time following cataract surgery is absolutely necessary in order to undertake jointly the orthoptic treatment of amblyopia.

[Difficulty in diagnosing an orbital non-Hodgkin's lymphoma]. / Difficulté du diagnostic d'un lymphome non Hodgkinien à localisation orbitaire.

History case of a patient treated for a bilateral orbital inflammatory pseudotumor has been rehospitalised for generalised lymphoma, despite the histological features of this tumor which evoked a pseudo-tumor. This error seems to be frequent because of the difficulties for the histological investigation of the tumor. Actually the application of immunohistochemical techniques and the study of immunoglobulin gene DNA rearrangement patterns allows the diagnosis. Often most of the orbital lymphomas, though apparently isolated, represent a localization of a systemic disease. Their treatment consists in either of radiotherapy in low grade malignancy localized cases or in chemotherapy in the others sometimes associated to radiotherapy.