[Surgery of ectopia lentis in Marfan disease in children and young adults]. / Chirurgie de l'ectopie cristallinienne dans la maladie de Marfan chez l'enfant et l'adulte jeune.

INTRODUCTION: To determine the visual outcome of surgical therapy and conservative management, we retrospectively reviewed all our patients with ectopia lentis and Marfan's disease. MATERIAL AND METHOD: Thirty-five eyes of 18 patients (age 8 to 47, average 25.3) were studied. Eight eyes were followed conservatively, the remaining 27 eyes underwent a lensectomy either with the Klöti vitreous stripper (18) or using other techniques (9). RESULTS: Over follow-up periods ranging from 0.5 to 13 years (average 6.2 years) low visual acuity was found in non-operated eyes. Retinal detachment occurred in 2 out of 8 eyes. Eyes undergoing other surgical techniques had a mean final visual acuity of 0.2. Retinal detachment occurred with high frequency (6 out of 9 eyes). Lensectomy-vitrectomy was a safe and effective procedure with a mean final visual acuity of 0.6. No retinal detachment or secondary glaucoma were found. COMMENT: Our study suggests that performing lensectomy-vitrectomy with the Klöti vitreous stripper is the best surgical approach in ectopia lentis with Marfan's disease. This technique allows the vitreous to be handled more effectively and has led to improve results.

[Surgical treatment of non-traumatic retinal detachment in children under 15 years of age]. / Traitement chirurgical des décollements de rétine non traumatiques de l'enfant de moins de 15 ans.

Etiological and clinical features of 151 retinal detachments in childhood are studied and discussed. Results of surgical management of 92 of them, after at least a 6 month follow-up, especially after ablation of silicon oil when used temporarily, are described. Clinical aspects of the contralateral eyes and their prophylactic treatment is also studied. Though surgical techniques have done great strides, prognosis of retinal detachment in childhood is still desperately poor. Proliferative vitreo-retinopathy, frequent, early and very progressive, is the leading complication and cause of failure in surgical of these retinal detachment. In this series of 151 retinal detachments, only 38% have achieved long term retinal reattachment because of it. If high grade (C-D) proliferative vitreo-retinopathy is associated 14.5% only achieved long term reattachment though 86% it when proliferative vitreo-retinopathy was moderate or absent. It is for this reason that we wish to emphasize the need to inform practitioners and "at risk" families. Strict and systematic follow-up of the "at risk eye" is essential. The most complete surgery at the outset seems to be the best way of managing high-grade proliferative vitreo-retinopathy associated with retinal detachments.

[Surgical treatment of traumatic retinal detachment in children under 15 years of age]. / Traitement chirurgical du décollement de rétine traumatique de l'enfant de moins de 15 ans.

The clinical features and results of surgical management of 68 out of a series of 101 cases of traumatic retinal detachment in childhood are described and analysed. Follow-up, in particular after the removal of silicon oil used temporarily, was always been longer than six months. Cases were divided into three groups: contusions, ocular injuries without foreign body and with foreign body. Cases of retinal detachment related to traumatic aphakia, severe myopia and associated with proliferative vitreo-retinopathy were also studied. The prognosis of retinal detachment in childhood, the main characteristics of which are late diagnosis and the early and rapid growth of proliferative vitreo-retinopathy appears to be worse in this series than usually published figures. Proliferative vitreo-retinopathy is the leading cause of surgical failure. The long term retinal reattachment rate did not exceed fifty per cent and the functional success rate was only thirty-three per cent. It is for this reason that the authors wish to emphasize the need for the provision of information to families and to ophthalmologists responsible for the management of these "high risk eyes". It is felt that the most complete surgery possible should be performed at the outset when proliferative vitreo-retinopathy exists.

[Cyclodiathermy: is it effective in the treatment of glaucoma?]. / La cyclodiathermie: a-t-elle encore une valeur dans le traitement du glaucome?

The authors describe the non penetrating technique of cyclodiathermy in the treatment of different types of glaucoma and publish the results and the complications of the application of this technique on thirty three eyes among their patients. These eyes included congenital glaucoma (8 eyes), aphakic glaucoma (5 eyes), after silicone surgery (5 eyes), neovascular glaucoma (4 eyes), traumatic glaucoma (4 eyes), secondary glaucoma (3 eyes), Sturge-Weber-Krabbe (2 eyes) and open angle glaucoma (2 eyes). Their study with a mean follow up of 2.5 years shows an overall success rate of 60%. The success is defined as an ocular tension less than or equal to 22 mmHg in the absence of serious complications. All the encountered complications are mentioned. Phtysis occurred in not more than 3% of cases (1 eye). They establish a comparison with the published results and complications of the other cyclodestructive procedures: Sonocare, transcleral laser Yag, transcleral laser Ruby and cyclocryotherapy. This comparison does not prove the superiority of these other procedures with regard to the non penetrating cyclodiathermy. They conclude that this non penetrating cyclodiathermy is a non expensive procedure which still have its good therapeutic value.

[Classification of retinal degeneration in children]. / Classification des dégénérescences rétiniennes de l'enfant.

A classification of the hereditary retinal degenerations in childhood is presented. It is based on an histopathologic approach. It includes rods and cones dysfunctions, pigment epithelium degenerations and vitreoretinal degenerations. Each of them is studied according its own clinical, electrophysiologic, evolutive and genetic characteristic features.

[Benign isolated 6th nerve paralysis in children. Developmental aspects apropos of 9 new cases]. / Les paralysies isolées et bénignes du VI nerf crânien chez l'enfant. Aspects évolutifs à propos de 9 nouveaux cas.

Benign isolated VI nerve palsy in children is a rare but now well-established clinical entity. The diagnosis is essentially an exclusion one. We report nine additional cases. The evolution is discussed, with reference to the literature.

[6 new cases of spasmus nutans]. / Six nouvelles observations de spasmus nutans.

Six new born presenting an acquired nystagmus, associated with rhythmic head movements, have been explored clinically, electrophysiologically and neuroradiologically. Etiopathogenic hypotheses and experimental models are proposed. A twenty last years literature review is mentioned. It seemed interesting to authors to practice head scanners to find an organic lesion. The ethnic incidence is not negligible.

[Juvenile aggressive palpebral fibromatosis. Apropos of a case]. / Fibromatose juvénile agressive palpébrale. A propos d'un cas.

Juvenile Aggressive Fibromatosis (JAF) is histologically characterized by a fibroblastic cellular proliferation. Even though benign, it is locally invasive and very often recurrent but never induces metastasis; the evolutionary argument is the only one which enables us to differentiate JAF from fibrosarcoma. The selected treatment is extensive surgical excision including healthy tissue with histological control. The prognosis is variable depending upon localization and therapeutic possibilities. We report the case of a seven year-old girl presenting an eyelid localization which is seldom reported. The extraorbital and intracranial extension was fatal in spite of the therapy associating surgery, radiotherapy and chemotherapy. The central nervous system proximity poses a difficult problem and speaks for an early mutilating surgery. However, the small number of published cases does not yet allow codification of treatment.

Magnetic resonance imaging of the opto-chiasmatic region. Report on 276 cases.

A total of 276 lesions in the sellar and parasellar region were studied with magnetic resonance imaging using two different magnets of 0.15 and 0.5 T, respectively. Examination in the sagittal plane is recommended. T1 weighted images will give anatomic details, T2 weighted images the tissue characteristics. No marked differences with regard to diagnostic results were noted with the two different magnets, although the signals appeared to be more intense when using a 0.5 T magnet.

[Sarcoidosis in a child disclosed by blindness with major hypercalcemia]. / Sarcoïdose de l'enfant révélée par une cécité avec hypercalcémie majeure.

We report here a case of sarcoidosis, observed in a 12 year-old algerian girl. When the child was examined, at an advanced stage of the disease, her clinical condition was very critical, associating blindness, cachexia, hepatic and splenic enlargement, and major hypercalcaemia. Though chest roentgenograms were normal, the results of both functional pulmonary tests and broncho-alveolar lavage were pathologic. The diagnosis of sarcoidosis was confirmed by the finding of an elevated level of Angiotensin-converting enzyme, and by histologic lesions observed in liver, kidney and conjunctiva biopsies. The corticosteroid treatment improved the general condition, but it failed to better the visual state.

[Strabismus and amblyopia. Value of their early detection]. / Strabisme et amblyopie. Intérêt de leur dépistage précoce.

The authors emphasize the importance of the relations between strabismus and amblyopia. Strabismus is often the symptom which leads to the discovery of amblyopia. Amblyopia may cause strabismus, as in organic amblyopias induced by congenital lesions of the retina or visual pathways, and visual deprivation amblyopias due to corneal opacities and/or congenital cataracts. Unilateral clouding of normally transparent media (cornea, lens, vitreum) prevents normal stimulation of the retina which is necessary to the development of binocular vision. Anisometropic amblyopias also belong to this category. More often, amblyopia is the consequence of strabismus: it is then termed "functional strabismic amblyopia". Loss of the parallelism of the two visual axes disrupts the functional balance between the images received from the two eyes. The two images are different; one is suppressed. This suppression may eventually lead to amblyopia. Amblyopia was discovered in 593 of 1 757 studied cases of strabismus. Amblyopia was the cause of strabismus in 87 patients and was functional in the remaining 506. Among these latter, visual acuity was inferior or equal to 1/10 in 241 cases and between 2/10 and 7/10 in 265. 147 children in the first group and 136 in the second could be treated. Among these patients, who were treated before the age of seven, complete recovery was obtained in approximately 1/2 of cases and improvement in more than 1/4 of first group patients and 1/3 of second group patients. Results were all the better that treatment was initiated earlier and that patients were younger.(ABSTRACT TRUNCATED AT 250 WORDS)