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To determine the visual outcome of surgical therapy and conservative management, we retrospectively reviewed all our patients with ectopia lentis and Marfan's disease.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).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.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.

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.

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.

A digital programme of wave form recognition was applied to a nystagmogram so as to pave the way for an average chronotopographic study of the occipital EEG activity triggered by the start of the rapid stage of the nystagmus. The programme was used in a clinical case of congenital nystagmus associated with a visual deficiency (coloboma of the optic nerve), with the following results: (i) in the patient examined, no average evoked response was observed either with eyes closed or with eyes open in the dark; (ii) an average response of small amplitude was observed with eyes open centred on a homogeneous illuminated field; this response was reinforced (greater amplitude and more complex structure) by a patterned field (checkerboard); (iii) the response to nystagmus showed characteristics similar to those of lambda response in the same subject. These results appear to confirm the conclusions of Bender and Shanzer (1964) according to which the calcarine activity in monkeys in response to experimental nystagmus is “light and vision dependent and correlates with the visual input and not with the oculomotor output”. Differences in amplitude, latency, and structure between the various responses recorded in the patient examined (response to nystagmus, to appearance of lighted checkerboard, and lambda response) and the same responses in normal subjects are considered and discussed in relation to factors associated with visual deficiency.

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.

Between April 3, 1977 and June 6, 1980, 97 children with congenital cataracts and 97 evenly matched control children were examined in order to clarify the biological criteria of congenital rubella and to estimate its importance in the etiology of congenital cataracts. Although the age limit had been fixed at 60 months, the 1978 rubella outbreak accelerated the immunization in young children and resulted in difficulties in interpreting some results. All children presenting with congenital cataracts associated with clinical symptoms of rubella embryopathy displayed anti-rubella antibodies including anti-rubella IgM up to the 13th month: they represent 16% of cases with congenital cataracts in this series. The rubella etiology could not be proven in children with clinically isolated congenital cataracts.

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.

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.

The anterior transcorneal approach, using Klötis apparatus, was employed to conduct phacophagia in 29 eyes. This transcorneal approach to an ectopic lens is sufficient if care is taken to place the keratotomy on the meridian opposite to that of the lens displacement. Patients have been followed up for from 1 to 5 years and anatomical results are excellent; no complications have been reported, but this technique cannot be employed in patients over 30 years of age. Indications and contraindications for this method are discussed, together with the advantages and inconveniences of other techniques: discussion-aspiration and phacophagia through the pars plana.