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To report the clinical investigation of isolated microspherophakia involving a Senegalese family in order to appreciate its functional impact.This is a rural family comprised of 7 members. The sibship included three girls and two boys. One of the girls, who lived in a distant zone, was unable to be examined. Of all the examined members of the family, only the father was unaffected by the illness. There was no consanguinity. The general medical examination was normal. The best-corrected visual acuity (VA) for the girls was 2/10. For one of the boys, BCVA was 8/10 for both eyes, and for the other, BCVA was 10/10 for the right eye and 8/10 for the left eye. The mother's VA was 10/10 and P2 without correction. Myopia and astigmatism were present in the 4 children of the sibship. During the examination, we noted the presence of small crystalline lenses, which were very round and presented an abnormal visibility of the lens equator and zonular fibers. The diagnosis of microspherophakia was confirmed by measurement of the lens diameters by ultrasound biomicroscopy. Complications were present in the girls, including pupillary block glaucoma and amblyopia for the elder, and retinal degeneration and amblyopia for the younger daughter. The elder daughter was managed medically with glaucoma drops. The younger daughter received optical correction and a prophylactic Argon LASER treatment. The two boys received optical correction. No treatment was recommended for the mother.Microspherophakia is a rare condition. Some serious complications can lead to amblyopia. A better multidisciplinary evaluation would allow for early detection and a better prognosis.

Diagnosing Marfan syndrome in young children is difficult because of the great variability of expression of the disease and because the phenotype evolves over the life course. The goal of this retrospective study was to describe the first clinical symptoms in children under 10 years of age with Marfan syndrome and to evaluate the pertinence of the new 2010 Ghent criteria in comparison with the 1996 criteria. Seventeen patients under 10 by the time of the first medical examination were included. All children had an FBN1 gene mutation that was secondarily demonstrated. Clinical data including ophthalmological, cardiac, and orthopaedic examinations obtained during the first medical examination were analyzed. The most frequent abnormalities encountered were high arched palate (82%), arachnodactyly (71%), and flatfoot (59%). Aortic aneurysm (47%) and ectopic lens (35%) were also seen at the time of diagnosis. According to the 2010 Ghent criteria, the diagnosis of Marfan syndrome could be obtained in 71% of patients after identification of the mutation of the FBN1 gene, whereas only 59% of patients were diagnosed using the older criteria. All organs can be affected during childhood. An early diagnosis is essential in order to set up specific management.

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 analysis of the results of surgical treatment of ectopia lentis by different technics used since twenty years on 113 cases demonstrates that: --In spite of satisfactory results obtained by discission alone of the lens when surgical possibilities were limited, this method must now be given up.--Intra-capsular extraction is still valuable when the lens is luxated in the anterior chamber or cataracted.--Extra-capsular extraction remains nowadays the best technic:--by classical discission aspiration associated to an anterior vitrectomy in case of rupture of the hyaloid, when the lens is moderately subluxated;--by means of the vitreotome through the pars plana when the lens is totally luxated.

Report of three cases of congenital leukomas with anomalies of the lens migration: first case is an early malformation with "Wedl" cells in the corneal stroma; second case is a Peters' anomaly with ectopic and atrophic lens; third case, with a six years follow up after birthday, shows corneal opacification and regression of the lens. Ultrastructural study of these two last cases.

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.