Introduction: Marfan syndrome is a rare genetic disorder linked to the mutation in the FBN1 gene, which codes for fibrillin. It is transmitted in the autosomal dominant mode. Its prevalence is estimated at 1/5000 individuals. Cardiovascular, skeletal and ophthalmic disorders are the most frequent.

Clinical Observation: We report the case of a 9-year-old male child with uneventful family history brought by his parents for blurred vision. The visual acuity without correction was “count fingers at 2 meters”. Slit lamp examination showed bilateral posterior dislocation of the lens in the vitreous, the Intra-ocular pressure (IOP) was 12 mmHg in both eyes, the fundus was normal. The general examination found a tall child measuring 1.48 m of height for 26 kg. He had slender limbs showing the sign of the thumb (Steinberg) and the sign of the wrist along with a moderate pectus excavatum. Cardiac ultrasound showed dilation of the ascending aorta. According to Gant's criteria (Ghent), we concluded to the diagnosis of Marfan syndrome.

Discussion: The diagnosis of Marfan syndrome is difficult to establish. At first, it is mainly clinical with the association of major and minor signs. Genetic research is possible as well as skin biopsy.

Lens ectopia is the most common ophthalmologic manifestation in Marfan syndrome. According to Gant's criteria, we concluded to the diagnosis of Marfan syndrome.

Conclusion: Marfan syndrome is oftentimes discovered during an ophthalmic consultation. The management of its various disorders and the follow-up imply a frank collaboration between many practitioners.

Keywords: Dislocation of the Lens; Marfan; Child

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Citation

Citation: Japhet Pobanou Thera.,et al. “Lens Dislocation in a 9-Year-Old Child with Marfan Syndrome”. Acta Scientific Ophthalmology 3.6 (2020): 24-26.

Copyright

Copyright: © 2020 Japhet Pobanou Thera., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.