Abstract | INTRODUCTION: CASE REPORT: This 8-year-old girl was a sporadic case, born of consanguineous parents. She was referred to our hospital for intermittent strabismus. Her visual acuity was 4/10 at the right eye and 3/10 at the left eye. Biomicroscopy revealed very fine crystals in the limbal area bilaterally. Mydriatic funduscopic examination showed bilateral macular pigment mottling and depigmentation, numerous tiny refractile yellow dots scattered throughout the posterior pole and the mid-periphery associated with diffuse retinal pigment epithelial atrophy and pigment accumulation. Fluorescein angiography revealed retinal pigmentary epithelium alterations. Indocyanine green (Infracyanin) angiography showed areas of choroidal atrophy. The electroretinogram noted a reduction in the number of both types of photoreceptors. COMMENTS: Ophthalmological lesions normally occur between 20 and 30 years of age. The particularity of our case report is the manifestation of the disease at an earlier age (8 years). The progression is characterized by a centrifuge expansion of lesions.
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Authors | N Chaker, F Mghaieth, R Baccouri, A Merdassi, F Turki, L El Matri |
Journal | Journal francais d'ophtalmologie
(J Fr Ophtalmol)
Vol. 30
Issue 1
Pg. 39-43
(Jan 2007)
ISSN: 1773-0597 [Electronic] France |
Vernacular Title | Caractéristiques cliniques et angiographiques de la dystrophie cristalline de Bietti: à propos d'une patiente âgée de 8 ans. |
PMID | 17287670
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Fluorescent Dyes
- Indocyanine Green
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Topics |
- Age of Onset
- Atrophy
- Child
- Choroid
(blood supply, diagnostic imaging, pathology)
- Consanguinity
- Corneal Dystrophies, Hereditary
(diagnostic imaging, pathology)
- Crystallization
- Disease Progression
- Electroretinography
- Esotropia
(etiology)
- Female
- Fluorescein Angiography
- Fluorescent Dyes
- Humans
- Indocyanine Green
- Limbus Corneae
(ultrastructure)
- Microscopy, Acoustic
- Mosaicism
- Pigment Epithelium of Eye
(pathology)
- Radiography
- Retinitis Pigmentosa
(diagnostic imaging, genetics, pathology)
- Turner Syndrome
(genetics)
- Visual Acuity
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