Korean J Ophthalmol.  2005 Mar;19(1):84-89. 10.3341/kjo.2005.19.1.84.

Anterior Lens Capsule Abnormalities in Alport Syndrome

• Choi JH
• Na KS
• Bae SH
• Roh GH

• KMID: 1099092
• DOI: http://doi.org/10.3341/kjo.2005.19.1.84

Abstract

Alport syndrome is a hereditary, progressive disease characterized by progressive nephritis, sensorineural deafness, and ocular abnormalities, including anterior lenticonus. The ultrastructure of the lens capsule abnormalities in Alport syndrome is reported. Four anterior lens capsules from 31-year-old patient and 26-year-old patient with lenticonus who were affected by the Alport syndrome were obtained at capsulectomy. And all four anterior lens capsules were examined by transmission electron microscopy. The histopathologic findings showed that the thickness of the anterior lens capsules was decreased (4~13 micrometer) and that there were many vascular dehiscences localized at the inner part of the lens capsule. There were large numbers of capsular dehiscences containing fibrillar materials and vacuoles. The anterior capsules were clearly fragile in this disease, forming the basis for the progressive lenticonus and anterior polar cataract.

Keyword

Alport syndrome; Capsular dehiscence; Lenticonus

MeSH Terms

Adult
Epithelial Cells/ultrastructure
Humans
Lens Capsule, Crystalline/*ultrastructure
Lens Diseases/genetics/*pathology
Lens Implantation, Intraocular
Male
Nephritis, Hereditary/genetics/*pathology
Phacoemulsification

Figure

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  Fig. 1 Slit-lamp photograph showing anterior lenticonus (arrowhead) and anterior subcapsular faint opacity (white arrow). Right eye (A), Left eye (B)

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  Fig. 2 Fundus photograph in the right eye. Yellow punctate lesions (black arrow) in the retinal pigment epithelium level that spared the macula.

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  Fig. 3 Transmission electron microscope of anterior lens capsule and epithelium. Capsule (C) is 4.8 µm thick with vertical dehiscences (black arrow). The dehiscences are located in the inner two-thirds of the anterior lens capsule. The outer one-third of the capsule was looser and of fibrillar texture. The lens epithelium (E) is highly irregular shaped. (Original magnification ×5400).

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  Fig. 4 Transmission electron microscope of the anterior lens capsule. The dehiscences forms a network (black arrow) within the capsule. (Original magnification ×6300).

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  Fig. 5 High transmission electron microscopic magnification of the anterior lens capsule. Dehiscences contain fibrillar material and vacuole (arrowhead). (Original magnification ×6300).

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  Fig. 6 Transmission electron microscopic view of the lens epithelium. (A) The lens epithelial cells are highly irregular and their lateral borders are indistinct. The nucleus (N) is smaller, darker, and irregular margined. The cytoplasm contains numerous lacunae (arrowhead). There is a paucity of other organelles. (B) Double-layered lens epithelial cells (black arrow). (C) In focal areas the lens epithelial cells are well preserved. Epithelial cells are cuboidal-shaped and their lateral borders are closed joined.

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