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. 2001 Dec;85(12):1421–1425. doi: 10.1136/bjo.85.12.1421

Histopathological examination of two cases of anterior staphyloma associated with Peters' anomaly and persistent hyperplastic primary vitreous

A Matsubara 1, H Ozeki 1, N Matsunaga 1, M Nozaki 1, M Ashikari 1, S Shirai 1, Y Ogura 1
PMCID: PMC1723813  PMID: 11734512

Abstract

AIMS—To clarify the developmental mechanism and critical period for the uncommon complex of Peters' anomaly and persistent hyperplastic primary vitreous (PHPV).
METHODS—Two eyes with Peters' anomaly and PHPV were histologically examined by serial section. One eye was enucleated at age 7 months (case 1) and the other at age 4 months (case 2) owing to severe anterior staphyloma.
RESULTS—In both eyes, defects in the endothelium, Descemet's membrane, and posterior stroma were observed in the central cornea, and the degenerative lens adhered to the posterior surface of the defective corneal stroma. Also, in both eyes, the anterior chamber space was not formed and the undifferentiated iris stroma adhered to the posterior surface of the peripheral cornea. Mesenchymal tissue containing melanocytes was observed behind the degenerative lens, and the pigment epithelium was absent at the lower nasal side of the ciliary body in case 1. In case 2, mesenchymal tissue containing scattered melanocytes in the vitreous cavity was seen on the posterior retina. Based on the histological findings, both cases were diagnosed as Peters' anomaly caused by the faulty separation of the lens vesicle, PHPV, maldevelopment of the iris and ciliary body, and goniodysgenesis.
CONCLUSION—Migratory disorders of neural crest cells from 4 to 7 weeks of gestation may be responsible for various ocular anomalies including Peters' anomaly and PHPV, as observed in these cases.



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Figure 1  .

Figure 1  

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(A) A photograph of anterior segment of case 1. Central corneal opacity, elongated ciliary processes, and whitish mass behind the lens are observed. (B) A photograph of anterior segment of case 2. The cornea is noticeably protruding and densely opaque overall. These findings correspond to anterior staphyloma.

Figure 2  .

Figure 2  

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(A) Photograph of the section of the eyeball of case 1. The degenerative lens (small arrow) adheres to the defective central cornea, the whitish mass (arrowhead) is observed behind the lens, and ciliary coloboma is seen (large arrow). Elongated ciliary processes are also observed. (B) Photograph of the section of the eyeball of case 2. The degenerative lens (arrow) adheres to the defective central cornea, and the whitish membranous tissue (asterisks) is observed on the posterior retina.

Figure 3  .

Figure 3  

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(A). A histological photograph of the anterior segment of case 1. The degenerative lens (large asterisks) adheres to the posterior surface of the defective corneal stroma. The anterior chamber space is not formed, and the undifferentiated iris stroma (arrows) adheres to the posterior surface of the peripheral cornea and lens. Mesenchymal tissue (small asterisk) is observed behind the degenerative lens. Elongated ciliary processes (arrowheads) are also observed. Haematoxylin and eosin. Bar = 700 µm. (B). A histological photograph of the anterior segment of case 2. In the central cornea, the defects in the endothelium, Descemet's membrane, and posterior stroma are observed, and the lens material (arrows) is detected in the defective corneal stroma. The anterior chamber space is not formed, and the undifferentiated iris stroma (asterisks) adheres to the posterior surface of the peripheral cornea. Haematoxylin and eosin. Bar = 280 µm.

Figure 4  .

Figure 4  

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(A) A histological photograph of the central cornea of case 1. In the central cornea, defects in the endothelium, Descemet's membrane, and posterior stroma are observed. Haematoxylin and eosin. Bar = 70 µm. (B) A histological photograph of the iris, ciliary body, and trabecular meshwork of case 2. The iris (asterisks), ciliary body (arrows), and trabecular meshwork are poorly differentiated, and Schlemm's canal is not observed. Haematoxylin and eosin. Bar = 175 µm.       

Figure 5  .

Figure 5  

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(A) A histological photograph of the mesenchymal tissue behind the lens of case 1. A few melanocytes (arrows) are observed in the mesenchymal tissue. Haematoxylin and eosin. Bar = 28 µm. (B) A histological photograph of the mesenchymal tissue on the retina of case 2. Mesenchymal tissue (asterisks) containing scattered melanocytes in the vitreous cavity are seen on the retina, inducing folds in the neural retina. Haematoxylin and eosin. Bar = 280 µm.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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