Síndrome de Axenfeld-Rieder associada à maculopatia tipo "Bull's eye"

Costa, Vital P.; Katz, L. Jay; McNamara, J. Arch

doi:10.5935/0004-2749.19950075

RESUMO

A Síndrome de Axenfeld-Rieger representa um espectro de anomalias de desenvolvimento caracterizadas por alterações da periferia da córnea, íris e seio camerular, além de malformações ósseas e dentárias. Existem algumas descrições esparsas de anormalidades de fundo de olho, porém estas não foram documentadas. Nós descrevemos, pela primeira vez, um caso de Síndrome de Axenfeld-Rieger associada à maculopatia bilateral do tipo "bull's eye".

Palavras chave:
Síndrome de Axenfeld-Rieder; "bull's eye"; maculopatia; glaucoma

SUMMARY

The Axenfeld-Rieger syndrome mesodermal dysgenesis represents a spectrum of developmental disorders characterized by abnormalities of the peripheral cornea, íris and anterior chamber angle associated with developmental defects of the teeth and facial banes. Sparse descriptions of fundus abnormalities have been made, but none have been well documented. We report, to our knowledge for the first time, a case of Axenfeld-Rieger syndrome associated with bilateral "bull's eye" maculopathy.

Key words:
Axenfeld-Rieger; "bull's eye"; maculopathy; glaucoma

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REFERENCES

¹
ALKAMADE, P. P. H.; Dysgenesis Mesodermalis of the Iris and Cornea: A Study of Rieger's Syndrome and Peter's Anomaly. Assen. The Netherlands. Van Gorcum. 1969
²
SHIELDS, M. B. - Axenfeld-Rieger syndrome. A theory of mechanisms and distinctions from the iridocorneal endothelial syndrome. Trans. Am. Ophthalmol. Soc., 81: 736-784, 1983.
³
RIEGER, H. - Dysgenesis mesodermalis corneae et iridis. Z. Augenheilk. 86: 333-344, 1935.
⁴
FALLS, H. F. - A gene producing various defects of the anterior segment of the eye. Am. J. Ophthalmol., 32: 41-52, 1949.
⁵
FORSIUS, H. & ERIKSSON, A. - Embryotoxon corneae posterius in a family with slit-pupil and in cases with other anomalies of the iris. Acta Ophthalmol., 42: 68-77, 1964.
⁶
FORSIUS, H. - Visibility of the anterior borderring of Schwalbe in congenital corneal anomalies, tumors of the limbus and dental anomalies. Acta Ophthalmol., 42: 97-107, 1964.
⁷
LEMMINGSON, W. - Multiple Dysplasien in Verbindung mit der Dysgenesis mesodermalis corneae et iridis. Klin. Monatsbl. Augenheilk., 138: 96-102, 1961.
⁸
FONTAINE, M.; ARON, D.; ROUSSELIE, F. - Un cas d'embryotoxon posterieur. Bull. Soc. Ophtal. Fr., 63: 191-195, 1963.
⁹
MARX, P. - Un cas d'atrophie congenitale de I'iris associee a des malformations faciales (maladie de Rieger). Bull. Soc. Ophtalmol. Fr., 65: 178-182, 1965.
¹⁰
GASS, J. D. M. - Stereoscopic Atlas of Macular Diseases. Diagnosis and Treatment. 3rd edition. St. Louis, MO. CV Mosby 1987, pp. 256-331.
¹¹
O'DONNEL, F. E. & WELCH, R. B. - Fenestrated sheen macular dystrophy. Arch. Ophthalmol., 97: 1292-1296, 1979.
¹²
DEUTMAN, A. F. - Benign concentric annular macular dystrophy. Am. J. Ophthalmol., 78: 384-388. 1974.
¹³
VAN den BIESEN, P. R.; DEUTMAN, A. F.; PINCKERS, A. J. L. G. - Evolution of benign concentric annular macular dystrophy. Am. J Ophthalmol., 100: 73-77, 1985.

Datas de Publicação

Publicação nesta coleção
Apr 1995

Este é um artigo publicado em acesso aberto (Open Access) sob a licença Creative Commons Attribution, que permite uso, distribuição e reprodução em qualquer meio, sem restrições desde que o trabalho original seja corretamente citado.

[1] ¹
ALKAMADE, P. P. H.; Dysgenesis Mesodermalis of the Iris and Cornea: A Study of Rieger's Syndrome and Peter's Anomaly. Assen. The Netherlands. Van Gorcum. 1969

[2] ²
SHIELDS, M. B. - Axenfeld-Rieger syndrome. A theory of mechanisms and distinctions from the iridocorneal endothelial syndrome. Trans. Am. Ophthalmol. Soc., 81: 736-784, 1983.

[3] ³
RIEGER, H. - Dysgenesis mesodermalis corneae et iridis. Z. Augenheilk. 86: 333-344, 1935.

[4] ⁴
FALLS, H. F. - A gene producing various defects of the anterior segment of the eye. Am. J. Ophthalmol., 32: 41-52, 1949.

[5] ⁵
FORSIUS, H. & ERIKSSON, A. - Embryotoxon corneae posterius in a family with slit-pupil and in cases with other anomalies of the iris. Acta Ophthalmol., 42: 68-77, 1964.

[6] ⁶
FORSIUS, H. - Visibility of the anterior borderring of Schwalbe in congenital corneal anomalies, tumors of the limbus and dental anomalies. Acta Ophthalmol., 42: 97-107, 1964.

[7] ⁷
LEMMINGSON, W. - Multiple Dysplasien in Verbindung mit der Dysgenesis mesodermalis corneae et iridis. Klin. Monatsbl. Augenheilk., 138: 96-102, 1961.

[8] ⁸
FONTAINE, M.; ARON, D.; ROUSSELIE, F. - Un cas d'embryotoxon posterieur. Bull. Soc. Ophtal. Fr., 63: 191-195, 1963.

[9] ⁹
MARX, P. - Un cas d'atrophie congenitale de I'iris associee a des malformations faciales (maladie de Rieger). Bull. Soc. Ophtalmol. Fr., 65: 178-182, 1965.

[10] ¹⁰
GASS, J. D. M. - Stereoscopic Atlas of Macular Diseases. Diagnosis and Treatment. 3rd edition. St. Louis, MO. CV Mosby 1987, pp. 256-331.

[11] ¹¹
O'DONNEL, F. E. & WELCH, R. B. - Fenestrated sheen macular dystrophy. Arch. Ophthalmol., 97: 1292-1296, 1979.

[12] ¹²
DEUTMAN, A. F. - Benign concentric annular macular dystrophy. Am. J. Ophthalmol., 78: 384-388. 1974.

[13] ¹³
VAN den BIESEN, P. R.; DEUTMAN, A. F.; PINCKERS, A. J. L. G. - Evolution of benign concentric annular macular dystrophy. Am. J Ophthalmol., 100: 73-77, 1985.

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