Abstract
Background
We report a new case of oculo-dento-digital syndrome.
Methods
Case report.
Results
We saw a 34-year-old women with oculo-dento-digital syndrome. Visual acuity was no perception of light (RE) and 1/35 (LE). Biomicroscopy revealed a flat anterior chamber and an advanced cataract. The intraocular pressure (IOP) was 60 mmHg in both eyes. Ultrasonographic biomicroscopy demonstrated a closed chamber angle in both eyes. Measurements of the axial length and of the diameter of the lens were 18.7 mm/6.0 mm for the RE and 18.7 mm/5.8 mm for the LE respectively. In the time following we conducted a cyclodestructive procedure in the RE and a cataract extraction with implantation of an intraocular lens in the LE. This led to a considerable reduction of the IOP in the RE and combined with local therapy to IOP regulation in the LE.
Conclusion
In this patient nanophthalmos, cataract and spherophakia led to angle-closure glaucoma in both eyes. We recommend early monitoring of IOP, axial length and lens diameter. This case demonstrates that an early cataract extraction might beneficially influence the natural course of the disease.
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References
Braun M, Seitz B, Naumann GOH (1996) Juveniles Offenwinkelglaukom mit Mikrocornea bei okulo-dento-digitaler Dysplasie (Meyer-Schwickerath-Weyers). Klin Monatsbl Augenheilkd 208:262–263
Dietlein TS, Mietz H, Jacobi PC, Krieglstein GK (1996) Spherophakia, nanophthalmia, hypoplastic ciliary body and glaucoma in brachydactyly-associated syndromes. Graefes Arch Clin Exp Ophthalmol 234:187–192
Dudgeon J, Chisholm IA (1974) Oculo-dento-digital dysplasia. Trans Ophthalmol Soc UK 94:203–210
Gladwin A, Donnai D, Metcalfe K, Schrander-Stumpel C, Brueton L, Verloes A, Aylsworth A, Toriello H, Winter R, Dixon M (1997) Localization of a gene for oculodentodigital syndrome to human chromosome 6q22-q24. Hum Mol Genet 6:123–127
Judisch GF, Aurelio MC, Hanson JW, Olin WH (1979) Oculodentodigital dysplasia. Four new reports and a literature review. Arch Ophthalmol 97:878–884
Marchesani O (1939) Brachydaktylie und angeborene Kugellinse als Systemerkrankung. Klin Monatsbl Augenheilkd 103:392–406
Meyer SJ, Holstein T (1941) Spherophakia with glaucoma and brachydactyly. Am J Ophthalmol 24:247–257
Meyer-Schwickerath G, Grüterich E, Weyers H (1957) Mikrophthalmussyndrome. Klin Monatsbl Augenheilkd 131:18–30
Novotny L, Sterbova S (1984) Meyer-Schwickerath Syndrom mit Mikrophthalmus. Klin Monatsbl Augenheilkd 185:282–285
Pfeiffer RA, Erpenstein H, Junemann G (1968) Oculo-dento-digitale Dysplasie. Klin Monatsbl Augenheilkd 152:247–262
Ritch R, Wand M (1981) Treatment of the Weil-Marchesani syndrome. Ann Ophthalmol 13:665–667
Sugar HS (1978) Oculodentodigital dysplasia syndrome with angle-closure glaucoma. Am J Ophthalmol 86:36–38
Traboulsi EI, Parks MM (1990) Glaucoma in oculo-dento-osseous dysplasia. Am J Ophthalmol 109:310–313
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Widder, R.A., Engels, B., Severin, M. et al. A case of angle-closure glaucoma, cataract, nanophthalmos and spherophakia in oculo-dento-digital syndrome. Graefe's Arch Clin Exp Ophthalmol 241, 161–163 (2003). https://doi.org/10.1007/s00417-002-0619-z
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DOI: https://doi.org/10.1007/s00417-002-0619-z