A case of angle-closure glaucoma, cataract, nanophthalmos and spherophakia in oculo-dento-digital syndrome

• Published in: Graefe's archive for clinical and experimental ophthalmology 2003-02, Vol.241 (2), p.161-163
• Main Authors: WIDDER, Randolf A, ENGELS, Bert, SEVERIN, Maria, BRUNNER, Richard, KRIEGLSTEIN, Günter K
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple - diagnosis; Abnormalities, Multiple - etiology; Adult; Biological and medical sciences; Cataract - diagnosis; Cataract - etiology; Cataract - therapy; Cataract Extraction; Dental Enamel - abnormalities; Dental Enamel - pathology; Diseases of the osteoarticular system; Eye Abnormalities - diagnosis; Eye Abnormalities - etiology; Female; Glaucoma; Glaucoma and intraocular pressure; Glaucoma, Angle-Closure - diagnosis; Glaucoma, Angle-Closure - etiology; Glaucoma, Angle-Closure - surgery; Humans; Intraocular Pressure; Lens Implantation, Intraocular; Lens, Crystalline - abnormalities; Lens, Crystalline - pathology; Malformations and congenital and or hereditary diseases involving bones. Joint deformations; Medical sciences; Microphthalmos - diagnosis; Microphthalmos - etiology; Ophthalmology; Syndactyly - diagnosis; Syndactyly - etiology; Syndrome; Visual Acuity
• ISSN: 0721-832X; 1435-702X
• DOI: 10.1007/s00417-002-0619-z

We report a new case of oculo-dento-digital syndrome. Case report. 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. 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.