Glaukom bei Marfan-Syndrom: Lageabhängige Druckmessung als diagnostisches Kriterium

Summary

Patient A 35-years-old female patient with Marian's syndrome presented with fast progressive reduction of visual acuity (hand movements) of her right eye and lens-related glaucoma on both eyes. On examination, subluxation of the lens and nuclear cataract were seen on both eyes. Diurnal pressure curve showed great fluctuations in pressure with normal IOP in the morning and elevated pressure in the evening. Pressure measurement revealed great fluctuations depending on positioning. Because of cataractous lens in the right eye blocking the visual axis and uncontrollable, phacogenic glaucoma removal of the lens by cryoextraction was necessary. Visual acuity increased to 0.5 and IOP was normal after three weeks. To improve safety of topical medication of lens-related glaucoma in Marian's syndrome regular measurement of IOP at different times of the day is especially important. Measurement of IOP at different positions seems to be an effective provocation test in cases of phacogenic glaucoma in Marian's syndrome. Examination of all members of a family with Marian's syndrome should include regular measurement of IOP at different times of the day. In Suspected cases, measurement of IOP at different positions that may provoce IOP-increase could be useful in the early detection of lens-related glaucoma.

Key words

Marian's syndrome - secondary glaucoma - provocation test

Schlüsselwörter

Marfan-Syndrom - Sekundärglaukom - Provokationstest