Asymmetric pigmentary glaucoma in a patient with Marfan's syndrome.
BACKGROUND
No relationship between Marfan's syndrome and pigmentary glaucoma has previously been described in the ophthalmic literature. We describe the case of a patient with Marfan's syndrome who had bilateral pigment dispersion syndrome and asymmetrical glaucoma.


METHODS
A 34-year-old man with Marfan's syndrome developed pigment dispersion bilaterally. In the right eye, elevated intra-ocular pressure was associated with marked glaucomatous excavation of the right disc and corresponding visual field loss. A localised zonular dehiscence was present at the 6 o'clock position in the right eye.


RESULTS
Ultrasound biomicroscopy showed marked iris concavity in the right eye, increased iridolenticular contact and superior subluxation of the right lens, resulting in increased dispersion of pigment unilaterally. This explains the asymmetric pattern of the glaucomatous damage.


CONCLUSIONS
Asymmetrical progression of pigmentary glaucoma is uncommon and should prompt a search for some other mechanical factor, which might increase the iridozonular contact on one side or reduce it on the other. The association between the two syndromes in this patient was most likely mechanical due to reduced fibrillin expression throughout the eye and particularly in the iris and the lens zonules, resulting in loss of iris tensile strength and marked iris concavity as well as zonular weakness and partial lens subluxation.
Doyle
Aoife
A
Institut du Glaucome, Fondation Hôpital St. Joseph, 185 rue Raymond Losserand, 75674 Paris, Cedex 14, France. aoife@fusio.net
Hamard
Pascale
P
Puech
Michel
M
Lachkar
Yves
Y
D000328:Adult; D000869:Anterior Eye Segment / Q000000981:diagnostic imaging; D017889:Exfoliation Syndrome / Q000000981:diagnostic imaging / Q000209:etiology*; D005902:Glaucoma, Open-Angle / Q000000981:diagnostic imaging / Q000209:etiology*; D006068:Gonioscopy; D006801:Humans; D007429:Intraocular Pressure; D007906:Lens Subluxation / Q000209:etiology*; D008022:Ligaments; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D033401:Microscopy, Acoustic; D009140:Musculoskeletal Diseases / Q000209:etiology*