UNILATERAL ARCUS LIPOIDES CORNEAE DUE TO STURGE-WEBERS SYNDROME
I. M. Velten, J. Hayler
Background: Single case reports of an unilateral hemiarcus lipoides in patients with unilateral absolute or relative hypotony exist in the literature. We report of a patient with unilateral arcus lipoides corneae combined with contralateral congenital glaucoma due to Sturge-Webers syndrome.
Patient and Methods: A 33-year-old man presented to us with unilateral juvenile Glaucoma due to Sturge-Webers syndrome. The glaucoma with a maximum intraocular pressure (IOP) of 40 mmHg had been diagnosed at the age of one year. During the first two years of life, a diathermal cyclopuncture had been performed twice, additionally a trabeculectomy at the age of six years. Since that, the IOP has ranged between 16 and 20 mmHg in the left eye. constantly being lower in the right eye. A hemangioma concerning the first and second branch of the facial nerve had been treated by radiotherapy after birth. There were no neurological deficits or symptoms. On examination, visual acuity was OD 30/30 and OS 20/30. The IOP was measured OD 16 mmHg and OS 20 mmHg. A static perimetry revealed an unremarkable result of the right eye, and diffuse visual field losses of the left eye (mean defect 8.7 dB). Except for mild intermittent hypercholosterolemia up to 260 mg/dl, the patients medical history was unremarkable. Slit-lamp examination showed a marked white-yellow arcus lipoides corneae of the right eye which was missing in the left eye. Except that, slit-lamp examination, gonioscopy, and funduscopy did not reveal anything remarkable in the right eye. The left eye showed marked episcleral vascularisation, the iris was darker on the left than on the right eye. The chamber angle was open circularly with many uveal residuals. The optic disc presented with moderate glaucomatous changes, the rest of the funduscopy was unremarkable.
Conclusions: This patient with unilateral hemiarcus lipoides in the eye with relative hypotony compared to the other eye with juvenile glaucoma suggests, according to earlier case reports, that unilateral lipid deposition is not only favoured by absolute hypotony with marked vascular congestion and increased vascular permeability, but also in cases of relative hypotony with normal morphological findings.
Supported by "Deutsche Forschungsgemeinschaft, Sonderforschungsbereich Glaukome (SFB 539) ".
Augenklinik mit Polinik der Universität Erlangen-Nürnberg, Schwabachanlage 6, D-91054 Erlangen