Angle abnormalities, heterochromia iridis may play role in glaucoma development in Sturge-Weber syndrome

J Pediatr Opthalmol Strabismus. 2010;47(6):361-365.

Anterior chamber angle abnormalities and heterochromic iridis may be linked to the development of glaucoma in infants with Sturge-Weber syndrome, according to a study.

"The characteristics observed in the anterior chamber angle in this large, uniquely examined series are the first reported to the authors' knowledge and may support a role for neural crest derived anomalies in the pathogenesis of glaucoma in these infant eyes," the study authors said.

Researchers conducted a retrospective review of 55 patients with Sturge-Weber syndrome who presented between January 1978 and December 2003; 44 of these patients had glaucoma.

Unilateral glaucoma was diagnosed in 30 eyes, and bilateral glaucoma was diagnosed in 14 eyes; thus, 58 eyes had glaucoma and 52 eyes did not have glaucoma.

According to the study, 32 of the glaucomatous eyes had distinct anatomic abnormalities in the anterior chamber angle, which was in contrast to the normal fellow eyes in patients with unilateral glaucoma and to the patients without glaucoma.

In addition, heterochromia iridis with a darker iris in the glaucomatous eye was present in 11 of the 30 patients with unilateral glaucoma (37%), but in no patients without glaucoma.

The authors noted the study was limited because there were more patients with glaucoma than without glaucoma. In addition, the sample population was drawn from one referral practice, and eyes were most often examined, diagnosed and treated by one physician.

"Future study is certainly warranted to elucidate the mechanisms by which heterochromic irides may be related to elevated intraocular pressure and to the other angle anomalies described here," they said.