Glaucoma in the Maroteaux-Lamy syndrome

We report four patients who had glaucoma in association with the Maroteaux-Lamy syndrome. Two of these patients had acute angle-closure glaucoma, one had chronic angle-closure glaucoma, and one was treated for open-angle glaucoma; however, the angle structures could not be seen by gonioscopy. Standard peripheral iridectomy was inadequate for treatment of this angle-closure glaucoma because of the considerably increased thickness of the peripheral cornea that occurs in these patients. Our findings suggest that glaucoma may be more common in the Maroteaux-Lamy syndrome than has been realized and that the initial mechanism is secondary acute or chronic angle closure not related to pupillary block.