A literature review of the recent discoveries and potential treatments for retinitis pigmentosa, Leber's congenital amaurosis, X-linked retinoschisis, Best's disease, Stargardt's disease, and congenital stationary night blindness is presented, along with a guide for clinicians seeking genetic testing of patients.

We report a case of bilateral central retinal artery occlusion associated with sickle cell disease and moyamoya syndrome. In a review of the literature, the relationship of sickle cell disease to moyamoya syndrome and of moyamoya syndrome to central retinal artery occlusion is discussed. The utility of magnetic resonance imaging in this case is highlighted.

<H4>BACKGROUND AND OBJECTIVES</H4> <P>To determine whether irrigation of the sub-Tenon’s space with anesthetic agents during pars plana vitrectomy (PPV) involving general anesthesia decreases postoperative pain, analgesic use, or nausea.</P> <H4>PATIENTS AND METHODS</H4> <P>A prospective, controlled trial of 46 consecutive patients requesting general anesthesia for PPV who were randomized to receive or not receive a sub-Tenon’s space injection prior to surgery. A mixture of 3 mL of 2% lidocaine with hyaluronidase and 3 mL of 0.5% bupivacaine was used to induce local blockade. Pain, postoperative nausea, and analgesia use were evaluated.</P> <H4>RESULTS</H4> <P>Local blockade did not significantly alter the proportion of reported pain at 30 minutes and 2, 4, and 24 hours after the operation. The local blockade had no effect on reducing postoperative nausea or the number of patients requiring pain medication.</P> <H4>CONCLUSIONS</H4> <P>Local blockade prior to surgery in patients undergoing PPV under general anesthesia does not significantly decrease postoperative pain, analgesic use, or nausea.</P> <P>[<CITE>Ophthalmic Surg Lasers Imaging</CITE> 2007;38:203-208.]</P> <H4>AUTHORS</H4> <P>From the Department of Ophthalmology (JOM, PLG, RMF), University of Alabama at Birmingham School of Medicine, Callahan Eye Foundation Hospital; and Retina Consultants of Alabama, P.C. (JOM, RMF, RSV), Birmingham, Alabama.</P> <P>Accepted for publication March 8, 2007.</P> <P>Supported by an unrestricted departmental grant from Research to Prevent Blindness, Inc., New York, New York.</P> <P>Address correspondence to John O. Mason, III, MD, Retina Consultants of Alabama, P. C., Callahan Eye Foundation Hospital, 700 South 18th Street, Suite 707, Birmingham, AL 35233.</P>

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