BACKGROUND: Chandler's Syndrome is a unilateral disease that typically affects women in their middle ages and is a variant of the iridocorneal endothelial (ICE) syndromes. The spectrum of ICE syndromes includes essential (progressive) iris atrophy, Chandler's syndrome and Cogan Reese (iris nevus syndrome). Clinically, Chandler's syndrome is characterized by mild iris thinning, pupillary distortion and marked corneal changes which may lead to secondary angle closure glaucoma and chronic corneal edema. Unfortunately, little is understood about the etiology of Chandler's Syndrome, although links to the Epstein-Barr virus (EBV) and/or a viral etiology have been proposed. This case report reveals the diagnosis of Chandler's Syndrome in a patient with history of Guillain-Barre Syndrome (GBS).
CASE REPORT(S): A 56 year old Caucasian female reported episodic blurred vision of the right eye, noting clouding upon awakening and again at the end of the day. She reported a diagnosis of GBS eight years prior with primarily neurologic manifestations. Upon examination, vision was best corrected to 20/25 in the right and 20/20 in the left eye. Slit lamp examination revealed mild corneal edema in the right eye and deep anterior chambers bilaterally. IOP readings were 32 mmHg in the right and 14 mmHg in the left by applanation. Gonioscopy showed scattered areas of scleral spur with broad based peripheral anterior synechia in the 4-5 oclock position with areas of peaked PAS at 1, 9:30 and 11 oclock. Pachymetry was measured at 596 microns in the right and 544 in the left eye. Dilated fundus ophthalmoscopy showed ratios of 0.1 bilaterally.
CONCLUSIONS: The category of ICE syndromes was first suggested in 1979 by Eagle and Yanoff. There have been several different etiologies proposed for the ICE syndromes, including a link to EBV, and most recently a link to the herpes simplex virus. As well, a strong, positive association has been made between infections with EBV and 20 fold increased GBS risk. This case supports the hypothesis of a potential link between ICE syndromes and EBV.