This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Kent W. Small, MD; Robert Letson, MD; Jon Scheinman, MD

Arch Ophthalmol. 1990;108(1):89-93.

Abstract
• Primary hyperoxaluria (primary oxalosis) is a rare autosomal recessive inborn error of glyoxylate metabolism that causes widespread calcium oxalate crystal deposition in diverse tissues. Because others have reported only occasional ocular involvement, we reviewed the ophthalmologic findings in our 24 patients with primary hyperoxaluria to document its funduscopic variability and to determine its visual prognosis and its possible systemic significance. Eight (30%) of our 24 patients with primary hyperoxaluria exhibited a bilaterally symmetrical retinopathy. The abnormalities were predominantly confined to the posterior pole and ranged from many small (100- to 200-µm) subretinal black ringlets to single large (2- to 3-disc diameter) geographic lesions. In 3 of the 8 patients with oxalate retinopathy, diffuse optic disc pallor was evident. Five patients with both normal-appearing optic discs and oxalate retinopathy had relatively good visual acuities. The maculopathy of primary hyperoxaluria caused mild visual impairment while optic nerve dysfunction associated with this disease appeared to be much more visually debilitating. Also, the presence of oxalate maculopathy was associated with a more severe systemic course for the disease.

Author Affiliations
From the Department of Neurology (Dr Small) and the Division of Pediatric Nephrology (Dr Scheinman), Duke University, Durham, NC; and the Department of Ophthalmology (Dr Letson), University of Minnesota, Minneapolis.

Footnotes
Accepted for publication August 14, 1989.

Presented as a poster at the Association for Research in Vision and Ophthalmology meeting, Sarasota, Fla, May 1,1989.

Reprint requests to Box 2900, Duke University Medical Center, Durham, NC 27710 (Dr Small).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

West African Crystalline Maculopathy
Sarraf et al.
Arch Ophthalmol 2003;121:338-342.
ABSTRACT | FULL TEXT  

Ophthalmic features of primary oxalosis after combined liver/kidney transplantation
ALCORN and FREDRICK
Br J Ophthalmol 2000;84:1318i-1318.
FULL TEXT  

Crystalline Retinopathy Associated With Chronic Retinal Detachment
Ahmed et al.
Arch Ophthalmol 1998;116:1449-1453.
ABSTRACT | FULL TEXT  

Enzyme Deficiency for Type 1 Primary Hyperoxaluria-Reply
Sakamoto and Inomata
Arch Ophthalmol 1992;110:13-13.
ABSTRACT  

Ocular Histopathologic Findings in a 46-Year-Old Man With Primary Hyperoxaluria
Sakamoto et al.
Arch Ophthalmol 1991;109:384-387.
ABSTRACT