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Ophthalmic Images |

Ultra–Wide-Field Autofluorescence in Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency Chorioretinopathy Online Only

Yoshihiro Yonekawa, MD1; Benjamin J. Thomas, MD1; Antonio Capone Jr, MD1
[+] Author Affiliations
1Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan
JAMA Ophthalmol. 2016;134(4):e155033. doi:10.1001/jamaophthalmol.2015.5033.
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This Ophthalmic Image shows ultra–wide-field autofluorescence findings in a patient with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency chorioretinopathy.

Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is an autosomal recessive disorder of mitochondrial fatty acid beta oxidation. Patients with LCHAD deficiency demonstrate early-onset cardiomyopathy, hypoglycemia, hepatic steatosis, rhabdomyolysis, and peripheral neuropathy.1 Ocular manifestations include a pigmentary retinopathy that progresses to chorioretinal atrophy.2 Fundus autofluorescence is largely unexplored in LCHAD deficiency, but we have been using the noninvasive imaging modality of ultra–wide-field autofluorescence as a method of monitoring progression in these patients. The images (Figure) are from a girl in her teens with LCHAD deficiency controlled with a low-fat, high-carbohydrate diet. Best-corrected visual acuity is 20/60 OU. The ophthalmic findings are bilateral and symmetric. There is a peninsula of residual choroidal vasculature centrally, which is permitting her central acuity.

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Ultra–wide-field autofluorescence imaging. A, Ultra–wide-field photograph demonstrates bare sclera centrally and centripetal chorioretinal atrophy. B, Autofluorescence imaging reveals scleral autofluorescence centrally with surrounding patchy hypoautofluorescence (devitalizing retinal pigment epithelium with pigment clumping) and hyperautofluorescence (bare sclera underlying loss of retinal pigment epithelium and choriocapillaris).

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