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S. R. Witherspoon, W. L. Dinges, K. M. Itani, D. M. Peterson; Ptosis and External Ophthalmoplegia Associated With Chronic Antiretroviral Therapy in Patients With HIV. Invest. Ophthalmol. Vis. Sci. 2007;48(13):5281.
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We present the clinical findings, treatment, and electron microscopy of 5 cases of myogenic ptosis in patients with HIV on long term antiretroviral therapy (ART).
Five patients with HIV were identified who presented with clinically significant myogenic ptosis. The median age of presentation was 50 years (range 46-53) and all patients were on antiretroviral therapy for a median of 7.8 years (range 4.9-11.2). No concurrent opportunistic infections were present in any case. All of the patients had HIV related lipodystrophy which was diagnosed a median of 4.7 years prior to the onset of ptosis. Four of the five underwent ptosis repair and tissue specimens were then prepared for electron microscopy to look for mitochondrial abnormalities.
At presentation themedian upper eyelid marginal reflex distance was 0.5mm (range 0-2mm). The levator function was decreased with a median of 10mm suggesting a myogenic process. Additionally, two of the five patients had an external ophthalmoplegia at the time of diagnosis and exotropia in primary position. No other ocular abnormalities were found. Evaluation was performed including myasthenia gravis antibody testing, tensilon testing, neuroimaging, and thyroid function studies which were unrevealing. On systemic evaluation, one of the patients was found to have heart block. Four of the five cases underwent surgical repair with levator resection. During surgery, it was noted that the levator aponeurosis needed to be advanced more than normally required in aponeurotic ptosis. One patient required re-operation for undercorrection. Biopsies were performed of the orbicularis muscle and subcutaneous adipose tissue. Electron microscopic analysis showed more numerous mitochondria than normal of the orbicularis muscle. The subcutaneous adipose sample showed marked abnormal mitochondrial morphology.
Chronic ART has been associated with the development of lipodystrophy, peripheral neuropathy, and peripheral skeletal muscle myopathy. The predominate theory implicates nucleoside reverse transcriptase inhibitor impairment of mitochondrial function. Ptosis and external ophthalmoplegia have not been previously associated with ART. These novel ophthalmic findings are consistent with a generalized mitochondrial disorder. The presence of heart block in one of the patients suggests the ophthalmologist may have an important role recognizing these potential adverse events.
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