Adams-Oliver syndrome associated with bilateral anterior polar cataracts and optic disk drusen

Lascaratos, Gerassimos; Lam, Wayne; Newman, William; MacRae, Mary

doi:10.1016/j.jaapos.2011.02.012

Cited by 5 publications

(3 citation statements)

References 10 publications

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“…10 Notably, our patient also had bilateral optic disc drusen, which has been previously reported in AOS. 6 We suspect that the disc drusen may be responsible for her abnormal visual field, which otherwise cannot be explained solely by the presence of peripheral retinal ischemia. A limitation in documenting this rare condition is that AOS is a clinical diagnosis.…”

Section: Discussionmentioning

confidence: 91%

“…1,2 Because early mortality in AOS is common when it is associated with internal organ involvement and/or ophthalmologic manifestations, its retinal associations have not been well-characterized. 3,5,6 The ability to perform clinic-based multimodal fundus imaging in this 13-year-old patient made it possible to comprehensively document some of the retinal manifestations of AOS.…”

Section: Discussionmentioning

confidence: 99%

“…2 The literature regarding diagnosis, treatment, and management of ocular findings in Adams-Oliver syndrome remains limited because of the high mortality rate associated with the condition in patients with internal organ and/or ophthalmic involvement. [4][5][6][7] Here, we describe comprehensive ophthalmic findings in a 13-year-old AOS patient and discuss our management approach for the associated proliferative retinopathy.…”

mentioning

confidence: 99%

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Proliferative Retinopathy in a 13-Year-Old With Adams–oliver Syndrome

Meyer

Williams

Hanif

et al. 2022

RETINAL Cases &Amp; Brief Reports

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Adams-Oliver syndrome is a rare, inherited disorder of embryologic development that affects multiple systems. Ocular manifestations have been poorly characterized because of the low prevalence and high mortality of the disease when it is associated with internal organ and/or ophthalmic manifestations. We present a case of Adams-Oliver syndrome in a 13-year-old patient whose multimodal retinal imaging findings helped direct management.Methods: Single patient case report reviewing medical records and imaging.Results: Visual acuity upon presentation was 20/40 in each eye. Ultra-widefield fluorescein angiography revealed peripheral nonperfusion with terminal vascular bulbs, and leakage from a temporal fibrovascular complex in the left eye. Fundus autofluorescence imaging showed hyperautofluorescence associated with optic disc drusen and the fibrovascular complex. Treatment with targeted laser photocoagulation was associated with regression of the neovascularization.Conclusion: Retinal manifestations of Adams-Oliver syndrome as observed with ultrawidefield fundus imaging may resemble those of familial exudative vitreoretinopathy and retinopathy of prematurity. Treatment of avascular retina with panretinal photocoagulation can be considered.

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Section: Discussionmentioning

confidence: 91%