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FIGURE 31.4 Rhegmatogenous retinal detachment (RRD) in an OI type I patient from University of Illinois at Chicago, Illinois Eye and Ear
Infirmary. This patient had bilateral RRDs. The left eye is shown here with blue sclera (upper left). The RRD was detected on B-scan ultraso-
nography (upper middle), involved the inferior retina on fundus photography (upper right), and was also detected by spectral domain optical
coherence tomography (bottom).
Central Retinal Vein Occlusion
Central retinal vein occlusion (CRVO) is a blockage
of the main draining vein of the retina as it exits through
the optic nerve. Risk factors include hypertension, dia-
betes, hypercoagulable states and glaucoma. Eyes with
CRVO may demonstrate extensive intraretinal hem-
orrhages, edema in the macula, neovascularization in
the retina or iris and even neovascular glaucoma from
abnormal vascular growth blocking the trabecular mesh-
work that leads to high eye pressures and optic nerve
damage. Eyes with CRVO require close evaluation and
follow-up from an ophthalmologist for treatment of isch-
emic complications (macular edema, neovascularization)
with laser or anti-vascular endothelial growth factor
(anti-VEGF) therapy. Smolinska reported on a case of OI
and bilateral glaucoma and CRVO in one eye. 45
detachment in one eye. The other sibling initially had
attached retinas with normal vasculature but later
developed bilateral retinal detachments: a complete
open funnel retinal detachment in the right eye and an
inferior tractional retinal detachment with an abnormal
fibrotic stalk from the optic nerve to the inferotemporal
retina in the left eye.
Optic Nerve and Neuro-Ophthalmologic
Findings in OI
Optic neuropathies - diseases of the optic nerve -
and other neuro-ophthalmologic problems have also
occurred in OI patients. As the optic nerve exits the
orbit through the lesser wing of the sphenoid bone,
traumatic fractures of this bony region may cause trau-
matic optic neuropathies and associated visual loss.
Even with treatment, sometimes involving high dose
steroids, traumatic optic neuropathies often have
poor outcomes. Traumatic optic neuropathies and
other neuro-ophthalmologic problems have also been
reported in conjunction with OI and other conditions.
Arnould reported ear and neuro-ophthalmologic find-
ings in OI congenita in 1957. 59 Gogolkiewicz reported
on optic nerve injury in a patient with OI tarda in 1974. 60
Other Retinopathies
Al Gazali also reported on two siblings of consan-
guineous parents with a retinopathy associated with
OI, optic atrophy and developmental delay. 58 These
patients had clinical features overlapping with osteo-
porosis pseudoglioma syndrome. One sibling had eyes
with abnormal tortuous and branching retinal vessels
and later was discovered to have a tractional retinal
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