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Paper text:
a 9-year - old male child presented with decreased vision in the left eye of 3 months duration .
visual acuity was 0.0 and 0.8 logmar in the right and left eye , respectively .
general examination revealed multiple cafe - au - lait skin spots over the back and arms [ fig . 1 ] .
fundus examination revealed bilateral mprap and chrrpe in the left eye [ figs . 2 and 3 ] .
suspecting nf-2 , neuroimaging was done ; magnetic resonance imaging revealed bilateral acoustic neuroma [ fig . 4 ] .
optical coherence tomography ( oct ) showed vitreoretinal traction in both eyes and disorganized inner retinal structures in the left eye [ fig . 5 ] .
fundus fluorescein angiography of mprap showed central hypofluorescence ( blocked fluorescence ) with surrounding hyperfluorescence ( staining ) [ fig .
systemic examination revealed multiple caf - au - lait spots over skin left eye fundus showed elevated , pigmented macular lesion with surface wrinkling and vascular tortuosity suggestive of combined hamartoma of retina and retinal epithelium .
both eye fundi showed multiple presumed retinal astrocytic proliferations ( arrows ) presumed retinal astrocytic proliferation ( circle ) in superonasal quadrant of right eye ( a ) , and closer view of combined hamartoma of retina and retinal epithelium in left eye ( b ) bilateral acoustic neuroma on magnetic resonance imaging ( arrows ) optical coherence tomography showed vitreoretinal traction in both eyes ( arrows ) and disorganized inner retinal structures in the left eye fundus fluorescein angiography showed central hypofluorescence ( blocked fluorescence ) with surrounding hyperfluorescence ( staining )
presumed solitary circumscribed retinal astrocytic proliferation is a recently described entity that occurs in middle - aged to older patients .
it appears as a small , solitary , well - defined opaque lesion confined to the retina .
our case exhibits the defined clinical characteristics but manifests with multiple lesions bilaterally in a 9-year - old boy , associated with chrrpe that we describe as mprap .
oct scan through the presumed retinal astrocytic proliferation in the right eye showed characteristics of retinal astrocytic hamartoma ( rah ) type ii , as described by pichi et al .
hence , it is possible to have multiple retinal astrocytic proliferations in a case of nf-2 .
the differential diagnosis of a well - circumscribed yellow - white lesion of the retina includes rah , acquired retinal astrocytoma , retinoblastoma , retinocytoma , myelinated retinal nerve fibers , granuloma , reactive gliosis , unifocal helioid choroiditis ( solitary idiopathic choroiditis ) , and solitary circumscribed retinal astrocytic proliferation .
rah is typically diagnosed early in life and often associated with tuberous sclerosis . in such cases , lesions are often multiple or bilateral .
our patient did not have any of the stigmata of tuberous sclerosis but presented with cafe - au - lait spots , chrrpe , and neuroimaging revealed bilateral acoustic neuroma - all suggestive of nf-2 .
retinocytomas appear as gray translucent tumors with intralesional calcification , surrounding retinal pigment epithelium alterations , and intralesional cysts .
our case had smaller lesions than what is typically seen with retinocytoma and did not have the characteristic features of that lesion .
unifocal helioid choroiditis ( solitary idiopathic choroiditis ) also presents with a yellow - white circumscribed lesion in the posterior pole .
our case differs because of its location within retina , rather than choroid . in summary
these lesions are well - circumscribed , opaque intraretinal lesions without exudation or feeding vessels .
these differ from other white or yellow - white lesions of the retina in important ways that enable the clinician to appropriately image the patient for possible features of nf-2 .
hence , recognition of this rare finding as presenting feature of nf-2 can lead to earlier diagnosis which is vital to appropriate surveillance and possible surgical intervention .
neurofibromatosis type 2 ( nf-2 ) is characterized by multifocal proliferation of neural crest - derived cells . 
 the characteristics finding of nf-2 is bilateral vestibular schwannomas . 
 combined hamartoma of retina and retinal epithelium ( chrrpe ) is another associated finding . 
 a 9 year - old - male child presented with left eye decreased vision for 3 months . 
 visual acuity was 0.0 and 0.8 logmar in the right and left eye , respectively . 
 left fundus showed an elevated , pigmented lesion with surface wrinkling and vascular tortuosity suggestive of chrrpe with multiple presumed retinal astrocytic proliferations in mid - periphery . 
 he had multiple caf - au - lait spots . 
 optical coherence tomography confirmed clinical findings . 
 magnetic resonance imaging brain showed bilateral acoustic neuroma . 
 recognition of this rare finding as presenting feature of nf-2 can lead to earlier diagnosis which is vital to appropriate surveillance and possible surgical intervention . 
 it is recommended that children with chrrpe be screened for nf-2 .