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Paper text:
a man in his early thirties presented with complaints of gradual diminution of vision in his right eye noted over a span of 34 years .
the patient did not have any history of recurrent redness , itching or pain in eyes or any known history of trauma .
he had been diagnosed as bilateral kc ( with greater involvement of right eye ) elsewhere and was referred for a second opinion . on presentation , his uncorrected visual acuity was 20/400 od and 20/60 os .
the right eye was being corrected to 20/60 with high myopic ( 12 diopter ) and low cylinder correction ( 2 diopter ) .
slit lamp examination showed steep cornea both eyes with a faint apical corneal scar in the right eye .
fleisher 's ring , prominent corneal nerves and vogt 's striae was present in both eyes , being more prominent in right eye .
right eye showed variable anterior chamber depth with iris nodules , atrophic areas on iris , ectropion uveae , and peripheral anterior synechiae .
pupil in right eye was approximately 5 mm with peaking at 10 o clock position .
left eye had normal anterior chamber depth with circular pupil , normally reacting to light .
( a ) diffuse illumination of right eye ; ( b ) magnified view showing areas of iris atrophy and iris nodules ( red arrow ) ; ( c and d ) cobalt blue filter illumination of right eye ( c ) and left eye ( d ) showing fleisher 's ring ( white arrow ) ; ( e ) right eye gonioscopy of temporal angle showing broad peripheral anterior synechiae at 1011 oclock position ( blue arrow ) with smaller early adjacent synechiae ( red arrows ) ; ( f ) gonioscopy of temporal - superior zone showing temporal synechiae ( blue arrow ) and pigmented superior angle intraocular pressure by goldmann applanation tonometer was 28 mmhg in the right eye and 8 mmhg in left eye .
2 ] showed a cup to disc ratio of approximately 0.4 in right eye with early glaucomatous changes suggested by inferior notching and inferior vessel bending ( bayoneting sign ) .
left eye showed a healthy neuroretinal rim with a cup to disc ratio of approximately 0.3 .
visual fields were unreliable but showed few scotomas in right eye and grossly normal left eye .
( a ) right eye showing early notching in inferior neuroretinal rim and bayoneting of vessel ( white arrow ) ; ( b ) left eye showing healthy neuroretinal rim .
( c ) right eye magnified specular microscopy shows pleomorphism , polymegathism , light - dark reversal and a low cell density of 986 cells / mm ; ( d ) normal polygonal endothelial cells of left eye with cell density of 2414 cells / mm specular examination ( topcon sp-2000p , topcon , tokyo , japan ) of right eye revealed poor endothelial morphology with pleomorphism , polymegathism , and probable ice cells .
corneal topography ( orbscan iiz , bausch and lomb , rochester , new york , usa ) established the diagnosis of kc both eyes with the right eye being more affected [ fig .
inferior - superior difference in both eyes in the central 15 mm zone was more than 1.2 diopter .
pachymetry map shows reduced corneal thickness in both eyes with the central corneal thickness being 0.356 mm in right eye and 0.443 mm in left eye . corneal topography .
maximum simulated keratometric value was 62.6 diopter in right eye and 57.3 diopter in the left eye .
posterior elevation was 0.159 mm in right eye and 0.128 mm in left eye the patient was advised rigid contact lens for both eyes . in view of high myopia with a low cylinder refractive correction in right eye , surgical modalities in the form of keratoplasty was deferred . keeping the high intraocular pressure , thin cornea and young age in mind
; he was started on beta blocker ( timolol maleate ) , and prostaglandin analog ( latanoprost ) eye drops for the right eye . on the patient 's successive visits , intraocular pressure of right eye
was lowered ( 16 mmhg on last visit ) and the patient was comfortable with his visual rehabilitation .
the patient has been advised a close monitoring to assess for kc and glaucoma progression .
we document a case of kc with cogan - reese syndrome and secondary glaucoma . in the past ,
blair et al . have reported the apparent bilateral association of essential iris atrophy and kc with coincident features of posterior polymorphous dystrophy ( ppd ) .
they have also proposed a hypothesis similar to the genetics of retinoblastoma for the pathogenesis of the ice syndrome with associated kc and/or ppd .
the study of cremona et al . presented a 10 year data of associations of kc and corneal dystrophies .
although chandler 's syndrome has been postulated to be associated with kc , there are limited published reports .
kc has been reported to be associated with ppd , iris heterochromia , and band keratopathy .
we consider our case as unique since there are no published reports of kc with cogan - reese syndrome .
our case appeared more like ice rather than ppd because of its unilaterality , nonfamilial nature , specific corneal morphology , presence of synechiae and glaucoma . because of the presence of iris nodules and pigmented iris lesions , we diagnosed it to be cogan - reese syndrome . although kc has multiple reported associations , our case appears to be the first reported case of a unique association .
if ophthalmologists are aware of the entities linked with kc , the management can be rendered better by timely diagnosis .
many studies have focused on the association of ppd claiming a common embryological origin together with simultaneous chromosomal alterations .
eiferman et al . reported a case of iridoschisis and kc in the same eye and suggested a related pathogenesis since the posterior layers of the cornea , and the iris stroma have a common embryological origin .
still , there is much to be learnt about the pathophysiology of kc and its related disease complexes .
chromosomal analysis would have given more insight to this case ; which could not be done and account as a limitation of our study .
future work may focus on the possible pathophysiologic or genetic links between these entities , although a chance association can not be excluded from this study .
this report documents , what we believe to be a new association of keratoconus- coganreese syndrome .
keratoconus ( kc ) , though one of the most common corneal degeneration , still continues to be a mystique regarding its pathogenesis , diagnosis , associations , and management ; with newer discoveries and evolutions being reported . 
 we report , what we believe to be another new association of kc- cogan reese syndrome with secondary glaucoma . 
 a 32-year - old male , diagnosed as bilateral kc , presented for examination . 
 unilateral cogan - reese syndrome and associated secondary glaucoma was identified . 
 these associations had been missed by previous ophthalmologists . the patient was managed with a rigid contact lens for kc and topical antiglaucoma agents for glaucoma . 
 he was advised regular reviews and is under observation till date . 
 we describe the first case known to us of a new association with kc . 
 this case not only highlights the ophthalmologist 's need to look for multiple entities linked to kc ; but may also pave way for future insights regarding pathogenesis and genetics of these associated diseases .