eight - year - old male presented with 360 peripheral corneal endotheliopathy and edema , granulomatous keratic precipitates , and mild iritis od .
twenty months later , os presented similarly and anterior chamber inflammatory cells , vitreous snowballs , and retinal vasculitis were observed ou .
this is the first case of endotheliopathy as the first manifestation of pars planitis and as a cause of a secondary central cornea ectasia developed
 white eye  , mild anterior chamber reaction ,  snowballs  in the anterior vitreous ,  snowbanks  in the peripheral retina and pars plana , retinal vasculitis , and cystoid macular edema .
the association of a peripheral corneal endotheliopathy ( pce ) and pars planitis starting in childhood has been previously described .
we report a cpp case in which pce was the first clinical manifestation , and which led to secondary corneal ectasia .
on the first visit , an 8-year - old male presented because the patient s mother noticed a white reflex in the right eye for about 2  years that became more evident with time .
his visual acuity od was 20/200 with improvement to 20/60 with pinhole occlusion , os 20/20 .
od showed mild ciliary injection , mild peripheral corneal edema , a 360 peripheral endothelial opacity delimitated by  mashed potato  keratic precipitates , and mild iritis ( fig .  1 ) .
vitreous examination revealed few cells and a single  snowball  in the inferior , peripheral retina , near an area of apparent retinal vasculitis .
granulomatous kps delineated the involved endothelial area pictures showing the right eye with a peripheral corneal stromal oedema and endothelial opacity .
granulomatous kps delineated the involved endothelial area a diagnosis of pars planitis was suspected and fluorescein angiography ( fa ) , ppd , vdrl , and fta - abs were ordered .
prednisolone acetate ( prednefrin , allergan ) q2h and timolol 0.5% ( imot , laboratorios sophia , guadalajara , mxico ) bid were started ; the steroid was slowly tapered over a period of 1  month .
all laboratory tests were either negative or normal and fluorescein angiography ou was also normal .
examination showed va : od 20/400 with improvement 20/80 with pinhole occlusion , os 20/20 ; iop : 10/14  mmhg , with pronounced peripheral corneal stromal oedema and endothelial opacity od .
granulomatous keratic precipitates ( kps ) delineated the involved endothelial area , and some deep stromal peripheral vessels were found .
javal s keratometric readings were od 40.00/46.25    10 and os 44.00/45.00    5 , he showed decreased corneal sensitivity , and there was no anterior chamber or vitreous inflammation .
both the stromal and endothelial opacities diminished and so the corticosteroids were tapered during the next 2  months .
six months later , a corneal topography ( orbscan topography system ii , orbscan inc . ,
2 shows the findings ) od shows a with the rule astigmatism with asymmetric bowtie pattern .
central pachymetry was similar in both eyes ( od 619  m , os 617  m ) but the peripheral pachymetry in the od was 900  m thicker than the os in the superior area and 60  m in the inferior and temporal area . in the left eye ( fig . 
specular microscopy ( topcon sp-2000p noncontact specular microscope , topcon corp . , tokyo , japan ) of the right eye showed decreased endothelial cell density ( 1,636 cell / mm ) with pleomorphism and polymegatism ; the left eye was normal ( 3,141 cell / mm ) . 

anterior elevation map within normal values ; posterior elevations map shows a central steepening of 96  mm ; and pachymetry map with increase of peripheral thickness .
anterior elevation map within normal values ; posterior elevations map shows a central steepening of 96  mm ; and pachymetry map with increase of peripheral thickness .
the keratometric map shows a asymmetrical bowtie pattern with an inferior steepest left eye orbscan topography map .
the keratometric map shows a symmetrical bowtie three years after the initial visit , an examination showed a decreased va ou , being od 20/400 ; os 20/30 .
his iop was normal and his od showed corneal findings very similar to those previously described , in the left eye a peripheral endothelial opacity in the inferior sector of the cornea was observed .
mild to moderate iritis and vitreous cells+ were found ou .  snowballs  in the inferior peripheral retina and some vascular sheathing were also seen . in both eyes , fa showed hyperfluorescence of the peripheral capillary vessels , parietal hyperfluorescence of the medium - sized veins , and optic disc and perifoveal hyperfluorescence ou . a complete blood cell count , urinalysis , angiotensin - converting enzyme , and chest x - ray were carried out .
an autoimmune endotheliopathy ( ae ) associated with a corneal graft rejection was first described by khodadust and attarzadeh .
this condition is characterized by a peripheral endothelial opacity and stromal edema , initially located in the lower quadrants , which may progress in a circumferential pattern .
small and mutton fat kps arranged linearly at the junction of the oedematous and non - oedematous portions of the cornea may also be present .
later , the same authors , as well as lucchese and tessler , described similar findings in cases of pars planitis   and sarcoidosis patients .
autoimmune responses to the corneal endothelium , and a fibrous metaplasia of the endothelium secondary to damage caused by kp , have been suggested to explain these findings .
ae has been described in 18.8% to 25.4% of cpp patients and manifests bilaterally in 70% of cases .
we have hypothesized that the peripheral swelling of the cornea may induce a modification of the central curvature .
these changes can be explained using the conceptual model proposed by roberts , in which the lamellar collagen structure of the cornea is conceived of as a series of stacked fibrils with interlamellar spaces filled with an extracellular matrix between each layer .
the collagen lamellas are in tension , due to intraocular pressure from below , and their ends are held tightly by the limbus . the amount of water at the extracellular matrix is related to the strain force at the lamellae .
the biomechanical response of the cornea in the present case could be the consequence of differences in the amount of swelling throughout the cornea and alterations in the stress distribution .
the peripheral corneal edema may function as a belt that exerts its force centripetally , allowing the central dome of the cornea to be displaced anteriorly , thus increasing its curvature .
a change in the shape of the cornea has been demonstrated to occur in the swollen eye but not in the normal hydration state . using small - angle x - ray diffraction , boote et al
.   demonstrated that in physiologic conditions collagen fibrils pack around the peri - pupillary cornea ( the interfibrillar space is 5% to 7% smaller than in the peripheral cornea ) . in the present case , this may have played a role in limiting swelling into the central cornea , explaining why the corneal edema was limited to the periphery .
this report is interesting because , to the best of our knowledge , this is the first report of a case in which corneal endotheliopathy is the first manifestation of pars planitis and induced the development of a secondary central cornea ectasia .
 purposeto report a case of corneal ectasia secondary to pars planitis corneal endotheliopathymethodsclinical case description and proposed hypothesis regarding development of corneal ectasiaresultseight - year - old male presented with 360 peripheral corneal endotheliopathy and edema , granulomatous keratic precipitates , and mild iritis od . 
 a progressive corneal ectasia then developed . 
 twenty months later , os presented similarly and anterior chamber inflammatory cells , vitreous snowballs , and retinal vasculitis were observed ou . 
 classic pars planitis was diagnosedconclusionthis is the first case of endotheliopathy as the first manifestation of pars planitis and as a cause of a secondary central cornea ectasia developed 
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