Ocular Manifestations of Alkaptonuric Ochronosis

On the ocular findings in ochronosis: a systematic review of literature

“…Conjunctival changes were also frequent (22 cases) but more heterogeneous in appearance [18,21-23,25-27,75-82,86-90]. There are four main types of appearance which may also occur in combination: 1) Small “vermiform” [88] or “tube-like” [76] pigment deposits (Figure 2, middle, with permission [88]) [18,27,76,81,86,88], 2) brown pingecula-like [81,87,89], 3) dot like [72,77] or 4) laminar structures [22]. Dilatated conjunctival vessels can be present and seemingly supply the pigmented areas [25,70,76,85,87,88].…”

“…Severe progressive astigmatism has been reported twice [21,22]. It was attributed by the authors to ochronotic accretions that were integrated from the sclera into the limbus and may have lead to corneoscleral thinning.…”

“…Both patients were in their 70ies when astigmatism began to develop and in both cases the axis of the cylinder was around 0° thus oriented in a right angle to the pigment. Astigmatism progressed by 6 dpt within two years in one case and by 7 dpt within 11 years in the other [21,22]. Ehongo et al attempted a corneal transplant and bilateral thread placement.…”

On the ocular findings in ochronosis: a systematic review of literature

“…Conjunctival changes were also frequent (22 cases) but more heterogeneous in appearance [18,21-23,25-27,75-82,86-90]. There are four main types of appearance which may also occur in combination: 1) Small “vermiform” [88] or “tube-like” [76] pigment deposits (Figure 2, middle, with permission [88]) [18,27,76,81,86,88], 2) brown pingecula-like [81,87,89], 3) dot like [72,77] or 4) laminar structures [22]. Dilatated conjunctival vessels can be present and seemingly supply the pigmented areas [25,70,76,85,87,88].…”

“…Severe progressive astigmatism has been reported twice [21,22]. It was attributed by the authors to ochronotic accretions that were integrated from the sclera into the limbus and may have lead to corneoscleral thinning.…”

“…Both patients were in their 70ies when astigmatism began to develop and in both cases the axis of the cylinder was around 0° thus oriented in a right angle to the pigment. Astigmatism progressed by 6 dpt within two years in one case and by 7 dpt within 11 years in the other [21,22]. Ehongo et al attempted a corneal transplant and bilateral thread placement.…”

Descemet’s membrane folds in ochronosis: a case report

“…This case describes the oldest patient with ocular ochronotic findings to be reported in international scientific literature [ 4 ]. Progressive, marked “against-the-rule” astigmatism has been reported twice in elderly patients [ 5 , 6 ]. Both patients were in their seventies when astigmatism began to develop, in both cases being oriented along the same axis as the pigment was found.…”

“…However, the presence of Descemet’s membrane folds has never been described. Cheskes et al believed that progressive astigmatism was due to corneoscleral thinning after scleral pigment accumulation [ 6 ].…”

On the ocular findings in ochronosis: a systematic review of literature

“…Conjunctival changes were also frequent (22 cases) but more heterogeneous in appearance [18,21-23,25-27,75-82,86-90]. There are four main types of appearance which may also occur in combination: 1) Small “vermiform” [88] or “tube-like” [76] pigment deposits (Figure 2, middle, with permission [88]) [18,27,76,81,86,88], 2) brown pingecula-like [81,87,89], 3) dot like [72,77] or 4) laminar structures [22]. Dilatated conjunctival vessels can be present and seemingly supply the pigmented areas [25,70,76,85,87,88].…”

“…Severe progressive astigmatism has been reported twice [21,22]. It was attributed by the authors to ochronotic accretions that were integrated from the sclera into the limbus and may have lead to corneoscleral thinning.…”

“…Both patients were in their 70ies when astigmatism began to develop and in both cases the axis of the cylinder was around 0° thus oriented in a right angle to the pigment. Astigmatism progressed by 6 dpt within two years in one case and by 7 dpt within 11 years in the other [21,22]. Ehongo et al attempted a corneal transplant and bilateral thread placement.…”

Descemet’s membrane folds in ochronosis: a case report

“…This case describes the oldest patient with ocular ochronotic findings to be reported in international scientific literature [ 4 ]. Progressive, marked “against-the-rule” astigmatism has been reported twice in elderly patients [ 5 , 6 ]. Both patients were in their seventies when astigmatism began to develop, in both cases being oriented along the same axis as the pigment was found.…”

“…However, the presence of Descemet’s membrane folds has never been described. Cheskes et al believed that progressive astigmatism was due to corneoscleral thinning after scleral pigment accumulation [ 6 ].…”

On the ocular findings in ochronosis: a systematic review of literature

“…Conjunctival changes were also frequent (22 cases) but more heterogeneous in appearance [18,21-23,25-27,75-82,86-90]. There are four main types of appearance which may also occur in combination: 1) Small “vermiform” [88] or “tube-like” [76] pigment deposits (Figure 2, middle, with permission [88]) [18,27,76,81,86,88], 2) brown pingecula-like [81,87,89], 3) dot like [72,77] or 4) laminar structures [22]. Dilatated conjunctival vessels can be present and seemingly supply the pigmented areas [25,70,76,85,87,88].…”

“…Severe progressive astigmatism has been reported twice [21,22]. It was attributed by the authors to ochronotic accretions that were integrated from the sclera into the limbus and may have lead to corneoscleral thinning.…”

“…Both patients were in their 70ies when astigmatism began to develop and in both cases the axis of the cylinder was around 0° thus oriented in a right angle to the pigment. Astigmatism progressed by 6 dpt within two years in one case and by 7 dpt within 11 years in the other [21,22]. Ehongo et al attempted a corneal transplant and bilateral thread placement.…”

Descemet’s membrane folds in ochronosis: a case report

“…This case describes the oldest patient with ocular ochronotic findings to be reported in international scientific literature [ 4 ]. Progressive, marked “against-the-rule” astigmatism has been reported twice in elderly patients [ 5 , 6 ]. Both patients were in their seventies when astigmatism began to develop, in both cases being oriented along the same axis as the pigment was found.…”

“…However, the presence of Descemet’s membrane folds has never been described. Cheskes et al believed that progressive astigmatism was due to corneoscleral thinning after scleral pigment accumulation [ 6 ].…”