Abnormal Scleral Collagen in Nanophthalmos

Macular Retinal Detachment in Hallermann-Streiff Syndrome

“…Although pathological confirmation is lacking in this case, a previous autopsy report of a patient with mucolipidosis type I demonstrated diffuse intracytoplasmic accumulation of lipofuscinlike pigment in the cerebral neurons. 5 This leads us to speculate that the ophthalmo-scopic changes observed in our patient are due to an accumulation of sialyloligosaccharides in the inner layers of the retina.…”

Macular Retinal Detachment in Hallermann-Streiff Syndrome

“…Although pathological confirmation is lacking in this case, a previous autopsy report of a patient with mucolipidosis type I demonstrated diffuse intracytoplasmic accumulation of lipofuscinlike pigment in the cerebral neurons. 5 This leads us to speculate that the ophthalmo-scopic changes observed in our patient are due to an accumulation of sialyloligosaccharides in the inner layers of the retina.…”

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“…Histopathological studies showed disorganization of collagen fiber bundles and variation in size of collagen fibrils. These findings were consistent with previous studies (7)(8)(9)(10)(11)(12)(13)17). Because of the histopathological abnormalities in the rigid and thickened sclera, fluid drainage was also compromised from the decreased function of uveoscleral outflow pathways, leading to excessive protein and fluid accumulation in the suprachoroidal space.…”

A Modified Ultrasound-Guided Surgical Technique for the Management of the Uveal Effusion Syndrome in Patients With Normal Axial Length and Scleral Thickness

“…The end result is accumulation of fluid in the choroid and subretinal space resulting in choroidal swelling and serous retinal detachment. [1][2][3][4][10][11][12][13][14] Although these findings were initially reported in nanophthalmic eyes, 3,10,11,14 similar findings were observed in non-nanophthalmic eyes with UES. 1,2,4,12,13 The hypothesis of reduced scleral hydraulic conductivity has been recently questioned, 15 and recent studies 15,16 showed that "reduced macromolecular diffusion may impede the normal transscleral egress of albumin with subsequent osmotic fluid retention" in some eyes with UES.…”

Macular Retinal Detachment in Hallermann-Streiff Syndrome

“…Although pathological confirmation is lacking in this case, a previous autopsy report of a patient with mucolipidosis type I demonstrated diffuse intracytoplasmic accumulation of lipofuscinlike pigment in the cerebral neurons. 5 This leads us to speculate that the ophthalmo-scopic changes observed in our patient are due to an accumulation of sialyloligosaccharides in the inner layers of the retina.…”

Image_1.PNG

“…Histopathological studies showed disorganization of collagen fiber bundles and variation in size of collagen fibrils. These findings were consistent with previous studies (7)(8)(9)(10)(11)(12)(13)17). Because of the histopathological abnormalities in the rigid and thickened sclera, fluid drainage was also compromised from the decreased function of uveoscleral outflow pathways, leading to excessive protein and fluid accumulation in the suprachoroidal space.…”

A Modified Ultrasound-Guided Surgical Technique for the Management of the Uveal Effusion Syndrome in Patients With Normal Axial Length and Scleral Thickness

“…The end result is accumulation of fluid in the choroid and subretinal space resulting in choroidal swelling and serous retinal detachment. [1][2][3][4][10][11][12][13][14] Although these findings were initially reported in nanophthalmic eyes, 3,10,11,14 similar findings were observed in non-nanophthalmic eyes with UES. 1,2,4,12,13 The hypothesis of reduced scleral hydraulic conductivity has been recently questioned, 15 and recent studies 15,16 showed that "reduced macromolecular diffusion may impede the normal transscleral egress of albumin with subsequent osmotic fluid retention" in some eyes with UES.…”

Macular Retinal Detachment in Hallermann-Streiff Syndrome

“…Although pathological confirmation is lacking in this case, a previous autopsy report of a patient with mucolipidosis type I demonstrated diffuse intracytoplasmic accumulation of lipofuscinlike pigment in the cerebral neurons. 5 This leads us to speculate that the ophthalmo-scopic changes observed in our patient are due to an accumulation of sialyloligosaccharides in the inner layers of the retina.…”

Image_1.PNG

“…Histopathological studies showed disorganization of collagen fiber bundles and variation in size of collagen fibrils. These findings were consistent with previous studies (7)(8)(9)(10)(11)(12)(13)17). Because of the histopathological abnormalities in the rigid and thickened sclera, fluid drainage was also compromised from the decreased function of uveoscleral outflow pathways, leading to excessive protein and fluid accumulation in the suprachoroidal space.…”

A Modified Ultrasound-Guided Surgical Technique for the Management of the Uveal Effusion Syndrome in Patients With Normal Axial Length and Scleral Thickness

“…The end result is accumulation of fluid in the choroid and subretinal space resulting in choroidal swelling and serous retinal detachment. [1][2][3][4][10][11][12][13][14] Although these findings were initially reported in nanophthalmic eyes, 3,10,11,14 similar findings were observed in non-nanophthalmic eyes with UES. 1,2,4,12,13 The hypothesis of reduced scleral hydraulic conductivity has been recently questioned, 15 and recent studies 15,16 showed that "reduced macromolecular diffusion may impede the normal transscleral egress of albumin with subsequent osmotic fluid retention" in some eyes with UES.…”