A C T A O P H T H A L M O L O G I C A VOL. 5 6 1 9 7 8
Department
of
Ophthalmology (Head: N . Ehlers), Arhus Konamunehospital, University of Aarhus, Denmark
CENTRAL THICKNESS IN CORNEAL DISORDERS BY
NlELS EHLERS and THORKILD BRAMSEN
In the single individual the central corneal thickness (CCT) shows only small variations. Therefore CCT has been studied in a number of corneal diseases in order to investigate if this dimension might contribute to the diagnosis or to the understanding of the pathogenesis. Normal CCT was found in hereditary dystrophies with the exception of the macular dystrophy of Groenouw (type 11), which showed a significantly reduced thickness. Reduced CCT was found in chronic degenerations of leutic, tuberculous or indefinite nature. Marginal degenerations of Fuchs-Terrien type also showed reduced CCT. The possible role of abiotrophic processes in this corneal thinning is discussed. Endothelial dysfunction is indicated by increased CCT. This occurs in bullous keratopathy and in many acute disorders. Vascularisation of the cornea does not preclude the occurrence of normal or even reduced thickness.
Key words: corneal thickness stroma
-
-
corneal dystrophy
-
corneal degeneration
-
endothelium.
T h e maintenance of a normal and constant corneal thickness is important for stromal transparency, and a very limited variation of this dimension may be expected in the normal cornea. As regards the pathologically altered cornea, the thickness is known to be reduced in keratoconus, while a considerable increase may be evident in keratitis and in other acute conditions. In the various chronic disorders, we have found no data available. W e therefore report below the findings in a series of patients with chronic corneal diseases. The number of cases is limited, and the findings are more likely to show the possible value of pachometry, rather than document the significance of the differences. Received October 6 , 1977.
412
Thickness
of
Diseased Cornea
Material and Methods T h e material comprises 54 patients showing various chronic corneal disorders. T h e diagnoses will appear in the section on results. Central corneal thickness was determined by the Haag-Streit pachometer, modified as previously reported (Ehlers 1974; Ehlers & Sperling 1977). When a perpendicular incidence of the light beam is optically secured, identical values are found in right and left eyes, and the previously used reference value of 0.520 mm in right eye and 0.524 in the left had to be altered. I n a group of 45 conscripts a value of 0.5097 & 0.0041 for right eyes and 0.5100 & 0.0040 for left eyes (mean k SEM, N = 45) was found. T h e frequency distribution does not differ from the normal.
Results Hereditary dystrophies
Table I summarizes the observations made in patients with stromal dystrophies. T h e CCT was normal in granular dystrophy of Groenouw (type I), in lattice dystrophy (Haab-Dimmer), in crystalline dystrophy of Schnyder (the cases reported by Ehlers & Matthiessen 1973) and in central cloudy dystrophy of Fransois (the cases reported by Bramsen et al. 1976). Reduced thickness was found in macular dystrophy of Groenouw (type 11) (t-test, P < 0.001 when compared with the above mentioned value for young men). In dystrophies interfering with the endothelial barrier function (bullous keratopathy - Fuchs’ dystrophy) a pathologically increased thickness is an early
Table I . Central cornea1 thickness in hereditary dystrophies.
Type of dystrophy
I
~~~~~s
Granular (Groenouw I) Macular (Groenouw 11) Lattice (Haab-Dimmer) Crystalline (Schnyder) Central Cloudy (FranGois) Fuchs Schlichting
1
Average
6 4 3 3 3
0.519 0.409 0.505 0.517 0.536
20 1
increased 0.480
413
1
Range
0.495-0.560 0.380-0.435 0.480-0.525 0.510-0.520 0.520-0.545
Niels Ehlers and Thorkild Brainsen Table I I . Central corneal thickness in stromal degenerations.
:ration
Luetic Tuberculous (scrophulous) Indefinite post-keratitic Marginal (Fuchs-Terrien)
patients 3 6 2 3
Average
0.3i3 0.470 0.472
0.340-0.400 0.400-0.530 0.420-0.525
0.4%
0.430-0.4 7 5
sign. In these cases the CCT varies with the stage of the disease and no meaningful average can be given. T h e 20 cases referred to have been presented in another paper (Bramsen 8c Ehlers 1977). In one case of Schlichtings dystrophy low normal thickness was found, clearly distinguishing this condition from Fuchs’ dystrophy. Stromal degenerations
Table I1 summarizes the observations made in patients with stromal degenerations. I t was found that all cases of long lasting degeneration had a reduced CCT. In the first three groups a reduced thickness could be seen in avascular corneae, corneae with ghost vessels, and corneae with blood filled vessels. Reduced thickness in marginal degeneration is probably a premorbid condition. I t was noted in relatives of one of the patients. I n this condition, as in other degenerations, increased thickness may be found with endothelial dysfunction, or in late cases with large ulcerations. Normal thickness is found in cases of arcus senilis but we have made no direct comparison between matched groups with and without this lipid deposition.
Keratoconus
In keratoconus the stromal thickness is reduced. This is a well known fact which need not be confirmed by pachometry. However, the thickness reduction is an early sign occurring before conus formation is visible in the slitlamp. I n two cases of keratoconus posterior the CCT was normal outside the conus proper, illustrating the difference in pathogenesis of this condition compared to keratoconus.
414
Thickness of Diseased Cornea
Discussion T h e most common corneal dystrophy, the arcus senilis or lipoides is not associated with any evident change in thickness. Similarly, most of the dystrophies studied have had a normal CCT. The only evident exception has been the macular dystrophy, Groenouw’s type 11, of which all the observed cases showed severely reduced thickness. Therefore, pachometry would seem to be a means of differentiating between the two types of “classical” stromal dystrophy. It may be recalled that the deposited substance is different in type I and type 11, in the former a hyaline, slightly PAS positive material, in the latter a mucoid material stained by Alcian blue and colloid iron. Histopathologically, the depositions in lattice dystrophy resemble those of Groenouw’s type I which is in good accordance with the normal thickness in both conditions. Reduced thickness has been a constant finding in the chronic corneal degenerations of luetic, tuberculous and indefinite nature. The marginal degenerations were also found to have a reduced central thickness. The term corneal atrophy is appropriate, implicating some kind of abiotrophic process in the stroma. In this connection it is probably relevant to mention the minimum thickness obtained about 6 months after corneal grafting (Eblers 1974), which may also be related to a long term affection of the stromal metabclism. Previously the corneal thinning syndromes were discussed by Etzine (1969), who, under this term, considered pellucid marginal dystrophy with or without localized ectasia, keratoglobus and keratoconus. T h e pathogenesis of keratoconus is unknown and it would for the moment seem to be better to consider reduced corneal thickness after long-lasting degeneration as different from keratoconus. T h e normal or even reduced thickness in a vascularized cornea suggests that the vascular endothelium is impermeable to water. Fluorescein angiography might provide a clue to the understanding of thickness regulation in the vascularized cornea. As emphasized in the introduction, the limited number of cases studied does not permit definite conclusions to be drawn. T h e observations made must be ccnsidered as an illustration of the use of pachometry in the clinical study of corneal disorders. However, it provides sufficient basis for the statement that future reports on corneal disorders should include the measurement of the corneal thickness.
References Bramsen T., Ehlers N. & Baggesen L. H. (1976) Central cloudy corneal dystrophy of Fransois. A c t a ophthal. ( K b h . ) 54, 221-226.
415
Niels Ehlers and Thorkild Bramsen Bramsen T. & Ehlers N. (1977) Bullous keratopathy treated by systemic tranexamic acid. A c t a ophthal. (Kbh.) 55, 665-673. Ehlers N. (1974) Graft thickness after penetrating keratoplasty. Acta ophthal. (Kbh.) 52, 893-903. Ehlers N. & Matthiesen M. E. (1973) Hereditary crystalline corneal dystrophy of Schnyder. A c t a ophthal. (Kbh.) 51, 316-324. Ehlers N . & Sperling S. (1977) A technical improvement of the Haag-Streit pachometer. A c t a ophthal. (Kbh.) 5,5, 333-336. Etzine S. (1969) Corneal thinning syndrome: Keratoleptynsis. Ophthalmologica 152, 263-267.
Author’s address: Niels Ehlers, M. D., Department of Ophthalmology, Arhus Kommunehospital, DK-8000 Arhus C, Denmark.
416
Department
of
Ophthalmology (Head: N . Ehlers), Arhus Konamunehospital, University of Aarhus, Denmark
CENTRAL THICKNESS IN CORNEAL DISORDERS BY
NlELS EHLERS and THORKILD BRAMSEN
In the single individual the central corneal thickness (CCT) shows only small variations. Therefore CCT has been studied in a number of corneal diseases in order to investigate if this dimension might contribute to the diagnosis or to the understanding of the pathogenesis. Normal CCT was found in hereditary dystrophies with the exception of the macular dystrophy of Groenouw (type 11), which showed a significantly reduced thickness. Reduced CCT was found in chronic degenerations of leutic, tuberculous or indefinite nature. Marginal degenerations of Fuchs-Terrien type also showed reduced CCT. The possible role of abiotrophic processes in this corneal thinning is discussed. Endothelial dysfunction is indicated by increased CCT. This occurs in bullous keratopathy and in many acute disorders. Vascularisation of the cornea does not preclude the occurrence of normal or even reduced thickness.
Key words: corneal thickness stroma
-
-
corneal dystrophy
-
corneal degeneration
-
endothelium.
T h e maintenance of a normal and constant corneal thickness is important for stromal transparency, and a very limited variation of this dimension may be expected in the normal cornea. As regards the pathologically altered cornea, the thickness is known to be reduced in keratoconus, while a considerable increase may be evident in keratitis and in other acute conditions. In the various chronic disorders, we have found no data available. W e therefore report below the findings in a series of patients with chronic corneal diseases. The number of cases is limited, and the findings are more likely to show the possible value of pachometry, rather than document the significance of the differences. Received October 6 , 1977.
412
Thickness
of
Diseased Cornea
Material and Methods T h e material comprises 54 patients showing various chronic corneal disorders. T h e diagnoses will appear in the section on results. Central corneal thickness was determined by the Haag-Streit pachometer, modified as previously reported (Ehlers 1974; Ehlers & Sperling 1977). When a perpendicular incidence of the light beam is optically secured, identical values are found in right and left eyes, and the previously used reference value of 0.520 mm in right eye and 0.524 in the left had to be altered. I n a group of 45 conscripts a value of 0.5097 & 0.0041 for right eyes and 0.5100 & 0.0040 for left eyes (mean k SEM, N = 45) was found. T h e frequency distribution does not differ from the normal.
Results Hereditary dystrophies
Table I summarizes the observations made in patients with stromal dystrophies. T h e CCT was normal in granular dystrophy of Groenouw (type I), in lattice dystrophy (Haab-Dimmer), in crystalline dystrophy of Schnyder (the cases reported by Ehlers & Matthiessen 1973) and in central cloudy dystrophy of Fransois (the cases reported by Bramsen et al. 1976). Reduced thickness was found in macular dystrophy of Groenouw (type 11) (t-test, P < 0.001 when compared with the above mentioned value for young men). In dystrophies interfering with the endothelial barrier function (bullous keratopathy - Fuchs’ dystrophy) a pathologically increased thickness is an early
Table I . Central cornea1 thickness in hereditary dystrophies.
Type of dystrophy
I
~~~~~s
Granular (Groenouw I) Macular (Groenouw 11) Lattice (Haab-Dimmer) Crystalline (Schnyder) Central Cloudy (FranGois) Fuchs Schlichting
1
Average
6 4 3 3 3
0.519 0.409 0.505 0.517 0.536
20 1
increased 0.480
413
1
Range
0.495-0.560 0.380-0.435 0.480-0.525 0.510-0.520 0.520-0.545
Niels Ehlers and Thorkild Brainsen Table I I . Central corneal thickness in stromal degenerations.
:ration
Luetic Tuberculous (scrophulous) Indefinite post-keratitic Marginal (Fuchs-Terrien)
patients 3 6 2 3
Average
0.3i3 0.470 0.472
0.340-0.400 0.400-0.530 0.420-0.525
0.4%
0.430-0.4 7 5
sign. In these cases the CCT varies with the stage of the disease and no meaningful average can be given. T h e 20 cases referred to have been presented in another paper (Bramsen 8c Ehlers 1977). In one case of Schlichtings dystrophy low normal thickness was found, clearly distinguishing this condition from Fuchs’ dystrophy. Stromal degenerations
Table I1 summarizes the observations made in patients with stromal degenerations. I t was found that all cases of long lasting degeneration had a reduced CCT. In the first three groups a reduced thickness could be seen in avascular corneae, corneae with ghost vessels, and corneae with blood filled vessels. Reduced thickness in marginal degeneration is probably a premorbid condition. I t was noted in relatives of one of the patients. I n this condition, as in other degenerations, increased thickness may be found with endothelial dysfunction, or in late cases with large ulcerations. Normal thickness is found in cases of arcus senilis but we have made no direct comparison between matched groups with and without this lipid deposition.
Keratoconus
In keratoconus the stromal thickness is reduced. This is a well known fact which need not be confirmed by pachometry. However, the thickness reduction is an early sign occurring before conus formation is visible in the slitlamp. I n two cases of keratoconus posterior the CCT was normal outside the conus proper, illustrating the difference in pathogenesis of this condition compared to keratoconus.
414
Thickness of Diseased Cornea
Discussion T h e most common corneal dystrophy, the arcus senilis or lipoides is not associated with any evident change in thickness. Similarly, most of the dystrophies studied have had a normal CCT. The only evident exception has been the macular dystrophy, Groenouw’s type 11, of which all the observed cases showed severely reduced thickness. Therefore, pachometry would seem to be a means of differentiating between the two types of “classical” stromal dystrophy. It may be recalled that the deposited substance is different in type I and type 11, in the former a hyaline, slightly PAS positive material, in the latter a mucoid material stained by Alcian blue and colloid iron. Histopathologically, the depositions in lattice dystrophy resemble those of Groenouw’s type I which is in good accordance with the normal thickness in both conditions. Reduced thickness has been a constant finding in the chronic corneal degenerations of luetic, tuberculous and indefinite nature. The marginal degenerations were also found to have a reduced central thickness. The term corneal atrophy is appropriate, implicating some kind of abiotrophic process in the stroma. In this connection it is probably relevant to mention the minimum thickness obtained about 6 months after corneal grafting (Eblers 1974), which may also be related to a long term affection of the stromal metabclism. Previously the corneal thinning syndromes were discussed by Etzine (1969), who, under this term, considered pellucid marginal dystrophy with or without localized ectasia, keratoglobus and keratoconus. T h e pathogenesis of keratoconus is unknown and it would for the moment seem to be better to consider reduced corneal thickness after long-lasting degeneration as different from keratoconus. T h e normal or even reduced thickness in a vascularized cornea suggests that the vascular endothelium is impermeable to water. Fluorescein angiography might provide a clue to the understanding of thickness regulation in the vascularized cornea. As emphasized in the introduction, the limited number of cases studied does not permit definite conclusions to be drawn. T h e observations made must be ccnsidered as an illustration of the use of pachometry in the clinical study of corneal disorders. However, it provides sufficient basis for the statement that future reports on corneal disorders should include the measurement of the corneal thickness.
References Bramsen T., Ehlers N. & Baggesen L. H. (1976) Central cloudy corneal dystrophy of Fransois. A c t a ophthal. ( K b h . ) 54, 221-226.
415
Niels Ehlers and Thorkild Bramsen Bramsen T. & Ehlers N. (1977) Bullous keratopathy treated by systemic tranexamic acid. A c t a ophthal. (Kbh.) 55, 665-673. Ehlers N. (1974) Graft thickness after penetrating keratoplasty. Acta ophthal. (Kbh.) 52, 893-903. Ehlers N. & Matthiesen M. E. (1973) Hereditary crystalline corneal dystrophy of Schnyder. A c t a ophthal. (Kbh.) 51, 316-324. Ehlers N . & Sperling S. (1977) A technical improvement of the Haag-Streit pachometer. A c t a ophthal. (Kbh.) 5,5, 333-336. Etzine S. (1969) Corneal thinning syndrome: Keratoleptynsis. Ophthalmologica 152, 263-267.
Author’s address: Niels Ehlers, M. D., Department of Ophthalmology, Arhus Kommunehospital, DK-8000 Arhus C, Denmark.
416
