POSTERIOR AMORPHOUS CORNEAL
DYSTROPHY
E M M E T T F . CARPEL, M . D . , R O B E R T J . SIGELMAN, M . D . , AND D O N A L D J . DOUGHMAN, M . D .
Minneapolis, P r e - D e s c e m e t ' s dystrophy, posterior polymorphous dystrophy, and c o n g e n i t a l hereditary corneal dystrophy are disorders of the deep layers of the c o r n e a that are classified a c c o r d i n g to the predominant layer or affected structure. T h e preD e s c e m e t ' s dystrophies have b e e n subdivided on the basis of their p l e o m o r p h i c appearance. T h e protean manifestations o f posterior polymorphous dystrophy and congenital hereditary corneal dystrophy have b e e n also d e s c r i b e d . 1 - 3
4 - 8
9 - 1 2
Several m e m b e r s o f three generations of a family had irregular sheet-like areas of opacification of the deep layers o f the corneal stroma with involvement o f D e s cemet's m e m b r a n e , and in s o m e instances alterations of the normal endothelial mosaic. T h e r e was no e v i d e n c e o f stromal edema, but a uniform t h i n n i n g was present. T h e corneal changes do not c o n f o r m to any o f these dystrophies, and appear to constitute a dystrophy o f t h e posterior cornea that, to our knowledge, has not been previously d e s c r i b e d . CASE REPORTS
This family (Fig. 1) was identified during a biomicroscopic examination of the propositus (II-3). Aside from the propositus, all family members were asymptomatic and were examined at the slit lamp. Corneal thickness was measured with a corneal pachymeter (Michima-Hedbys) mounted on a Haag-Streit slit lamp (Model 900). Three measurements of central corneal thickness were made and the measurements were averaged. Case 1-1—The 75-year-old father of the propositus had bilateral aphakia with best corrected visual acuity of R . E . : 6/6 (20/20), and L . E . : 6/9 (20/30). Slit-lamp examination revealed the appearance of a generalized mild corneal thinning and an irregular
From the Department of Ophthalmology, University of Minnesota, Minneapolis, Minnesota. Reprint requests to Emmett F . Carpel, M.D., 2500 Como Ave., St. Paul, MN 55108.
Minnesota amorphous deep stromal sheet-like opacification. Scattered areas of superficial stromal haziness in the upper one third of the cornea were also present. The deep changes were irregular in depth and clear areas within the sheet-like opacification were present. The depth of the opacification extended to and involved Descemet's membrane with alterations of the endothelial mosaic. Vitreous had prolapsed into the anterior chamber but there was no corneal touch. No corneal vascularization was present. Case 1-2—The 70-year-old mother of the propositus had best corrected visual acuity of R.E.: 6/7.5 (20/25), and L.E.: 6/24 (20/80) with a cataract in the left eye. The corneas were normal aside from moderate fine cornea gutatta centrally. Case II-1—The 35-year-old brother of the propositus had visual acuity of 6/6 (20/20) bilaterally. He had similar but slightly milder corneal changes than the propositus. He gave a history of an injury to his left eye and he had a paracentral small subepithelial scar. Case II-2—A 31-year-old sister of the propositus had best corrected visual acuity of R.E.: 6/12 (20/ 40), and L . E . : 6/6 (20/20). Slit-lamp examination showed the same findings as in Cases 1-1 and II-3 except for less pronounced corneal opacification in both eyes. The pachymetric readings were R.E.: 0.26 mm, and L . E . : 0.25 mm. The other findings were normal. Case II-3—The propositus is a 30-year-old woman with best corrected visual acuity of R.E.: 6/9 (20/30), and L.E.: 6/7.5 (20/25). Biomicroscopic examination revealed a generalized uniform thinning with a widespread amorphous sheet-like area of opacification of the posterior corneal stroma and Descemet's membrane (Fig. 2). No flecks or vesicular structures were noted, but alterations of the endothelial cell mosaic were seen. Keratometry showed no distortion of the mires, and corneal sensation was normal. There was no evidence of active vascularization, nor were ghost vessels seen. Central corneal thickness measured with the corneal pachymeter was R.E,: 0.30 mm, and L.E.: 0.34 mm. The remainder of the ocular examination was normal. The patient's auditory and vestibular functions were normal, and results of the VDRL and FTA-ABS tests were negative. There was no clinical history compatible with previous episodes of interstitial keratitis. Case III-l—A 14-year-old nephew (son of II-l) had visual acuity of 6/6 (20/20) bilaterally. He had the mildest form of corneal involvement, similar in appearance to that in Case III-4. Case III-2—A 12-year-old niece (daughter of II-l) had visual acuity of 6/6 (20/20) in each eye. The slit-lamp examination was normal except for a prominent ring of Schwalbe at the periphery of the corneas.
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MAY, 1977
I II in 18 mos.
Fig. 1 (Carpel, Sigelman, and Doughman). Pedigree of family with posterior amorphous corneal dystrophy.
• _ / - ) Consanguineous marriage (Second
cousins)
•
•
M
® Minimally Affected
•
O Unaffected ¿
Affected
Propositus
Case III-3—A 9-year-old nephew (son of II-l) had best corrected visual acuity of R.E.: 6/6 (20/20), and L.E.: 6/7.5 (20/25). Slit-lamp examination disclosed the same corneal changes as the more involved family members (Cases 1-1, II-l, II-2, and II-3). Generalized thinning was present but not as marked. The deep sheet-like opacification was present centrally, unlike the milder changes manifested by his brother (Case III-l) and his first cousin (Case III-4), who only had peripheral corneal involvement. The opacification was deep in the stroma centrally and varied in thickness. Breaks in the opacification with interposed normal stroma were present. In addition, his right eye had corectopia, pseudopolycoria, and a large focal midiris adherence to the posterior cornea. There was no iris atrophy.
Fig. 2 (Carpel, Sigelman, and Doughman). II-3. Drawing of slit-lamp appearance with the full manifestations of posterior amorphous dystrophy. Note the uniform corneal thinning and the diffuse, irregular opacification involving posterior stroma and Descemet's membrane, with interposed clear areas.
Case III-4—A 5-year-old niece (daughter of II-2) had visual acuity of 6/6 (20/20) in each eye with the Snellen E test. Slit-lamp examination showed that the central corneal areas were normal. However, at the periphery, there was a homogeneous, amorphous, deep stromal sheet-like opacification with a thin area of clear stroma posterior to it (Fig. 3). The homogeneous density, the lack of breaks in the sheet-like areas of opacification, and the constant depth of the opacification also contrasted with the more marked involvement of the above family members. The pachymeter measurements were R.E.: 0.55 mm, and L.E.: 0.51 mm. Case III-5—An 18-month-old nephew (son of II-2) had vision that fixed and followed with each eye. Slit-lamp examination was normal.
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AMORPHOUS CORNEAL DYSTROPHY
631
sions a c i r c u m s c r i b e d stromal edema or thickening is often found. In our cases, the opacities exhibit a more confluent and amorphous appearance, as well as an absence o f stromal e d e m a or discrete vesicular structures. Affected individuals in our series could not b e placed in any o f Grays o n ' s classifications o f deep polymorphous dystrophy except for one ( I I I - 3 ) who has some of the features o f a more widespread mesodermal abnormality. 8
O t h e r dystrophies o f the posterior cornea considered in the differential diagnosis were central cloudy d y s t r o p h y and congenital hereditary corneal dystrophy. Central cloudy corneal dystrophy is characterized by grayish, irregular, snowflake opacities with indefinite structures and indistinct margins that occur in the central one third o f the cornea, with normal corneal t h i c k n e s s . T h e y b e c o m e more dense near D e s c e m e t ' s membrane, may occupy all areas of the stroma but do not involve B o w m a n ' s or D e s c e m e t ' s membranes, and spare the corneal periphery. Vision is unaffected and the condition is nonprogressive. I n the dystrophy in the family we studied, D e s c e m e t ' s membrane and endothelium were involved, and the opacities were widespread without predilection for the central cornea. T h e r e was no evidence o f discrete snowflake opacification, but large amorphous sheet-like areas o f opacification were present. Congenital hereditary corneal dystrophy of the cornea most often occurs as bilateral congenital corneal clouding with a significant decrease in visual a c u i t y . The slit-lamp findings reveal a loss of the normal endothelial mosaic, an uneven posterior surface of the cornea, and a grayish white haze or opacification of D e s c e m e t ' s m e m b r a n e , with overlying stromal thickening or e d e m a . However, a broader spectrum o f involvement includes asymptomatic family members with minimal corneal changes consisting 13
9
Fig. 3 (Carpel, Sigelman, and Doughman). III-4. Drawing of the slit-lamp view shows the minimal type of corneal involvement. Note normal corneal thickness, only peripheral deep stromal opacification of constant depth, and the rim of clear cornea posteriorly.
DISCUSSION
Grayson and W i l b r a n d t classified the opacities anterior to D e s c e m e t ' s membrane into three types, based on their distinct morphologic appearances. Other disorders c o m m o n to the pre-Descemet's location are deep filiform dystrophy, deep punctate dystrophy, parenchymatous dystrophy, and polymorphic stromal dystrophy. ' T h e s e disorders c o m m o n l y share the pre-Descemet's location, little or no effect on visual acuity, and the fact that the opacities themselves, w h i l e varying in size, shape, or density, have a discrete and distinct outline. In our cases, D e s c e m e t ' s m e m b r a n e was involved, the opacities were amorphous and sheet-like in appearance, and the corneas in the more advanced cases were thin. Posterior polymorphous dystrophy involves D e s c e m e t ' s m e m b r a n e and the deepest layers of the stroma with polymorphous discrete opacities, depressions, and v e s i c l e s . Surrounding these le1
2
3
2 , 6 , 7
13
9 , 1 0 , 1 2
1 2 , 1 4
632
AMERICAN JOURNAL O F OPHTHALMOLOGY
of whitish lesions and clear spots at the level of Descemet's membrane that appear similar to those o f posterior polymorphous dystrophy. In these cases, the posterior stroma is not involved, whereas in our cases involvement occurred at all deep layers o f the cornea. In congenital hereditary corneal dystrophy one expects to find the initial involvement centrally with peripheral progression, but the youngest affected individual in our series had early peripheral involvement with none noted centrally. T h e corneal disorder appears only mildly progressive since the youngest severely affected individual ( I I I - 3 ) and the eldest (1-1) had no significant visual impairment. However, the cornea may undergo a uniform thinning in time, an apparently unique finding in posterior corneal dystrophies. Although there is evidence o f consanguinity, an autosomaldominant mode o f inheritance seems most likely.
MAY, 1977
the three-generation pedigree indicated an autosomal-dominant inheritance pattern.
11
SUMMARY
A family o f ten individuals aged 18 months to 7 5 years had biomicroscopic findings consisting o f large, amorphous, sheet-like opacifications of the posterior stroma and Descemet's membrane, and alterations o f the endothelium. A uniform thinning of the cornea was present. T h e s e findings do not conform to previously described corneal dystrophies. T h e condition appears minimally progressive and
REFERENCES
1. Grayson, M., and Wilbrandt, H.: Pre-Descemet dystrophy. Am. J. Ophthalmol. 64:276, 1967. 2. Duke-Elder, S., and Leigh, A. G.: Diseases of the Outer Eye. Cornea and Sclera. In Duke-Elder, S. (ed.): System of Ophthalmology, vol. 8, pt. 2. London, Henry Kimpton, 1965, pp. 947, 950, 951. 3. Thomsitt, J . , and Bron, A. J . : Polymorphic stromal dystrophy. Br. J. Ophthalmol. 59:125,1975. 4: Klouman, O. F.: A peculiar type of corneal endothelial dystrophy. Acta Ophthalmol. 43:534, 1965. 5. Theodore, F . H.: Congenital type of endothelial dystrophy. Arch. Ophthalmol. 21:626, 1939. 6. McGee, H. B., and Falls, H. F.: Hereditary polymorphous deep degeneration of the cornea. Arch. Ophthalmol. 50:462, 1953. 7. Hogan, M. J., and Bietti, G.: Hereditary deep dystrophy of the cornea (polymorphous). Am. J . Ophthalmol. 68:777, 1969. 8. Grayson, M.: The nature of hereditary deep polymorphous dystrophy of the cornea: Its association with iris and anterior chamber dysgenesis. Trans. Am. Ophthalmol. Soc. 72:516, 1974. 9. Maumenee, A. E.: Congenital hereditary corneal dystrophy. Am. J. Ophthalmol. 50:1114, 1960. 10. Malbran, E . S.: Corneal dystrophies: A clinical, pathological, and surgical approach. Am. J . Ophthalmol. 74:771, 1972. 11. Levenson, J . E . , Chandler, J. W., and Kaufman, H. E.: Affected asymptomatic relatives in congenital hereditary endothelial dystrophy. Am. J . Ophthalmol. 76:967, 1973. 12. Antine, B.: Histology of congenital hereditary corneal dystrophy. Am. J. Ophthalmol. 69:964, 1970. 13. François, J.: Heredity in Ophthalmology. St. Louis, C. V. Mosby, 1961, p. 312. 14. Pearce, W. G., Tripathi, R. C , and Morgan, G.: Congenital endothelial corneal dystrophy. Clinical, pathological, and genetic study. Br. J. Ophthalmol. 53:577, 1969.
DYSTROPHY
E M M E T T F . CARPEL, M . D . , R O B E R T J . SIGELMAN, M . D . , AND D O N A L D J . DOUGHMAN, M . D .
Minneapolis, P r e - D e s c e m e t ' s dystrophy, posterior polymorphous dystrophy, and c o n g e n i t a l hereditary corneal dystrophy are disorders of the deep layers of the c o r n e a that are classified a c c o r d i n g to the predominant layer or affected structure. T h e preD e s c e m e t ' s dystrophies have b e e n subdivided on the basis of their p l e o m o r p h i c appearance. T h e protean manifestations o f posterior polymorphous dystrophy and congenital hereditary corneal dystrophy have b e e n also d e s c r i b e d . 1 - 3
4 - 8
9 - 1 2
Several m e m b e r s o f three generations of a family had irregular sheet-like areas of opacification of the deep layers o f the corneal stroma with involvement o f D e s cemet's m e m b r a n e , and in s o m e instances alterations of the normal endothelial mosaic. T h e r e was no e v i d e n c e o f stromal edema, but a uniform t h i n n i n g was present. T h e corneal changes do not c o n f o r m to any o f these dystrophies, and appear to constitute a dystrophy o f t h e posterior cornea that, to our knowledge, has not been previously d e s c r i b e d . CASE REPORTS
This family (Fig. 1) was identified during a biomicroscopic examination of the propositus (II-3). Aside from the propositus, all family members were asymptomatic and were examined at the slit lamp. Corneal thickness was measured with a corneal pachymeter (Michima-Hedbys) mounted on a Haag-Streit slit lamp (Model 900). Three measurements of central corneal thickness were made and the measurements were averaged. Case 1-1—The 75-year-old father of the propositus had bilateral aphakia with best corrected visual acuity of R . E . : 6/6 (20/20), and L . E . : 6/9 (20/30). Slit-lamp examination revealed the appearance of a generalized mild corneal thinning and an irregular
From the Department of Ophthalmology, University of Minnesota, Minneapolis, Minnesota. Reprint requests to Emmett F . Carpel, M.D., 2500 Como Ave., St. Paul, MN 55108.
Minnesota amorphous deep stromal sheet-like opacification. Scattered areas of superficial stromal haziness in the upper one third of the cornea were also present. The deep changes were irregular in depth and clear areas within the sheet-like opacification were present. The depth of the opacification extended to and involved Descemet's membrane with alterations of the endothelial mosaic. Vitreous had prolapsed into the anterior chamber but there was no corneal touch. No corneal vascularization was present. Case 1-2—The 70-year-old mother of the propositus had best corrected visual acuity of R.E.: 6/7.5 (20/25), and L.E.: 6/24 (20/80) with a cataract in the left eye. The corneas were normal aside from moderate fine cornea gutatta centrally. Case II-1—The 35-year-old brother of the propositus had visual acuity of 6/6 (20/20) bilaterally. He had similar but slightly milder corneal changes than the propositus. He gave a history of an injury to his left eye and he had a paracentral small subepithelial scar. Case II-2—A 31-year-old sister of the propositus had best corrected visual acuity of R.E.: 6/12 (20/ 40), and L . E . : 6/6 (20/20). Slit-lamp examination showed the same findings as in Cases 1-1 and II-3 except for less pronounced corneal opacification in both eyes. The pachymetric readings were R.E.: 0.26 mm, and L . E . : 0.25 mm. The other findings were normal. Case II-3—The propositus is a 30-year-old woman with best corrected visual acuity of R.E.: 6/9 (20/30), and L.E.: 6/7.5 (20/25). Biomicroscopic examination revealed a generalized uniform thinning with a widespread amorphous sheet-like area of opacification of the posterior corneal stroma and Descemet's membrane (Fig. 2). No flecks or vesicular structures were noted, but alterations of the endothelial cell mosaic were seen. Keratometry showed no distortion of the mires, and corneal sensation was normal. There was no evidence of active vascularization, nor were ghost vessels seen. Central corneal thickness measured with the corneal pachymeter was R.E,: 0.30 mm, and L.E.: 0.34 mm. The remainder of the ocular examination was normal. The patient's auditory and vestibular functions were normal, and results of the VDRL and FTA-ABS tests were negative. There was no clinical history compatible with previous episodes of interstitial keratitis. Case III-l—A 14-year-old nephew (son of II-l) had visual acuity of 6/6 (20/20) bilaterally. He had the mildest form of corneal involvement, similar in appearance to that in Case III-4. Case III-2—A 12-year-old niece (daughter of II-l) had visual acuity of 6/6 (20/20) in each eye. The slit-lamp examination was normal except for a prominent ring of Schwalbe at the periphery of the corneas.
629
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AMERICAN JOURNAL O F OPHTHALMOLOGY
MAY, 1977
I II in 18 mos.
Fig. 1 (Carpel, Sigelman, and Doughman). Pedigree of family with posterior amorphous corneal dystrophy.
• _ / - ) Consanguineous marriage (Second
cousins)
•
•
M
® Minimally Affected
•
O Unaffected ¿
Affected
Propositus
Case III-3—A 9-year-old nephew (son of II-l) had best corrected visual acuity of R.E.: 6/6 (20/20), and L.E.: 6/7.5 (20/25). Slit-lamp examination disclosed the same corneal changes as the more involved family members (Cases 1-1, II-l, II-2, and II-3). Generalized thinning was present but not as marked. The deep sheet-like opacification was present centrally, unlike the milder changes manifested by his brother (Case III-l) and his first cousin (Case III-4), who only had peripheral corneal involvement. The opacification was deep in the stroma centrally and varied in thickness. Breaks in the opacification with interposed normal stroma were present. In addition, his right eye had corectopia, pseudopolycoria, and a large focal midiris adherence to the posterior cornea. There was no iris atrophy.
Fig. 2 (Carpel, Sigelman, and Doughman). II-3. Drawing of slit-lamp appearance with the full manifestations of posterior amorphous dystrophy. Note the uniform corneal thinning and the diffuse, irregular opacification involving posterior stroma and Descemet's membrane, with interposed clear areas.
Case III-4—A 5-year-old niece (daughter of II-2) had visual acuity of 6/6 (20/20) in each eye with the Snellen E test. Slit-lamp examination showed that the central corneal areas were normal. However, at the periphery, there was a homogeneous, amorphous, deep stromal sheet-like opacification with a thin area of clear stroma posterior to it (Fig. 3). The homogeneous density, the lack of breaks in the sheet-like areas of opacification, and the constant depth of the opacification also contrasted with the more marked involvement of the above family members. The pachymeter measurements were R.E.: 0.55 mm, and L.E.: 0.51 mm. Case III-5—An 18-month-old nephew (son of II-2) had vision that fixed and followed with each eye. Slit-lamp examination was normal.
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AMORPHOUS CORNEAL DYSTROPHY
631
sions a c i r c u m s c r i b e d stromal edema or thickening is often found. In our cases, the opacities exhibit a more confluent and amorphous appearance, as well as an absence o f stromal e d e m a or discrete vesicular structures. Affected individuals in our series could not b e placed in any o f Grays o n ' s classifications o f deep polymorphous dystrophy except for one ( I I I - 3 ) who has some of the features o f a more widespread mesodermal abnormality. 8
O t h e r dystrophies o f the posterior cornea considered in the differential diagnosis were central cloudy d y s t r o p h y and congenital hereditary corneal dystrophy. Central cloudy corneal dystrophy is characterized by grayish, irregular, snowflake opacities with indefinite structures and indistinct margins that occur in the central one third o f the cornea, with normal corneal t h i c k n e s s . T h e y b e c o m e more dense near D e s c e m e t ' s membrane, may occupy all areas of the stroma but do not involve B o w m a n ' s or D e s c e m e t ' s membranes, and spare the corneal periphery. Vision is unaffected and the condition is nonprogressive. I n the dystrophy in the family we studied, D e s c e m e t ' s membrane and endothelium were involved, and the opacities were widespread without predilection for the central cornea. T h e r e was no evidence o f discrete snowflake opacification, but large amorphous sheet-like areas o f opacification were present. Congenital hereditary corneal dystrophy of the cornea most often occurs as bilateral congenital corneal clouding with a significant decrease in visual a c u i t y . The slit-lamp findings reveal a loss of the normal endothelial mosaic, an uneven posterior surface of the cornea, and a grayish white haze or opacification of D e s c e m e t ' s m e m b r a n e , with overlying stromal thickening or e d e m a . However, a broader spectrum o f involvement includes asymptomatic family members with minimal corneal changes consisting 13
9
Fig. 3 (Carpel, Sigelman, and Doughman). III-4. Drawing of the slit-lamp view shows the minimal type of corneal involvement. Note normal corneal thickness, only peripheral deep stromal opacification of constant depth, and the rim of clear cornea posteriorly.
DISCUSSION
Grayson and W i l b r a n d t classified the opacities anterior to D e s c e m e t ' s membrane into three types, based on their distinct morphologic appearances. Other disorders c o m m o n to the pre-Descemet's location are deep filiform dystrophy, deep punctate dystrophy, parenchymatous dystrophy, and polymorphic stromal dystrophy. ' T h e s e disorders c o m m o n l y share the pre-Descemet's location, little or no effect on visual acuity, and the fact that the opacities themselves, w h i l e varying in size, shape, or density, have a discrete and distinct outline. In our cases, D e s c e m e t ' s m e m b r a n e was involved, the opacities were amorphous and sheet-like in appearance, and the corneas in the more advanced cases were thin. Posterior polymorphous dystrophy involves D e s c e m e t ' s m e m b r a n e and the deepest layers of the stroma with polymorphous discrete opacities, depressions, and v e s i c l e s . Surrounding these le1
2
3
2 , 6 , 7
13
9 , 1 0 , 1 2
1 2 , 1 4
632
AMERICAN JOURNAL O F OPHTHALMOLOGY
of whitish lesions and clear spots at the level of Descemet's membrane that appear similar to those o f posterior polymorphous dystrophy. In these cases, the posterior stroma is not involved, whereas in our cases involvement occurred at all deep layers o f the cornea. In congenital hereditary corneal dystrophy one expects to find the initial involvement centrally with peripheral progression, but the youngest affected individual in our series had early peripheral involvement with none noted centrally. T h e corneal disorder appears only mildly progressive since the youngest severely affected individual ( I I I - 3 ) and the eldest (1-1) had no significant visual impairment. However, the cornea may undergo a uniform thinning in time, an apparently unique finding in posterior corneal dystrophies. Although there is evidence o f consanguinity, an autosomaldominant mode o f inheritance seems most likely.
MAY, 1977
the three-generation pedigree indicated an autosomal-dominant inheritance pattern.
11
SUMMARY
A family o f ten individuals aged 18 months to 7 5 years had biomicroscopic findings consisting o f large, amorphous, sheet-like opacifications of the posterior stroma and Descemet's membrane, and alterations o f the endothelium. A uniform thinning of the cornea was present. T h e s e findings do not conform to previously described corneal dystrophies. T h e condition appears minimally progressive and
REFERENCES
1. Grayson, M., and Wilbrandt, H.: Pre-Descemet dystrophy. Am. J. Ophthalmol. 64:276, 1967. 2. Duke-Elder, S., and Leigh, A. G.: Diseases of the Outer Eye. Cornea and Sclera. In Duke-Elder, S. (ed.): System of Ophthalmology, vol. 8, pt. 2. London, Henry Kimpton, 1965, pp. 947, 950, 951. 3. Thomsitt, J . , and Bron, A. J . : Polymorphic stromal dystrophy. Br. J. Ophthalmol. 59:125,1975. 4: Klouman, O. F.: A peculiar type of corneal endothelial dystrophy. Acta Ophthalmol. 43:534, 1965. 5. Theodore, F . H.: Congenital type of endothelial dystrophy. Arch. Ophthalmol. 21:626, 1939. 6. McGee, H. B., and Falls, H. F.: Hereditary polymorphous deep degeneration of the cornea. Arch. Ophthalmol. 50:462, 1953. 7. Hogan, M. J., and Bietti, G.: Hereditary deep dystrophy of the cornea (polymorphous). Am. J . Ophthalmol. 68:777, 1969. 8. Grayson, M.: The nature of hereditary deep polymorphous dystrophy of the cornea: Its association with iris and anterior chamber dysgenesis. Trans. Am. Ophthalmol. Soc. 72:516, 1974. 9. Maumenee, A. E.: Congenital hereditary corneal dystrophy. Am. J. Ophthalmol. 50:1114, 1960. 10. Malbran, E . S.: Corneal dystrophies: A clinical, pathological, and surgical approach. Am. J . Ophthalmol. 74:771, 1972. 11. Levenson, J . E . , Chandler, J. W., and Kaufman, H. E.: Affected asymptomatic relatives in congenital hereditary endothelial dystrophy. Am. J . Ophthalmol. 76:967, 1973. 12. Antine, B.: Histology of congenital hereditary corneal dystrophy. Am. J. Ophthalmol. 69:964, 1970. 13. François, J.: Heredity in Ophthalmology. St. Louis, C. V. Mosby, 1961, p. 312. 14. Pearce, W. G., Tripathi, R. C , and Morgan, G.: Congenital endothelial corneal dystrophy. Clinical, pathological, and genetic study. Br. J. Ophthalmol. 53:577, 1969.
