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Heterochromia Associated with Coloboma of the Iris
NOTES, CASES, INSTRUMENTS HETEROCHROMIA ASSOCIATED W I T H COLOBOMA O F T H E IRIS ROBERT C. DREWS,
M.D.
Clayton, Missouri AND
Pico, M.D. Santwce, Puerto Rico
GUILLERMO
Each of us has seen a patient who had both heterochromia and a coloboma of the iris. We could find no prior description of this association. CASE REPORTS
Case 1—A six-year-old white girl was seen in November, 1966, at the University of Puerto Rico because of a defective left eye, present since birth. Her medical history was non-contributory. There were no other physical abnormalities. The right iris was thinner at the six o'clock posi tion than elsewhere. There was a complete coloboma of the left iris at the six o'clock position. The right iris was blue and the left was brown. The left cornea was smaller than the right, but the eye was not microphthalmic. Left esotropia measured 55 prism diopters for distance and near. A small coloboma was present inferiorly in the right choroid. A large coloboma of the left choroid extended from a point near the disk to involve a large area inferiorly. The optic disks were normal. Visual acuity was 20/25 in the right eye and light perception and projection in the left. Retinoscopy following atropine instilla tion indicated a correction of +2.50 sph C —0.75D cyl.ax.l80° for the right eye. It was impossible to perform accurate retinoscopy on the left eye. When this child was last seen in May, 1970, the findings were unchanged. Case 2—A 57-year-old woman was first seen in May, 1955, for a mild conjunctivitis in both eyes. A coloboma had been present in the left iris since birth. Vision in this eye, always slightly less than in the right, had previously been corrected to 20/38. Corrected vision in the right eye was 20/15. The right iris was blue and the left a yellowish blue. There was no microphthalmos. Early arteriolarsclerosis was present. There was no sign of a choroidal coloboma in either eye, but a partial colo boma was present in the left iris. The right eye From the Department of Ophthalmology and the Oscar Johnson Institute, Washington University School of Medicine, and the Department of Oph thalmology of the University of Puerto Rico. Reprint requests to Robert C. Drews, M.D., 211 North Meramec, Clayton, Missouri 63105.
showed no evidence of prior iritis. Astigmatism in the left eye measured 3.50 diopters. Visual acuity with best correction was 20/25 in the right eye and 20/60 in the left. This patient has been followed at irregular inter vals. In 1963, Goldmann perimetry showed normal visual fields. In 1968, a cortical cataract in the left eye was more advanced then one in the right eye. In a rabbit colony bred by Jesberg1 for colo boma of the uvea, a few of the animals were born with heterochromia rather than a coloboma. Fol lowing this experience, Jesberg saw a patient with both coloboma of the iris and heterochromia. In Jesberg's patient, the eye without the coloboma had hyperpigmentation of the conjunctiva as well as the iris. We submit that Jesberg's experience, together with the patients reported herein, tends to suggest the association of heterochromia and coloboma as a true syndrome. SUMMARY
Coloboma of the iris was associated with heterochromia in two female patients. In both, the darker and amblyopic eye had the coloboma. Another investigator has described a similar patient, and in this case, too, the eye without a coloboma had hyperpigmentation. In a colony of rabbits bred for coloboma of the uvea, a few were born without coloboma, but with heterochromia instead. REFERENCE
1. Jesberg, D.: Personal communication.
N E W SURGICAL I N S T R U M E N T CARRYING CASE DAVID J. MCINTYRE,
M.D.
Bellevue, Washington Although this is an age of rapid advance ment in instrumentation, we have seen little innovation in the area of surgical instrument storage and carrying. Existing cases are rather archaic combinations of little elastic straps, cardboard and loose boxes. T h e pri-
From the Eye Clinic of Bellevue, Bellevue, Washington. Reprint requests to David J. McIntyre, M.D., 1300 116th Avenue N.E., Bellevue, Washington 98004. 827
M.D.
Clayton, Missouri AND
Pico, M.D. Santwce, Puerto Rico
GUILLERMO
Each of us has seen a patient who had both heterochromia and a coloboma of the iris. We could find no prior description of this association. CASE REPORTS
Case 1—A six-year-old white girl was seen in November, 1966, at the University of Puerto Rico because of a defective left eye, present since birth. Her medical history was non-contributory. There were no other physical abnormalities. The right iris was thinner at the six o'clock posi tion than elsewhere. There was a complete coloboma of the left iris at the six o'clock position. The right iris was blue and the left was brown. The left cornea was smaller than the right, but the eye was not microphthalmic. Left esotropia measured 55 prism diopters for distance and near. A small coloboma was present inferiorly in the right choroid. A large coloboma of the left choroid extended from a point near the disk to involve a large area inferiorly. The optic disks were normal. Visual acuity was 20/25 in the right eye and light perception and projection in the left. Retinoscopy following atropine instilla tion indicated a correction of +2.50 sph C —0.75D cyl.ax.l80° for the right eye. It was impossible to perform accurate retinoscopy on the left eye. When this child was last seen in May, 1970, the findings were unchanged. Case 2—A 57-year-old woman was first seen in May, 1955, for a mild conjunctivitis in both eyes. A coloboma had been present in the left iris since birth. Vision in this eye, always slightly less than in the right, had previously been corrected to 20/38. Corrected vision in the right eye was 20/15. The right iris was blue and the left a yellowish blue. There was no microphthalmos. Early arteriolarsclerosis was present. There was no sign of a choroidal coloboma in either eye, but a partial colo boma was present in the left iris. The right eye From the Department of Ophthalmology and the Oscar Johnson Institute, Washington University School of Medicine, and the Department of Oph thalmology of the University of Puerto Rico. Reprint requests to Robert C. Drews, M.D., 211 North Meramec, Clayton, Missouri 63105.
showed no evidence of prior iritis. Astigmatism in the left eye measured 3.50 diopters. Visual acuity with best correction was 20/25 in the right eye and 20/60 in the left. This patient has been followed at irregular inter vals. In 1963, Goldmann perimetry showed normal visual fields. In 1968, a cortical cataract in the left eye was more advanced then one in the right eye. In a rabbit colony bred by Jesberg1 for colo boma of the uvea, a few of the animals were born with heterochromia rather than a coloboma. Fol lowing this experience, Jesberg saw a patient with both coloboma of the iris and heterochromia. In Jesberg's patient, the eye without the coloboma had hyperpigmentation of the conjunctiva as well as the iris. We submit that Jesberg's experience, together with the patients reported herein, tends to suggest the association of heterochromia and coloboma as a true syndrome. SUMMARY
Coloboma of the iris was associated with heterochromia in two female patients. In both, the darker and amblyopic eye had the coloboma. Another investigator has described a similar patient, and in this case, too, the eye without a coloboma had hyperpigmentation. In a colony of rabbits bred for coloboma of the uvea, a few were born without coloboma, but with heterochromia instead. REFERENCE
1. Jesberg, D.: Personal communication.
N E W SURGICAL I N S T R U M E N T CARRYING CASE DAVID J. MCINTYRE,
M.D.
Bellevue, Washington Although this is an age of rapid advance ment in instrumentation, we have seen little innovation in the area of surgical instrument storage and carrying. Existing cases are rather archaic combinations of little elastic straps, cardboard and loose boxes. T h e pri-
From the Eye Clinic of Bellevue, Bellevue, Washington. Reprint requests to David J. McIntyre, M.D., 1300 116th Avenue N.E., Bellevue, Washington 98004. 827