Coloboma of the lens is characterized by notching of the equator of the lens. The cause of the condition is faulty development of the zonule. The lens deprived of its normal pull in the defective region is thicker and more spherical as if there were no loss of its substance. We report five cases of lens coloboma and localized zonular defect without any associated systemic anomalies. One case included retinal detachment; another included an iris coloboma; and a third included iris, choroid and optic disk colobomas and retinal detachment with lens coloboma. The remaining two cases were without associated ocular anomalies. The pathogenic mechanisms and relationship of this defect with the differentiation of the tertiary vitreous and development of the zonule is discussed. The defect most likely occurs in the third to fourth months of gestation during differentiation of the tertiary vitreous in response to toxic, inflammatory or genetic factors.
Colobomatous macrophthalmia with microcornea syndrome (MIM 602499) is a very rare eye malformation. To date, only two families with a total number of eight patients have been reported. We report on 13 additional cases in a single three-generation family and describe the eye findings and quantitative parameters of corneal curvature and axial ocular dimensions. All affected relatives had bilateral involvement with typical inferonasal iris coloboma, chorioretinal coloboma, microcornea, and varying degrees of axial enlargement associated with myopia. Additional findings included flatter corneal curvatures and shallower anterior chambers. Iridocorneal angle abnormalities associated with elevation of intraocular pressure were detected in three patients. The pedigree confirms the autosomal dominant pattern of inheritance with complete penetrance.
The pupils of neonates and premature infants often need to be dilated for retinal examination. The drops used for this purpose have some known side effects. This study investigated the effects and side effects of these drops. In this prospective randomized study, 80 healthy neonates were randomly assigned to eight groups. In Group A 1% cyclopentolate was used; in Group B 1% tropicamide; in Group C 2.5% phenylephrine; in Group D 1% cyclopentolate and 1% tropicamide; in Group E 2.5% phenylephrine and 1% tropicamide; in Group F 0.5% cyclopentolate, 0.5% tropicamide and 2.5% phenylephrine; in Group G 1% cyclopentolate and 2.5% phenylephrine and in Group H 0.9% NaCl. Heart rate, systolic and diastolic blood pressures were recorded before, and 5, 10, 15, 30, 45, 60 minutes after instillation of the drops. Pupillary size was measured at baseline, 30 and 60 minutes. To stimulate the conditions of indirect ophthalmoscopic examination, the pupil sizes were measured under the intense beam of a halogen light. The results were analyzed statistically. Maximum side effects were seen in group C; the safest was group B and maximum mydriasis was achieved in group F.
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