Coloboma of the lens is characterized by notching of the equator of th
e lens. The cause of the condition is faulty development of the zonule
. The lens deprived of its normal pull in the defective region is thic
ker and more spherical as if there were no loss of its substance. We r
eport five cases of lens coloboma and localized zonular defect without
any associated systemic anomalies. One case included retinal detachme
nt; another included an iris coloboma; and a third included iris, chor
oid and optic disk colobomas and retinal detachment with lens coloboma
. The remaining two cases were without associated ocular anomalies. Th
e pathogenic mechanisms and relationship of this defect with the diffe
rentiation of the tertiary vitreous and development of the zonule is d
iscussed. The defect most likely occurs in the third to fourth months
of gestation during differentiation of the tertiary vitreous in respon
se to toxic, inflammatory or genetic factors.