Abstract
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.
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References
Duke-Elder. Anomalies of the lens. In: Congenital deformities, normal and abnormal development (System of ophthalmology, Vol. 3). London: Henry Kimpton, 1964: 706–708.
Scott L, Creig H. Lens. In: Taylor D, ed. Pediatric Ophthalmology. Boston: Blackwell, 1990: 300.
Spencer HW. Ophthalmic pathology: An atlas and textbook. Philadelphia: Saunders, 1985: 433–434.
Hovland KR, Schepens CL, Freeman HM. Developmental giant retinal tears associated with lens coloboma. Arch Ophthalmol 1968; 80: 325–31.
Scheie HG, Albert DM. Adler's textbook of ophthalmology. Philadelphia: Saunders, 1969: 74–83.
McDonnell JM. Ocular embryology and anatomy. In: Ryan SJ, ed. Retina, Vol. 1. St. Louis: Mosby, 1989: 5–11.
Mann I. Developmental abnormalities of the eye. London: British Medical Association, 1957: 315–18.
Pau H. Differential diagnosis of eye diseases. Stuttgart: Georg Thieme Verlag, 1988: 304–305.
Yanoff M, Fine BS. Ocular pathology: A text and atlas. Philadelphia: Lippincott, 1989: 369–374.
Boniuk M, Hittner HM. Congenital retinal disinsertion syndrome. Trans Am Acad Ophthalmol Otolaryngol 1975; 79: 827–34.
Barrie KP, Jacobson E. Unilateral cataract with lens coloboma and bilateral corneal edema in a guanaco. J Am Vet Med Assoc 1978; 173: 1251–52.
Cenamo G, Liguori G, Pezone A, Laccarino G. Morning glory syndrome associated with marked persistent hyperplastic primary vitreous and lens colobomas. Br J Ophthalmol 1989; 73: 684–86.
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Bavbek, T., Ōgūt, M.S. & Kazokoglu, H. Congenital lens coloboma and associated pathologies. Doc Ophthalmol 83, 313–322 (1993). https://doi.org/10.1007/BF01204333
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DOI: https://doi.org/10.1007/BF01204333