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M.-L. Quintyn-Ranty, J.-C. Quintyn, C. Mazerolles, S. Moalic, M.-B. Deslisle; Congenital Cystic Eye. Invest. Ophthalmol. Vis. Sci. 2007;48(13):3599.
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To report a case of a congenital cystic eye
During the neonatal period, a baby girl presented an intraorbital mass in the left eye, which pushed back the upper eyelid without infiltrating it. There was no palpebral defect or facial dysmorphism. Tomodensitometry revealed a solid, cystic orbital mass, with no calcification or bone lysis. The brain scan was normal. On the seventh day following birth, the mass was excised . It presented a hematic content with peripheral adherences which led to its fragmentation. There was no identifiable ocular globe within the orbit. Microscopic examination revealed that the excised material was essentially made up of neuroglial cells. Throughout a fibrillar base composed of numerous vascular sections, astrocytic elements were dispersed, around a regular, unexceptional nucleus, with a few neurons. Examination revealed positive anti-neurofilament antibody staining of axonal extensions observed in the fibrillar zones. Retinal type elements were mixed with the neuroglial tissue, or distributed around the edges. Epithelial structures with papillary architecture, and resembling ciliary bodie srevealed irregular staining for AE1-AE3 anti-cytokeratin antibodies. Areas of sclerosed collagen and some dystrophic calcification were also present. A diagnosis of congenital cystic eye was made (cystic anopthalmia). At seven months, the infant was in excellent general health, with good adaptation to monocular vision. The child’s psychomotor development was perfectly normal.
Congenital cystic eye is characterized by arrested development of the eye, which remains at the embryonic stage of the optic vesicle. Teratogenesis occurs around the fourth week. Development of a soft, bluish, retro-palpebral mass can be observed, usually at the center of the orbit. The histological aspects vary from one case to another. The cyst is usually surrounded by dense, fibrous conjunctive tissue, resembling the sclera, to which the muscular and adipose tissues are attached. The interior of the cyst is surrounded by immature retinal tissue. The cyst lumen generally contains neuroglial tissue. The lens is absent due to the non-development of crystalline placodes. Serial sections should be used to examine for an ocular remnant in order to exclude a colobomatous cyst (microphthalmia with cyst).
Congenital cystic eye usually affects only one eye, its etiology is unknown, particularly since there is no hereditary characteristic. It is a rare ocular malformation, with fewer than 30 cases described.
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