View source for Congenital cystic eye

'''Congenital cystic eye''' (CCE) is an extremely rare [[ocular]] [[malformation]] where the eye fails to develop correctly ''[[in utero]]'' and is replaced by [[benign]], fluid-filled [[Tissue (biology)|tissue]]. <ref>[http://www.biomedcentral.com/1471-2415/3/7 BioMed Central | Full text | Congenital cystic eye with multiple dermal appendages: a case report<!-- Bot generated title -->]</ref>  Its [[incidence (epidemiology)|incidence]] is unknown, due to the very small number of cases reported.  An [[audit]] by Duke-Elder of the [[medical literature]] in [[English language|English]] from [[1880]] to [[1963]] discovered only 28 cases.  The term was coined in 1937 by the renowned [[ophthalmologist]] [[Ida Mann]].<ref>[http://www.ijo.in/article.asp?issn=0301-4738;year=1987;volume=35;issue=2;spage=88;epage=91;aulast=Pillai Congenital cystic eye-A case report with CT scan Pillai AM, R, Sambasivan M, - Indian J Ophthalmol<!-- Bot generated title -->]</ref>

[[Embryology|Embryologically]], the [[defect]] is thought to occur around day 35 of [[gestation]], when the [[vesicle]] fails to [[invaginate]]. Dysgenesis of the vesicle later in development may result in [[coloboma]], a separate and less severe malformation of the ocular structures.

CCE is almost always [[unilateral]], but at least 2 cases of [[bilateral]] involvement have been described. Patients may also present with [[cutaneous]] [[appendages]] attached to the skin surrounding the eyes.

[[Treatment]] of CCE is usually by [[enucleation of the eye|enucleation]], followed by insertion of an ocular [[implant (medicine)|implant]] and [[prosthesis]].

== References ==
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[[Category:Ophthalmology]]
[[Category:Eye]]
[[Category:Head and neck]]
[[Category:Congenital disorders]]
[[Category:Pediatrics]]


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