Congenital cystic eye (also known as CCE or cystic eyeball) is an extremely rare ocularmalformation where the eye fails to develop correctly in utero and is replaced by benign, fluid-filled tissue.[1] Its incidence is unknown, due to the very small number of cases reported. An audit by Duke-Elder of the medical literature from 1880 to 1963 discovered only 28 cases. The term was coined in 1937 by the renowned ophthalmologistIda Mann.[2]
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 skin appendages attached to the skin surrounding the eyes. Association with intracranial anomalies has been reported.[3]
Treatment
Treatment of CCE is usually by enucleation, followed by insertion of an ocular implant and prosthesis.
^Pillai, AM; Rema; Sambasivan, M (Mar–Apr 1987). "Congenital cystic eye--a case report with CT scan". Indian Journal of Ophthalmology. 35 (2): 88–91. PMID3450626.
^Tsitouridis, I; Michaelides, M; Tsantiridis, C; Spyridi, S; Arvanity, M; Efstratiou, I (June 2010). "Congenital cystic eye with multiple dermal appendages and intracranial congenital anomalies". Diagnostic and Interventional Radiology (Ankara, Turkey). 16 (2): 116–21. doi:10.4261/1305-3825.DIR.2054-08.1. PMID19847771. S2CID10299449.
