Research Letter |

Congenital Cystic Eye In Utero:  Novel Prenatal Magnetic Resonance Imaging Findings

James R. Singer, DO1,2; Patrick J. Droste, MS, MD2,3; Adam S. Hassan, MD2,3
[+] Author Affiliations
1Metro Health Hospital, Wyoming, Michigan
2Department of Neurology and Ophthalmology, Michigan State University, East Lansing
3Helen DeVos Children’s Hospital, Grand Rapids, Michigan
JAMA Ophthalmol. 2013;131(8):1092-1095. doi:10.1001/jamaophthalmol.2013.328.
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First described in 1939,13 congenital cystic eye is an exceedingly rare orbital malformation due to failure of optic vesicle invagination during embryogenesis.1 Approximately 30 to 40 cases have been reported. The malformation consists of anophthalmic orbit containing a fluid-filled cyst and, frequently, rudimentary ocular derivatives.1 Concomitant nonocular malformations including intracranial anomalies and systemic malformations are often found in association.1 Discovery is typically after birth. Diagnosis is based on clinical, imaging, and histopathological characteristics. Generally, surgical excision is performed, ameliorating the expansile cyst and facial dysmorphism.

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Figure 1.
T2-Weighted Magnetic Resonance Images

Coronal (A), axial (B), and sagittal (C) T2-weighted magnetic resonance images of the brain and orbits, showing a left orbital multiloculated cyst with homogeneous high signal intensity in the anterior cavity and a heterogeneous posterior cavity with mixed signal intensity. This heterogeneous appearance corresponds with multiple internal septations. Anophthalmia is noted.

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Figure 2.
Clinical Photographs and Axial T2-Weighted Magnetic Resonance Image

A, A large cystic orbital mass markedly distending the upper eyelid. Excoriated conjunctiva is present between the eyelids. B, Axial T2-weighted magnetic resonance image showing extraconal optic nerve projecting posteriorly from the mass. No significant formation of ocular structures is present. C, Patient at age 12 months with an ocular prosthesis fitted.

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Figure 3.
Hematoxylin-Eosin–Stained Histopathological Sections

A, The cystic eye is composed primarily of neuroglial tissue. Connective tissue forms the walls, and the inner layer is lined by ependymal epithelium (original magnification ×2). B, Microvilli on the surface of the ependymal epithelium (original magnification ×60). C, Scattered neural rosettes resembling primitive retinal elements (original magnification ×20). D, Foci of pigmented neuroepithelium, similar to retinal pigment epithelium (original magnification ×20).

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