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Related Experiment Videos

Congenital cystic eye.

Rainer Guthoff1, Rüdiger Klein, Wolfgang E Lieb

  • 1Department of Ophthalmology, Julius Maximilians University, Josef-Schneider-Strasse 11, 97080 Wuerzburg, Germany. r_guthoff@yahoo.de

Graefe'S Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Fur Klinische Und Experimentelle Ophthalmologie
|December 17, 2003
PubMed
Summary

Congenital cystic eye, a rare orbital malformation, presents as an intraorbital cavity lined by neuroglial tissue. This case details a boy with an enlarging cyst, confirmed via imaging and histology to be neuroglial tissue, requiring surgical excision.

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Area of Science:

  • Ophthalmology
  • Developmental Biology
  • Pathology

Background:

  • Congenital cystic eye is a rare orbital malformation characterized by an intraorbital cavity lined with neuroglial tissue.
  • This condition represents a primary developmental abnormality of the globe.

Observation:

  • A 4-year-old boy presented with anophthalmia and a progressively enlarging congenital cystic lesion in the right orbit.
  • Imaging revealed a hyperintense cystic tumor with wall calcifications, lacking globe structures or intracranial communication.

Findings:

  • Histopathological examination showed fibrovascular tissue lined by neuroglial tissue with calcified bodies.
  • Immunohistochemical staining confirmed glial fibrillary acid protein (GFAP), neuron-specific enolase (NSE), and neurofilament protein (NF).

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  • No ocular structures like cornea, lens, or retinal pigmented epithelium were identified.

    • Implications:

    • Congenital cystic eye results from an invaginational arrest during early fetal development (2-7mm stage).
    • Enlargement may be due to fluid secretion by the glial tissue.
    • Differential diagnoses include microphthalmia with cyst, cystic teratoma, ectopic brain tissue, and meningoencephalocele.