CYSTIC MICROPHTHALMOS WITH CEREBROSPINAL FLUID (CSF) PRODUCING EPENDYME.

97th DOG Annual Meeting 1999

P526
CYSTIC MICROPHTHALMOS WITH CEREBROSPINAL FLUID (CSF) PRODUCING EPENDYME.

C. Belting¹, B. Lorenz¹, S. Kern¹, P. Held², M. Friedrich³, F. Hofstädter⁴

Introduction: Cystic microphthalmos is a rare disease entity. In some cases surgery may become necessary because of functional and/ or aesthetic reasons. Patient and Methods: We report on a little boy whom we first saw at the age of 1 week. At that time he presented a massively swollen, everted left lower eyelid. The eyeball was not visible. The right eye had a retinochoroidal coloboma including the optic nerve head. The patient was examined with various neuroimaging methods (orbital ultrasound, MRI, CT). The cyst content was examined biochemically, the cyst wall by light microscopy.

Results: B-scan detected a rudimentary globe, 1x1.5 cm in size, in the left upper nasal orbit. The orbit was filled with a cystic formation 2x3 cm in size, and consisting of various compartments. The orbital MRI confirmed the diagnosis of cystic microphthalmos. Because of an increasing eversion of the lower eyelid the cyst content was aspirated at the age of 4 months and analyzed. Biochemistry revealed cerebrospinal fluid (CSF) quality. Neuroimaging using both MRI and CT did not disclose any connection of the cyst with the subarachnoidal space. As the cyst refilled within a few weeks after aspiration, most of the cyst was excised surgically. Intraoperatively, an adhesion of at least 1 cm was found between the cyst and the optic nerve. There was no direct connection to the CSF. Light microscopy of the cyst disclosed ependymocytes. At follow-up, there was no clinically detectable refill of the remaining cyst.

Conclusions: Removal of the cyst was effective because the cyst was not connected to the subarachnoidal space. The rapid refill of the cyst after aspiration is explained by the presence of CSF producing ependymocytes. The microphthalmic eye important to induce orbital growth could be preserved.

¹Dept. of Pediatric Ophthalmology, Strabismology & Ophthalmogenetics,
²Dept. of Radiology,
³Dept of Neurosurgery,
⁴Dept. of Pathology; University of Regensburg, 93042 Regensburg

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