ATYPICAL RETINOCHOROIDAL COLOBOMA IN PATIENTS WITH DYSPLASTIC OPTIC DISCS AND TRANSSPHENOIDAL ENCEPHALOCELE - REPORT OF 5 CASES

Objective: To evaluate the clinical significance of V- or tongue-shape d infrapapillary retinochoroidal depigmentation in association with dy splastic optic discs. Methods: Clinical data from all patients with V- or tongue-shaped infrapapillary retinochoroidal depigmentation and dy splastic optic discs were evaluated retrospectively. Results: Five pat ients with this atypical colobomatous anomaly had transsphenoidal ence phalocele. A sixth patient had an ectopic midline pharyngeal mass with no skull-base defect. Conclusion: In patients with optic disc dysplas ias, the finding of this V- or tongue-shaped retinochoroidal pigmentar y anomaly should prompt neuroimaging to look for transsphenoidal encep halocele.