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PURPOSE: To describe the first case of intraocular teratoma associated with eyelid coloboma and the second reported case of intraocular teratoma. DESIGN: Interventional case report. METHODS: A left intraocular tumor was surgically resected from a 2-day-old female with an associated lower eyelid coloboma. RESULTS: Pathologic evaluation revealed a completely intraocular tumor comprising derivatives of all three germ cell layers giving a diagnosis of intraocular teratoma. The eyelid coloboma was repaired, and a scleral-wrapped hydoxyapatite-integrated orbital implant was placed. CONCLUSION: To our knowledge, this is the second reported instance of teratoma originating within the globe and the only reported case of teratoma associated with eyelid coloboma. Although exceedingly rare, intraocular teratoma should be added to the differential diagnosis of congenital intraocular tumors.

In a retrospective study, 50 (8.2%) of 610 patients with malignant melanoma of the ciliary body or choroid had extrascleral extension. Of 46 patients for whom follow-up data were available, 24 (52%) survived five years, 20 (42%) survived ten years, and 17 (37%) survived 15 years. Factors that seemed to affect survival significantly included intraocular tumor size and extraocular tumor size and location. Our observations did not support the role of primary exenteration. Five patients (11%) with extrascleral extension had orbital recurrences and all died within 31 months of surgery regardless of treatment.

Two patients with incompletely excised invasive papillary squamous cell carcinoma of the corneoscleral limbal conjunctiva had subtle clinical signs suggesting incomplete transmural extension or localized intraocular tumor invasion. We attempted a wide en bloc resection procedure in each case. Histopathologic examination disclosed that the surgical margins were involved with tumor cells and the eyes were subsequently enucleated. The enucleated eyes disclosed more extensive microscopic intraocular involvement than had been anticipated.

These various intraocular disorders, which, either in their primary manifestations or after the fundus picture has become obscured by secondary developments, may suggest the existence of intraocular malignancy, are discussed seriatim from the point of view of differential diagnosis and as to their literary history. Some personal cases are reported, historically significant illustrations from the literature are reproduced, and the monograph is supplemented by ample bibliographies under the respective heads. (In addition to the text illustrations, see the colored frontispiece, containing a drawing of the author's case of Hippel's disease and a drawing of a large choroidal tuberculoma.)

The case of intraocular tumor here reported is of especial interest because its course was followed five years before its removal, and it illustrates the unreliability of intraocular tension, in differentiating intraocular tumor from simple detachment of the retina. Read before the American Ophthalmological Society, June, 1920.

The difficulty of determining whether a detached retina hides an intraocular tumor makes every symptom bearing on the subject important. Two cases are reported in which deep ocular pain was present and an intraocular tumor found when the eye was enucleated. Read before the American Ophthalmological Society, June, 1923.