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Multicentric melanomas in the same eye are rare, with few cases substantiated by histology and serial sectioning. We report a patient with two documented choroidal malignant melanomas in one eye. The initial tumor spontaneously decreased in size for over 2 years before a second tumor appeared in a noncontiguous location in the same eye. After enucleation, serial sections showed that the two lesions were independent choroidal melanomas. We review the literature regarding multiple, independent intraocular choroidal malignant melanomas. ( Surv Ophthalmol 42 :441–448, 1998.

The clinical and histopathologic features of Axenfeld's anomaly and Rieger's anomaly and syndrome are reviewed, and recent findings regarding the pathogenesis of this spectrum of developmental disorders are discussed. Based on these observations, it has been suggested that a developmental arrest, in the third trimester of gestation, of tissues derived from the neural crest cells accounts for the ocular and most of the nonocular abnormalities in this group of disorders. Since previous collective terms, such as mesodermal dysgenesis and anterior chamber cleavage syndrome, are not consistent with these new observations, the alternative name, Axenfeld-Rieger syndrome, has been proposed. The differential diagnosis of the syndrome includes two additional spectra of disorders: the iridocorneal endothelial syndrome and the posterior polymorphous dystrophies. The most serious ocular problem in Axenfeld-Rieger syndrome is the associated glaucoma, which occurs in a high percentage of patients and is typically difficult to control. Recent observations regarding the mechanism of the glaucoma, as reviewed in this paper, provide guidance in the management of this aspect of Axenfeld-Rieger syndrome.

Cerebro-rhino-orbital phycomycosis (CROP) occurs predominantly in individuals with diabetes mellitus in a state of metabolic acidosis. Other forms of metabolic acidosis, especially in infants, may predispose to phycomycotic infections. CROP has also been reported in patients with leukemia or lymphoma. CROP usually begins in the palate or paranasal sinuses and rapidly spreads to the orbital contents. Proptosis, loss of vision, and ophthalmoplegia occur and death from cerebral involvement commonly ensues. The fungus tends to invade arteries and cause thrombosis and tissue infarction. Rhizopus is the most commonly isolated genus in CROP, accounting for almost all cases. The diagnosis can be strongly suspected by the characteristic clinical manifestations. Therapy includes treatment of the underlying disease, surgical excision of the necrotic tissue containing fungal elements and the systemic administration of amphotericin-B. The effect of treatment has improved since the disease was first described, but the condition still has a high mortality, especially if it is not diagnosed early.