Ocular manifestations in Delleman syndrome (Oculocerebrocutaneous syndrome, OCC-syndrome) and encephalocraniocutaneous lipomatosis (ECCL). Report of three cases.

The authors present two patients with Delleman syndrome and one in with possible ECCL. Two boys with Delleman syndrome showed characteristic dysmorphic features with cerebral, ocular and skin malformations. The ocular anomalies consisted of eyelid coloboma, microphthalmia, iris coloboma and epibulbar lypodermoids. A third boy with possible ECCL syndrome had limbal lypodermoids, ectopia pupillae and aberrant iris tissue in the right eye. He showed an ipsilateral focal dermal hypoplastic defect within an area of alopecia of the scalp.

Sneddon's syndrome in a patient with homonymous hemianopia with macular sparing.

A 48-year-old women is described with the infrequent association of generalized livedo reticularis and cerebrovascular accident of idiopathic origin (Sneddon's syndrome-SS). Visual field testing revealed a left homonymous hemianopia with macular sparing. Though visual field impairments in SS have been reported, the type could usually not be specified precisely because of the dementia and lack of cooperation of the patients.