The Carney complex with ocular signs suggestive of cardiac myxoma

We treated a patient who had ophthalmic findings of the Carney complex that led to a search for and the discovery of asymptomatic cardiac myxoma. Substantial morbidity and mortality are associated with the complex because of the occurrence of cardiac myxoma. Facial and eyelid lentigines, conjunctival and caruncle pigmentation and eyelid pigmentation may precede signs or symptoms of cardiac myxoma. A study of the patient's primary relatives disclosed manifestations of the complex transmitted in a manner consistent with mendelian autosomal dominant inheritance.