Editor—Corneal dermoids (CND) (MIM 304730) are rare, congenital, benign tumours involving the cornea. Histologically, the dermoids consist of a combination of ectodermal elements including keratinised epithelium, hair, and sebaceous glands and mesodermal elements including fibrous tissue, fat, and blood vessels in different proportions The tumour is by definition a choristoma since it is histologically a normal tissue in an abnormal site. Clinically, these tumours appear as opacification of the cornea at birth and if untreated may result in blindness (fig 1). Treatment includes surgical removal of the tumour with corneal transplantation. When the tumours affect both eyes, surgical intervention is indicated within the first 3 months of life since untreated cases may be irreversible by that time.

Most CND cases are sporadic and unilateral. However, three hereditary forms associated with congenital dermoids involving the cornea have been described. The Goldenhar syndrome (oculoauriculovertebral dysplasia, MIM 164210) is a multiple anomaly syndrome that involves the eyes, ears, and the vertebra.1 The dermoids are mostly unilateral, originate in the corneal-scleral border, also known as the limbus, and rarely affect the centre of the cornea. Although most cases are sporadic, familial cases with an autosomal dominant mode of inheritance have been reported. The second form with hereditary dermoids of the cornea is ring dermoids (MIM 180550), an autosomal dominant condition with isolated dermoids at the periphery of the cornea at the limbus.2

A third form of congenital dermoids involving the cornea was initially described by our institution in a family of Puerto-Rican ancestry.3 The three affected males had congenital, bilateral, and central corneal opacification at birth and no additional abnormalities.4 The described pattern of transmission was suggestive of X linked recessive inheritance. To date, this is the only family …