FIG. 4.

Systemic features of Fraser syndrome. A, Digital malformations. Syndactyly of the fingers, which is the most common nonocular feature of Fraser syndrome. B, Ear malformations. Low-set, malformed, and posteriorly rotated ears. C, Musculoskeletal abnormalities. A furrow in the forehead corresponding to a groove in the frontal bone with an overlying abnormal brow hair pattern. Also, note the bifid nose. D, Cerebral malformations. Macrocephaly due to hydrocephalus. Fraser syndrome is compatible with normal cranial development but both microcephaly and macrocephaly have been reported.^14,145 Note contralateral hypoplastic left nostril and microphthalmia. E, Musculoskeletal abnormalities. Talipes varus. F, An abnormal tongue of hair extending from the temple to the eyebrow is not uncommon in Fraser syndrome, and is considered by some authors to be a minor feature of the disease.³⁸ G, High arched palate. H, Nasal malformations. A broad nose and a depressed broad nasal bridge are common nasal abnormalities in Fraser syndrome and a minor feature of the disease.