A typical Hallermann-Streiff syndrome in a 3 year old child.

Hallermann Streiff syndrome is a rare congenital disorder characterized by dyscephaly, dental anomalies, proportionate nanism, hypotrichosis, cutaneous atrophy limited to the head, bilateral congenital cataracts and bilateral microphthalmia. Despite the marked craniofacial characteristics and oral findings, a relative lack of reports in the dental literature has been noted. In this article, a review of the literature regarding the general clinical features and differential diagnosis is presented. A case of a 3-year-old female is reported with special consideration on the management of her dental problems under general anesthesia. The anesthetic risks of such a procedure as well as the prognosis of her dental development are being discussed.