Abstract

Tricho-Dento-Osseous syndrome is a rare autosomal dominant disorder. Immunosuppressive drugs, though critical to the treatment, have undesirable effects on patient's healing process. This is a case report of a 10-year-old female, suffering from Tricho-Dento-Osseous syndrome who was under treatment with penicillamine. As the dental treatment was sought too late, both central and lateral incisors on the right side of mandible had severe enamel hypoplasia and tooth necrosis was evident. A large lesion was found in anterior mandible. After proper treatment of the teeth with appropritately considering the systemic condition of the patient, the favorable result was achieved.

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