This case report presents the orthodontic treatment of a patient with Witkop syndrome, an autosomal dominant genetic disorder characterized by the absence of several teeth and abnormalities of the nails. The patient, a 6-year 4-month-old boy, was referred to our clinic for treatment of severe overjet and openbite. Radiographic and clinical evaluations showed peg-shaped maxillary lateral incisors and the congenital absence of three mandibular incisors as well as spoon-shaped fingernails. Treatment of openbite and overjet was initiated with functional appliances, and fixed orthodontic appliances were inserted at age 10 years 3 months. The edentulous spaces are being maintained for implants that will be provided once the patient's growth is complete. Evaluations of long-term treatment results to date have indicated improvements in both facial and dental esthetics.
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