Orthodontic Treatment for Bloch-Sulzberger Syndrome in Patient with Cleft Lip and Palate

Abstract

We performed orthodontic treatment, fitted prostheses, and provided restorative treatment in a patient with Bloch-Sulzberger syndrome and cleft lip and palate during the early mixed dentition period. We report the case after a subsequent 6-year retention phase including the period of pubertal growth. A girl aged 8 years 4 months visited our hospital with the chief complaint of crowding of the anterior teeth and anterior crossbite. She had bilateral cleft lip, alveolus, and palate; a Class II molar relationship; winging of both the maxillary bilateral central incisors; and spacing in the mandibular anterior teeth arches. Anterior crossbite comprised 0 mm overbite and −1 mm overjet. The crown diameter was at least one standard deviation smaller than normal in both the deciduous and permanent teeth, and the crowns were slightly peg-shaped. Panoramic radiograph confirmed congenital absence of 21 permanent teeth. Cephalometric analysis revealed poor growth of the maxilla, downward growth of the mandible, and lingual inclination of the maxillary central incisors. The diagnosis was skeletal anterior crossbite with cleft lip, alveolus, and palate, accompanied by hypodontia. Orthodontic treatment comprised an edgewise appliance and an expansion arch to improve crowding and anterior crossbite. The appliance was removed 2 years after treatment initiation, followed by crown restorations of the maxillary central incisors and mandibular deciduous anterior teeth. A metal retainer was then fitted to the maxillary dentition. She was subsequently placed in a 6-year retention phase including pubertal growth, during which occlusal stability and esthetics were maintained.