Treatment of the median mandibular cleft in Richieri-Costa-Pereira syndrome with a customized total mandibular prosthesis: A case report

Abstract

Richieri-Costa-Pereira syndrome (RCPS) is a rare genetic disease with an autosomal recessive inheritance pattern characterized by craniofacial alterations such as microsomia, Pierre-Robin sequence, mandibular cleft, absence of lower central incisors, short stature, and changes in the upper and lower limbs. Mandibular clefts are rare and can vary from a slight notch in the lower lip or mandibular alveolus to a complete mandibular cleavage. Only three cases of total mandibular rehabilitation with a customized prosthesis have been published, and none have described a patient born with a mandibular cleft. With the aid of computer-aided design/computer-aided manufacturing technology, virtual surgical planning, and customized manufacturing of surgical materials, the rehabilitation of this case became attainable. In this study, we describe the case of a patient with RCPS and median mandibular cleft treated with a customized total mandibular prosthesis.

Keywords

• Mandibular reconstruction
• TMJ prosthesis
• Virtual surgical planning
• Clef mandible
• Robin sequence