Syndromes/Craniofacial Anomalies
Niharika Jain, DMD
Pediatric Dental Resident
Case Western Reserve University — UH Rainbow Babies and Children’s Hospital, Cleveland, OH
Case Western Reserve University & UH Rainbow Babies and Children's Hospital
Cleveland, Ohio, United States
Gerald A. Ferretti, DDS, MS, MPH
Professor and Chair
Case Western Reserve University
Cleveland, Ohio, United States
Margaret Elaine Ferretti, DMD, MPH
Program Director
Case Western Reserve University
Rainbow Babies and Children's Hospitals, Case Western Reserve University
Cleveland, Ohio, United States
Introduction: Oligodontia is defined as congenitally missing 6 or more permanent teeth not including third molars. The prevalence of oligodontia is 3.9% in the North American population. Identification of such cases is important because a gene mutation causing oligodontia may also be part of a syndrome affecting skin, nails, eyes, ears, and skeleton. Specifically, the WNT10A gene may be associated with odontoonychodermal dysplasia and schopf-schulz-passarge syndrome which may present with thin hair, smooth tongue surface, and/or microdontia.
Case study: This presentation discusses a 14-year-old boy who was sent for consult at University Hospital’s Rainbow Babies and Children’s Hospital for the management of 19 congenitally missing permanent teeth diagnosed on an orthopantomogram. The patient is ASA-1, taking no medications and no known drug allergies. The patient’s physical exam consisted of normal hair distribution, symmetric face, and normal philtrum. The patient was devoid of smooth tongue, hyperkeratosis of the palms, and microdontia. The clinical and radiographic exam was evident of the patient in mixed dentition consisting of 13 erupted permanent teeth and 13 erupted primary teeth. Both patient’s mother and maternal uncle have a history of congenitally missing 2 maxillary lateral incisors. In this case study, the diagnosis and management of the patient’s WNT10A mutation explaining the congenitally missing teeth will be discussed.