Singleton Merten syndrome: root resorption or root development defects? Two case reports with 5-year follow-up data

Abstract Purpose Singleton Merten syndrome (SMS) is a rare immunogenetic disorder with highly variable expression that affects multiple systems. Dental anomalies in patients with SMS are important diagnostic features but are poorly reported in the literature. Methods This case report describes the case of two brothers with SMS and presents their oral phenotype. A comparative analysis of the two patients’ panoramic X-rays at different ages was performed using two quantitative radiographic measurement methods for root development. Results No significant difference in the root length of permanent teeth was observed between the different periods tested. These data confirm the absence of root development in permanent teeth. Moreover, delayed root resorption of the primary teeth was observed. Conclusion Five-year follow-up of these two patients revealed that the roots of the permanent teeth were not affected by root resorption but presented a root development defect. These findings specify the dental phenotype of patients with SMS.