Work

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

Year: 2026

Type: article

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.

Source: Journal of Rare Diseases

DOI: 10.1007/s44162-026-00178-2

Authors: Marine Petit, Léa Hilt, Magali Hernandez

Institutions: Université de Lorraine, Centre Hospitalier Régional et Universitaire de Nancy, Stress, IMmunité, PAthogènes

Language: English

Topic: dental development and anomalies

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: diamond

OpenAlex ID: W7136151325