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Purpose <p>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.</p> Methods <p>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.</p> Results <p>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.</p> Conclusion <p>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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月16日

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  • Marine Petit,
  • Léa Hilt,
  • Magali Hernandez

摘要

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.