261 - Sotos Syndrome Full-Mouth Rehabilitation: A Case Report

Abstract:

Introduction: Sotos syndrome is a rare autosomal dominant overgrowth disorder characterized by distinctive facial features, developmental delay, and macrocephaly, most commonly caused by NSD1 haploinsufficiency . It is associated with multisystem involvement, including cardiac, renal, neurologic, and otolaryngologic abnormalities. Common oral manifestations include premature tooth eruption, high-arched palate, prognathism, and dental anomalies, with increased caries risk due to psychomotor impairment .

Case Report: This case report presents a 3-year-old female patient with Sotos syndrome who was diagnosed with early childhood caries involving multiple teeth with pulpal pathology. Fusion of the mandibular right primary central and lateral incisors was observed. Due to extensive treatment needs and limited cooperation related to the patient’s developmental condition, comprehensive dental rehabilitation under general anesthesia was performed, including pulpectomy and placement of stainless steel and zirconia crowns. Regular postoperative follow-up and topical fluoride therapy were provided. Clinical recovery was satisfactory, and preventive care focusing on caregiver instruction and dietary counseling was implemented to support long-term oral health.

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