Retained primary teeth in STAT3 hyper-IgE syndrome: Early intervention in childhood is essential

Abstract Objectives: To optimize dental management we evaluated development of the dentition and long-term outcome of dental treatment in STAT3 hyper-IgE syndrome (STAT3-HIES), a rare primary immunodeficiency clinically overlapping with atopic dermatitis. Next to eczema, elevated serum-IgE, and recurrent infections, STAT3-HIES patients present with characteristic facies, midline defects, and retained primary teeth. Materials and Methods: We assessed dental histories of 13 molecularly defined STAT3-HIES patients by questionnaires, radiographs, and dental investigations. Results: Primary tooth eruption was unremarkable in all STAT3-HIES patients. Primary tooth exfoliation and permanent tooth eruption was delayed in 83% of patients due to unresorbed tooth roots. Permanent teeth erupted spontaneously in all patients receiving primary teeth extraction of retained primary teeth during average physiologic exfoliation time. A complex orthodontic treatment was needed in one patient receiving delayed extraction of primary molars and canines. Conclusion: To enable spontaneous eruption of permanent teeth in children with STAT3-HIES, we recommend extracting retained primary incisors not later than 9 years of age and retained primary canines and molars not later than 13 years of age after having confirmed the presence of the successor permanent teeth by radiograph. Clinical Relevance: The association of STAT3-HIES with retained primary teeth is important knowledge for dentists in any field as timely extraction of retained primary teeth avoids dental complications.