Paediatric patients with Fanconi anaemia, considerations for their stomatological approach. Review of the literature.

Abstract

Fanconi´s anemia, described in 1927 by pediatrician Guido Fanconi, is a hereditary disease with autosomal recessive transmission pattern, associated with multiple mutations in at least 20 genes. It is a syndrome of genomic instability with a predisposition to congenital anomalies, bone marrow failure and cancer. The most frequent congenital anomalies include those seen in the VACTERL-H association. The main method to confirm the diagnosis is the chromosomal breakage test. Upon diagnosis of Fanconi anemia, following a management plan in a specialized haemato-oncology center where regular surveillance of the systems affected by the disease is recommended . Oral findings may include microdontia, supernumerary teeth, congenital absences, gingivitis, and periodontitis. Objectives: To analyze a stomatological care protocol for pediatric patients with Fanconi's anaemia by means of a review of the literature. Materials and methods: Literature review of scientific articles from the PubMed and Google Scholar platforms. Photographs of patients from the National Institute of Pediatrics. Results: periodic check-ups should be scheduled for these patients due to the risk of developing neoplasia; cellular values and the use of antimicrobial prophylaxis should be considered when carrying out dental treatment, the approach to periodontopathies should be in accordance with the severity, and the use of local hemostatics should be indicated in some cases. Conclusion: The pediatric stomatologist must be familiar with this pathology and its systemic implications to provide safe, quality care. The approach is multidisciplinary, and the success of treatment depends largely on the cooperation of the patient and family.