Dyskeratosis congenita: Anesthetic management

Aim: The most severe complications developed in DKC patients are bone marrow failure (BMF), difficult airway and pulmonary fibrosis. Therefore, the clinical and laboratory data of the patients should be assessed and the systemic examination should be carefully made prior to general anesthesia. Materials and Methods: The present case will discuss the preoperative preparation and the anesthetic method in a pediatric DKC patient with BMF, who was scheduled for dental treatment under general anesthesia. Discussion and Conclusion: DKC patients should be screened for BMF-related pancytopenia, difficult airway and opportunistic infections. Pulmonary examination should be made carefully and it should be prepared for the postoperative respiratory distress. Operation should be scheduled when the patient's clinical and laboratory values are optimal. And ancillary equipment should be prepared for patients who are expected to have difficult airway problems