Anesthetic management of a child with diamond-Blackfan Anemia and cyanotic congenital heart disease undergoing cleft lip repair

Diamond-Blackfan anemia (DBA) is a rare congenital form of pure red cell aplasia that is often complicated by congenital anomalies such as craniofacial anomalies and heart disease. We report a case of cleft lip repair performed under general anesthesia in a child with DBA. The patient was a male aged 12 years old, 95.8cm in height, and 12.8 kg in body weight He had anemia, a double-outlet right ventricle (DORV), and coarctation of the aorta (CoA). He had been treated with the administration of prednisolone and red blood cell transfusion for the anemia. Because steroid therapy increases the risk of infection, he was unable to undergo a radical operation for DORV. He exhibited cyanosis with an S_pO2 of 75% under 2//min oxygen, and we considered tracheal intubation to be difficult because of the craniofacial anomaly. Before the induction of anesthesia, his S_pO2 was 76%. Anesthesia was induced with thiopental. Because tracheal intubation was difficult, we performed oral intubation using a video laryngoscope. Anesthesia was maintained with oxygen, air, sevoflurane, and fentanyL To prevent infective endocarditis (IE), we administered ampicillin just before the operation, and 2% lidocaine with 1/240, 000 adrenalin was used for local anesthesia Under mechanical ventilation with the pressure mode, the F_IO2 was kept at 0.5 to maintain the S_pO2 at around 75%. In the present case, we needed to manage respiration and circulation because of cyanotic congenital heart disease, to take measures because of a difficult tracheal intubation, to prevent IE, and to prepare for red blood cell transfusion for anemia As a result, we were able to manage the general condition of a child with Diamond-Blackfan anemia and cyanotic congenital heart disease during general anesthesia safely without any complications.