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

Yukiko Nishioka, Hiroshi Tanimura, Rieko Onishi, Yuka Honda-Wakasugi, Akiko Kawase, Hitoshi Higuchi, Shigeru Maeda, Takuya Miyawaki

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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 SpO2 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 SpO2 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 FIO2 was kept at 0.5 to maintain the SpO2 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.

Original languageEnglish
Pages (from-to)669-671
Number of pages3
JournalJournal of Japanese Dental Society of Anesthesiology
Issue number5
Publication statusPublished - 2017


  • Cleft lip repair
  • Diamond-Blackfan anemia
  • General anesthesia

ASJC Scopus subject areas

  • Dentistry(all)
  • Anesthesiology and Pain Medicine


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