Anesthetic management of a child with Diamond-Blackfan syndrome

Abstract

Diamond-Blackfan anemia (DBA) is a rare congenital disorder that can pose a variety of anesthetic challenges to a clinician. A disorder of pure red cell aplasia is associated with other congenital anomalies such as craniofacial malformations, thumb or upper limb abnormalities, cardiac defects, urogenital malformations, and cleft palate. These anomalies are compounded by problems of iron overload and chronic steroid therapy. Anesthetic management of a child with DBA requires knowledge and skill to handle a difficult pediatric airway and a thorough understanding of the congenital heart lesion of the child and its interaction with anesthetic agents and surgery. Rarity of DBA, along with its associated problems and lack of awareness of these by the anesthesiologist, makes the occasional surgery in this population very challenging. We report a 1-year 3-month-old child, diagnosed to have DBA during infancy, posted for laparoscopic orchidopexy.