faculty

Publications

Anesthetic Management of a Child with Diamond‑Blackfan Syndrome

Groups and Associations Alok Narasimha, Nischala Dixit, Latha P John, Swaraj Cherian
Karnataka Anaesthesia Journal 2018

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.
Key words: Anemia, Diamond-Blackfan, general anesthesia, pure red cell aplasia