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Anesthetic Management of a Child with Diamond‑Blackfan Syndrome


Affiliations
1 Department of Anaesthesiology, St. John’s Medical College and Hospital, Bengaluru, Karnataka, India
 

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.

Keywords

Anemia, Diamond‑Blackfan, general anesthesia, pure red cell aplasia
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  • Anesthetic Management of a Child with Diamond‑Blackfan Syndrome

Abstract Views: 128  |  PDF Views: 73

Authors

Alok Narasimha
Department of Anaesthesiology, St. John’s Medical College and Hospital, Bengaluru, Karnataka, India
Nischala Dixit
Department of Anaesthesiology, St. John’s Medical College and Hospital, Bengaluru, Karnataka, India
Latha P. John
Department of Anaesthesiology, St. John’s Medical College and Hospital, Bengaluru, Karnataka, India
Swaraj Cherian
Department of Anaesthesiology, St. John’s Medical College and Hospital, Bengaluru, Karnataka, India

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.

Keywords


Anemia, Diamond‑Blackfan, general anesthesia, pure red cell aplasia