Anesthetic Management of an Adolescent With Diamond-Blackfan Syndrome During Posterior Spinal Fusion

Abstract

Diamond-Blackfan syndrome (DBS) is a rare autosomal dominant ribosomal disorder. Patients present primarily with severe hypoplastic anemia and reticulocytopenia. Due to the craniofacial defects, cardiac pathology, short stature, and skeletal abnormalities, surgical and anesthetic care are frequently required. The associated structural anomalies and end-organ involvement may impact the perioperative care. We present an 18-year-old adolescent with Diamond-Blackfan anemia (DBA) who required anesthetic care for posterior spinal fusion. Previous reports of anesthetic care for these patients are reviewed, the end-organ involvement is discussed, and options for anesthetic care are presented.