Anesthetic implications of Jacobsen syndrome

Jacobsen syndrome (JS), also known as 11q-syndrome, is a congenital disorder associated with a deletion of the long arm of chromosome 11. Patients with JS characteristically exhibit multiple dysmorphic features, developmental delay, cardiac anomalies, and platelet abnormalities. Anesthetic issues related to the care of patients with JS concern airway management secondary to short neck, abnormal mouth shape and micrognathia/retrognathia, a high incidence of cardiac anomalies, and platelet dysfunction. Importantly, platelet abnormalities affect 95% of reported JS patients and involve platelet number, size and function. Two children with JS who required open heart surgery are presented and anesthetic management issues discussed. These patients represent the first reports regarding the perioperative issues in caring for patients with JS.