Anesthetic implications of Jacobsen syndrome

R. Blaine Easley, Devin Sanders, Jamie McElrath-Schwartz, Jackie Martin, J. Mark Redmond

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)66-71
Number of pages6
JournalPaediatric anaesthesia
Volume16
Issue number1
DOIs
StatePublished - Jan 1 2006

Keywords

  • 11q terminal deletion disorder
  • 11q-syndrome
  • Anesthesia
  • Cardiac surgery
  • Jacobsen syndrome
  • Paris-Trousseau thrombocytopenia syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Anesthesiology and Pain Medicine

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