Pediatric Anesthesia ISSN 1155-5645
CASE REPORT
Hemodynamic instability during anesthesia in an adolescent with Loeys-Dietz syndrome: a case report Alexandra C. Bunting1 & M. Dylan Bould2 1 Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada 2 Department of Anesthesiology, The Children’s Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada
Keywords Loeys-Dietz syndrome; familial thoracic aneurysm; anesthesia; angiotensin receptor antagonists/adverse effects; hypotension/surgery; hypotension/drug effects Correspondence Alexandra C. Bunting, Faculty of Medicine, University of Ottawa, 451 Smyth Road, Ottawa, ON K1H 8M5, Canada Email: [email protected]
Summary We present a case of a 12-year-old male with Loeys-Dietz syndrome (LDS), a rare life-threatening genetic disorder. Multiple manifestations of LDS were present, including easy bruising, aortic root dilatation, multiple areas of vessel tortuosity, and joint laxity. The patient’s medications included a betablocker and an angiotensin II receptor antagonist for prophylaxis against further aortic root dilatation due to his LDS. He experienced intraoperative hemodynamic instability and became pulseless during an orthopedic procedure, which had to be abandoned. Anesthesia was reattempted 2 days after cessation of the patient’s antihypertensive medications and was successful with only minor postoperative hypertension.
Section Editor: David Polaner Accepted 26 July 2014 doi:10.1111/pan.12511 Implication Statement: We present the second published report of anesthesia for Loeys-Dietz syndrome and the first with significant complications along with a strategy for managing intraoperative hemodynamic compromise for these patients.
Introduction Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder similar to Marfan’s syndrome, with a prevalence of
CASE REPORT
Hemodynamic instability during anesthesia in an adolescent with Loeys-Dietz syndrome: a case report Alexandra C. Bunting1 & M. Dylan Bould2 1 Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada 2 Department of Anesthesiology, The Children’s Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada
Keywords Loeys-Dietz syndrome; familial thoracic aneurysm; anesthesia; angiotensin receptor antagonists/adverse effects; hypotension/surgery; hypotension/drug effects Correspondence Alexandra C. Bunting, Faculty of Medicine, University of Ottawa, 451 Smyth Road, Ottawa, ON K1H 8M5, Canada Email: [email protected]
Summary We present a case of a 12-year-old male with Loeys-Dietz syndrome (LDS), a rare life-threatening genetic disorder. Multiple manifestations of LDS were present, including easy bruising, aortic root dilatation, multiple areas of vessel tortuosity, and joint laxity. The patient’s medications included a betablocker and an angiotensin II receptor antagonist for prophylaxis against further aortic root dilatation due to his LDS. He experienced intraoperative hemodynamic instability and became pulseless during an orthopedic procedure, which had to be abandoned. Anesthesia was reattempted 2 days after cessation of the patient’s antihypertensive medications and was successful with only minor postoperative hypertension.
Section Editor: David Polaner Accepted 26 July 2014 doi:10.1111/pan.12511 Implication Statement: We present the second published report of anesthesia for Loeys-Dietz syndrome and the first with significant complications along with a strategy for managing intraoperative hemodynamic compromise for these patients.
Introduction Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder similar to Marfan’s syndrome, with a prevalence of
