Faculty Collaboration Database

Successful use of spinal anesthesia in a patient with severe Klippel-Trénaunay syndrome associated with upper airway abnormalities and chronic Kasabach-Merritt coagulopathy. J Anesth 2010 Feb;24(1):134-8

Date

01/08/2010

Pubmed ID

20054587

DOI

10.1007/s00540-009-0835-3

Scopus ID

2-s2.0-76649115597 (requires institutional sign-in at Scopus site)   16 Citations

Abstract

Klippel-Trénaunay syndrome is a rare disorder characterized by the triad of capillary or cavernous hemangiomas, venous varicosities or malformations, and soft tissue or bone hypertrophy. Neuraxial anesthesia in patients with Klippel-Trénaunay syndrome has been infrequently described and has not been previously reported when accompanied by consumptive coagulopathy with thrombocytopenia (Kasabach-Merritt syndrome). The authors describe their clinical management of a 23 year-old woman with Klippel-Trénaunay syndrome who presented for elective total knee arthroplasty. Her past medical history was notable for chronic Kasabach-Merritt syndrome, hypersplenism with pancytopenia, and intermittent lower gastrointestinal bleeding resulting from colonic hemangiomas. The physical examination revealed several large cavernous hemangiomas located on her right face, neck, chest, arm, and leg. No hemangiomas were noted within the dermatomal levels innervated by the upper lumbar spine. The neck hemangioma was very large and filled with blood when the patient assumed a supine position, making it almost impossible for her to breathe. The oropharynx revealed markedly hypertrophied soft tissue, pharyngeal, and hypopharyngeal hemangiomas, and a Mallampati class IV airway. Spinal and epidural hemangiomas were excluded based on a magnetic resonance imaging study before surgery. Kasabach-Merritt coagulopathy was corrected preoperatively by administration of cryoprecipitate. These interventions allowed the authors to safely perform a spinal anesthetic for the operation. The current case illustrates that major conduction anesthesia may be safely performed in patients with Klippel-Trénaunay disease provided that preoperative imaging studies exclude neurovascular involvement and coexisting coagulopathy is appropriately corrected.

Author List

Holak EJ, Pagel PS

MESH terms used to index this publication - Major topics in bold

Adult
Airway Obstruction
Anesthesia, Spinal
Arthroplasty, Replacement, Knee
Chronic Disease
Factor VIII
Female
Fibrinogen
Hemangioma, Cavernous
Humans
Klippel-Trenaunay-Weber Syndrome
Magnetic Resonance Imaging
Severity of Illness Index
Spine
Syndrome
Thrombocytopenia
Treatment Outcome
Young Adult