title: Successful use of spinal anesthesia in a patient with severe Klippel-Trénaunay syndrome associated with upper airway abnormalities and chronic Kasabach-Merritt coagulopathy.
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.
authors.affiliation: Clement J. Zablocki Veterans Affairs Medical Center, Anesthesia Service, 5000 W. National Avenue, Milwaukee, WI 53295, USA. eholak@mcw.edu
authors.forename: Elena J
authors.initials: EJ
authors.lastname: Holak
authors.forename: Paul S
authors.initials: PS
authors.lastname: Pagel
mesh_terms: D000328:Adult; D000402:Airway Obstruction / Q000209:etiology*; D000775:Anesthesia, Spinal; D019645:Arthroplasty, Replacement, Knee; D002908:Chronic Disease; D005169:Factor VIII / Q000627:therapeutic use; D005260:Female; D005340:Fibrinogen / Q000627:therapeutic use; D006392:Hemangioma, Cavernous / Q000150:complications*; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications*; D008279:Magnetic Resonance Imaging; D012720:Severity of Illness Index; D013131:Spine; D013577:Syndrome; D013921:Thrombocytopenia / Q000150:complications*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: C026912:cryoprecipitate coagulum; D005169:Factor VIII; D005340:Fibrinogen