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.

Klippel-Trenaunay syndrome: a rare cause of disabling pain after a femoral fracture

“…As regards the choice of anaesthesia for Klippel-Trenaunay syndrome, neuraxial techniques should be considered only if possible neurovascular malformations have been excluded by spine imaging [7][8][9][10]. In our case, owing to the impossibility of performing investigations that could validate recourse to local anaesthesia, we decided to use general anaesthesia.…”

Neuraxial techniques in patients with pre‐existing back impairment or prior spine interventions: a topical review with special reference to obstetrics

“…Pregnancy and uterine contractions during labour may enhance the bleeding risk due to engorgement of the vessel malformations 21,22 . In addition, Klippel–Trenaunay syndrome may be complicated by Kasabach–Merritt coagulopathy 23 . Magnetic resonance imaging (MRI) may allow to verify the possible safety of a neuraxial thoracic or a lumbar block but it should be performed as close as possible to the day of planned intervention.…”

Anesthestic management of Kassabach-Meritt Phenomenon in an adult: case report

“…Replacement of depleted clotting factors using fresh-frozen plasma has been recommended in patients with KMP before surgery [5]. Marked hypofibrinogenemia may necessitate the administration of cryoprecipitate to correct the underlying coagulopathy [16]. Recombinant factor VII therapy was also reported in a child with KMP undergoing major surgery [17].…”