Anesthesia for surgery related to Klippel-Trenaunay syndrome: a review of 136 anesthetics.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation characterized by the triad of varicose veins or venous malformations, capillary malformations that may involve neurovascular structures, and bony or soft tissue hypertrophy in affected limbs. Areas such as the trunk, bowel, bladder, and spinal cord may be involved as well. KTS should not be confused with Klippel-Feil syndrome, which involves abnormalities of the cervical vertebrae. Anesthetic management for patients with KTS has only been described in limited case reports that caution about potential airway difficulty but do not report surgical hemorrhage requiring transfusion.

METHODS: We performed an electronic search of the Mayo Clinic medical record database to identify patients who had undergone an anesthetic for surgery related to KTS. Review of medical records was performed for type of surgery, anesthetic technique, airway management and difficulty, medications used, intraoperative fluid administration, transfusion requirements, vascular access used, and postoperative complications.

RESULTS: Eighty-two unique patients were identified who underwent 134 general anesthetics and 2 lumbar neuraxial anesthetics for surgeries related to KTS. Preoperatively, 27% of patients had a history of recurrent bleeding, 24% recurrent cellulitis, 9% deep vein thrombosis, and 2% pulmonary embolism. The mean age at time of surgery was 21 ± 15 years. The majority of surgical procedures involved laser coagulation or varicose vein sclerotherapy or stripping. All of the 74 direct laryngoscopies and tracheal intubations were performed on the first attempt without difficulty. Mask ventilation was possible in all 131 patients for whom this was attempted, with only 1 requiring an oral airway. Documented estimated blood loss ranged from 20 to 18,000 mL, with a mean of 740 ± 2739 mL. Use of a tourniquet did not obviate the possibility of substantial blood loss. The only significant postoperative complication involved a calf hematoma after vein stripping and avulsion that required return to the operating room for evacuation.

CONCLUSIONS: Patients with KTS have multiple associated comorbidities relevant to perioperative management. In contrast to previous reports, difficulty with airway management was not encountered. Surgery related to severe KTS may be associated with massive hemorrhage despite tourniquet use, and the anesthesiologist should anticipate the need for appropriate fluid resuscitation. Neuraxial techniques may be considered only if the possibility of trauma to neurovascular malformations has been excluded with recent spine imaging.