Tracheal Varicose Veins Associated with Klippel–Trenaunay Syndrome

Klippel-Trenaunay syndrome is a rare congenital syndrome involving blood vessels and bony or soft tissue hypertrophy. Vascular malformations may present in any organ or tissue. General anesthesia is generally preferred for surgery, owing to the risks of vascular malformation and coexisting coagulopathy.¹  A 26-yr-old parturient with Klippel-Trenaunay syndrome was scheduled for Cesarean delivery. She had no history of hemoptysis. We performed awake preoperative bronchoscopic evaluation of the airway, because massive bleeding in the trachea was reported in a patient with Klippel-Trenaunay syndrome.²  Varicose veins on anterior wall of the upper trachea, dilated blood vessels, and abrasion of the mucosa were observed (panels A and B). Cesarean delivery was performed under spinal anesthesia; however, general anesthesia was performed owing to postoperative hemorrhage. Flexible fiberoptic intubation was performed, and the cuff was carefully placed distal to the varicose vein because of limited space between the glottis and the varices. To minimize coughing and bleeding, the endotracheal tube was removed over a bronchoscope with sedation and muscle paralysis then bridged with a supraglottic airway device, which was removed after confirming spontaneous ventilation. Opioids, dexmedetomidine, or lidocaine may be administered for preventing cough. Endotracheal intubation may cause massive hemoptysis in patients with tracheal varices.¹  If intubation is necessary, flexible fiberoptic intubation should be used. In case of massive hemoptysis, a rigid bronchoscope, a balloon-tipped vascular catheter, and a double-lumen endotracheal tube should be prepared.³  A thoracic surgeon, pulmonologist, or interventional radiologist should be on stand-by or immediately available if there is high risk of airway bleeding.