Anaesthetic Management of a Patient with Klippel–Trenaunay Syndrome for Above-knee Amputation

Dear Editor, Klippel–Trenaunay syndrome (KTS) is a rare congenital disorder and the main features include port-wine stain birthmark, capillary and venous malformations, varicose veins, lymphatic malformations and overgrowth of soft tissues and bones.[1] We are reporting a case of a 25-year-old-female having KTS with arteriovenous (AV) malformations. The patient was posted for left above-knee amputation for ischemic below-knee (BK) stump. She had large AV malformations of the left thigh, gluteal region and pelvis and underwent BK amputation 11 years back due to ischaemic limb. The current aortogram with peripheral angiogram showed high-flow AV malformation in the left lower limb with feeders from the left superficial femoral artery, external iliac artery and multiple right internal iliac artery branches. Transarterial embolisation was done 1 day before surgery. Pre-operative haemoglobin was 10.2 g/dL. Other blood investigations and echocardiography were normal. The patient was on enoxaparin 40 mg twice daily, which was stopped on the night before surgery. Our plan of anaesthesia was general anaesthesia (GA) with invasive haemodynamic monitoring. The patient received midazolam 1 mg, fentanyl 100 μg and propofol 80 mg and was intubated following vecuronium 4 mg. Two 16G peripheral venous accesses were obtained. The left radial artery and internal jugular vein were cannulated for haemodynamic monitoring. Anaesthesia was maintained with isoflurane (1%–2%) in oxygen-air (1:1) mixture. Intra-operatively, despite the application of tourniquet above the knee with pressures kept at 250 mmHg, the patient bled profusely, mainly from the bone. The patient lost approximately 2 L of blood intraoperatively and received six units of packed red blood cells, four units of fresh frozen plasma, 1 L of hydroxyethyl starch and 4 L of Ringer’s lactate. Noradrenaline at 0.02–0.1 μg/kg was required to maintain mean arterial pressure >60 mmHg. The patient was extubated on the table at the end of surgery and the post-operative period was unremarkable. Anaesthetic concerns in patients with KTS are the possibility of difficult airway (facial anomalies, upper airway angiomas and soft-tissue hypertrophy of airway), the potential for massive intraoperative bleeding, local intravascular coagulation within the malformation, disseminated intravascular coagulation, consumptive coagulopathy and thrombocytopenia.[2,3] In patients with KTS, venous thrombophlebitis is common, which could lead to pulmonary thromboembolism, pulmonary hypertension and right ventricular failure.[4] AV malformations can produce high-output congestive heart failure. Therefore, pre-operative echocardiography and anti-thrombotic prophylaxis are mandatory. Intraoperative surges in blood pressure should be avoided in view of possible brain haemangiomas leading to cerebrovascular accidents. The central neuraxial block may be considered only in those patients who do not have haemangioma of the spine and surrounding structures. If pre-operative radiological imaging of the spine is not available, it is advisable to administer only GA. Unexpected bleeding, even in minimally invasive surgery, should be anticipated in patients with KTS.[5] The reason for massive intraoperative bleeding in our patient, despite the use of tourniquet, could be attributed to non-collapsibility of bone vasculature. The central neuraxial block was avoided due to the presence of AV malformations in the lumbar epidural space. It is concluded that patients with KTS pose unique challenges to the anaesthetist and require detailed pre-operative assessment and preparedness for managing massive intraoperative haemorrhage. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.